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Nephrol. Dial. Transplant.-2003-E. Paediatric Nephrology-255-80

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					Monday, June 9                                                 E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                          255

In conclusion, our data indicate that alpha3beta1 integrin is not required       The aim of this study was to identify differences in protein expression
for adhesion of podocytes to the ECM but instead modulates focal ad-             in mononuclear cells from patients with INS during relapse and healthy
hesion strength, size and focal contact composition. The further study of        patients.
alpha3beta1 integrin and its regulatory function will provide new insights       Peripheral blood mononuclear cells were taken from 5 chidren with INS
into the molecular machinery mediating podocytes foot process efface-            during relapse and 6 controls. Mononuclear cells proteins were solubilized
ment.                                                                            and isoelectrofocusing was performed using non-linear immobilized pH
                                                                                 gradient. Second dimension was performed using a horizontal 12-14% gra-
                                                                                 dient polyacrylamide gel. Gels were silver stained. After scanning with a
 M793         LEVEL OF INTERLEUKIN-5 IN PLASMA AND URINE IN                      laser densitometer, gels were analysed and matched with the Melanie Soft-
              CHILDREN WITH STEROID-SENSITIVE NEPHROTIC                          ware (GeneBio) to highlight significant differences in protein patterns. For
              SYNDROME                                                           protein identification, preparative gels were stained using SYPRO Ruby.
                                                                                 After automatic matching, each group of spots was statistically analysed
Olga Morozova, Elena Moskaleva, Olga Turpitko, Vladimir Dlin.
                                                                                 using spots relative volume and manual editing was performed. 9 spots
Department of Nephrology, Institute of Pediatrics & Child Surgery,
                                                                                 were chosen that differ significantly between relapse versus control and
Moscow, Russian Federation
                                                                                 were picked, destained and trypsinised. Sequencing was performed using
As known, steroid-sensitive nephrotic syndrome (SSNS) often associates           TOFTOF-MALDI-MS/MS mass spectrometer.
with atopic diseases. It might be suggest the same mechanism, predom-            After matching of the 11 gels, 9 spots were highlighted as having a differ-
inance activity of Th2 cytokines such as interleukin (IL)-4, -5, -13, par-       ential expression pattern in relapsing versus control patients.
ticipates in pathogenesis of these disorders. The results of different recent
investigations of activity Th1/Th2 cells in NS are contradictory and dis-        Unidentified spots with a differential expression pattern
cussed. The aim of our study was determine level of IL-5, cytokine Th2                            Protein 1 Protein 2 Protein 3 Protein 4 Protein 5 Protein 6 Protein 7
cells, in plasma and urine of children with SSNS.




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                                                                                 Isoelectric point 5.99       5.46       6.43      6.49     5.54      5.48      6.41
The level of IL-5 was determined in plasma and urine of 12 children              Measured
aged from 3 to 15 with SSNS by using CYTELISA Human IL-5 (CY-                       molecular
TIMMUNE, USA). Two children with onset and 10 with relapse (mean                    weight (Da)    57000     35000      26000     24000     24000    22000     21000
2.8±0.5) of NS were observed. None of the children were receiving pred-          Relative volume
nisolone therapy neither at the time of investigation nor within the preced-        INS/control     0.17/     0.45/     0.13/     0.43/     0.16/     0.38/    0.36/
                                                                                                    0.47      0.03      0.00      0.00      0.00      0.00     0.004
ing 2 weeks. All children were divided on two groups: the 1st group, 8
children who had NS associated with atopic diseases and/or allergic family
history; the 2nd , 4 patients with NS but without any atopy in presentation.     Mass spectrometry revealed a rise in the expression of L-plastin (Ip: 5.19,
The control group consisted of 6 healthy children.                               Mw: 67 000 Da) and a decrease in the expression of triosephosphate iso-
It was recorded detectable level of IL-5 in the plasma of 7 children from the    merase (Ip: 6.29, Mw: 29 000 Da) in INS in relapse. The 7 other spots are
1st group and in only 1 from the 2nd (sensitivity of assay 0.92 pg/ml). The      being currently identified using higher amounts of loaded proteins.
level of IL-5 in the plasma of children from the 1st group was significant        L-plastin is an actin-binding protein involved in signal transduction in leu-
higher to compare with control (M±m 160.13±50.47 vs. 8.7±2.11 pg/ml;             cocytes through its phosphorylation and interaction with integrins. Using a
p<0.01). The level of IL-5 in the plasma depended on neither severity of         subtracted cDNA library, Sahali and al. (J Am Soc Nephrol, 2002) showed
NS nor frequency relapse. Furthermore, it was shown positive correlation         an upregulation of several genes in T-lymphocytes in INS patients in re-
between plasma level of IL-5 and degree of increase of serum total IgE           lapse implicating cell activation, including L-plastin. Further studies should
(r=0.69; p<0.02).                                                                focus on the role of this protein in the lymphocytes dysfunction in INS.
It was found detectable level of IL-5 in the urine of only 3 children (11;       Triosephosphate isomerase, a key component of the glycolytic pathway,
92.5 and 98 pg/ml) from the 1st group, but was not revealed in other patients    was found to be augmented in lung, colon and bladder carcinomas. The
and control. These children were characterized by present atopic disease,        link between this protein and INS remains to be determined.
allergy between first degree relatives, the highest level of serum total IgE      These data show an increase expression of a mononuclear cell protein
(more 17-fold increase than in healthy) and serum specific IgE antibody to        involved in the cytoskeleton integrity and signalling only present in INS
dust-mite and/or pollen allergens. It is interested to note that level of IL-5   patients in relapse. A proteomic approach may contribute to precise the
in the plasma of two patients was also high (318 and 150 pg/ml), but one         role of mononuclear cells in the pathogenesis of INS.
child had non-detectable level of IL-5 in the plasma.                                                                           Free Communication June 12
Thus, it was shown increase of the level IL-5 in the plasma and urine
of children with SSNS associated with atopic diseases. IL-5 detectable in
urine might be result of local production by cells situated in kidney or          M795           NEPHRIN GENE (NPHS1) IN PATIENTS WITH MINIMAL
migrated to it. The results support that Th2 pattern might be involved in the                    CHANGE NEPHROTIC SYNDROME
pathogenesis SSNS, especially in children with atopy.
                                                                                 Anne-Tiina Lahdenkari1 , Marjo Kestilä2 , Christer Holmberg1 ,
                                                                                 Olli Koskimies1 , Hannu Jalanko1 . 1 Hospital for Children and
                                                                                 Adolescents, University of Helsinki, Helsinki, Finland; 2 Department of
                                                                                 Molecular Medicine, National Public Health Institute, Helsinki, Finland
E2 Paediatric clinical nephrology, acute and
                                                                                 Nephrin is a major component of the glomerular filtration barrier. Muta-
   chronic renal failure, bone and mineral                                       tions in the nephrin gene (NPHS1) lead to congenital nephrotic syndrome
   metabolism, anemia                                                            of the Finnish type (CNF). The role of nephrin in acquired kideney diseases
                                                                                 is not known. In this work we studied whether genetic changes in nephrin
                                                                                 might predispose to the development of minimal change nephrotic syn-
 M794         MONONUCLEAR CELLS PROTEINS INVOLVED IN                             drome (MCNS), which is the most common form of nephrotic syndrome
              IDIOPATHIC NEPHROTIC SYNDROME OF CHILDHOOD:                        in childhood. The analysis of the NPHS1 gene in the Finnish MCNS pa-
              A PROTEOMIC APPROACH                                               tients is especially relevant, since high frequency of NPHS1 mutations
                                                                                 (Fin-major and Fin-minor) occur in this population.
Elsa Gonzalez1 , Markus Kemper2 , Neuhaus Thomas2 , Eric Girardin1 .
1                                                                                Genomic DNA was isolated from 25 adults who had been treated for MCNS
  Pediatrics, Geneva University Hospital, Geneva, Switzerland;
2                                                                                in the childhood (1960-1980). Clinical data, biopsy findings and the present
  Pediatrics, Kinderspital, Zurich, Switzerland
                                                                                 health status were recorded. All 29 exons of NPHS1 were sequenced after
Clinical and laboratory data suggest the implication of T-lymphocytes in         PRC amplification. Five new missense mutations were found in MCNS
the pathogenesis of idiopathic nephrotic syndrome (INS) but the proteins         patients. In addition, three MCNS patients had a T294I -change in exon 8
involved are still unknown.                                                      which has previously been found in a mother of a CNF child. One MCNS
256      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                       Monday, June 9

patient was heterozygous for Fin-major mutation which corresponds to the         M797 BONE MINERAL DENSITY AND BONE TURNOVER
frequency of the general Finnish population (1/40 individuals).                           MARKERS IN CHILDREN WITH PRIMARY NEPHROTIC
In MCNS patients, totally thirteen different nucleotide changes were ob-                  SYNDROME
served in eleven exons with a mean of 3.0 variants/patient. Ten of these
                                                                                Ashraf Bakr, Magdy El-Zeny, Samer Abu Al-Hassan, Amr Sarhan,
caused an amino acid substitution. In order to evaluate the significance of
                                                                                Ayman Hammad, Nehad Shalaby. Pediatrics, Mansoura University
the findings, the same eleven exons were sequenced in 25 healthy controls.
                                                                                Children’s Hospital, Mansoura, Dakahlia, Egypt
A mean of 2.5 nucleotide changes per person were observed in this group
indicating a high frequency of polymorphism in the nephrin gene. Nine of        This study was conducted to highlight the prevalance as well as the pat-
the 20 steroid sensitive MCNS patients had a mild disease and 11 were           tern of osteopenia in children with primary nephrotic syndrome (PNS)
frequent relapsers (up to 27 episodes). The mean number of nucleotide           at normal level of renal function. Measurment of bone mineral density
changes was 2.3 and 3.7 in the two groups, respectively (P< 0.05). Six of       (BMD) in the lumber spine region [L2-L4] using dual energy x-ray ab-
the frequent relapsers were steroid-depent and had clearly more nucleotide      sorpometry (DEXA) by lunar DPX-IQ system was done in 42 patient with
variants (mean 4.8) as compared to the milder cases (mean 2.4) (p< 0.001).      PNS (27 males and 15 females, aged 3 to 15 years) and 352 healthy age-
While the pathogenesis of MCNS is still unknown, an immunologiacl at-           and sex-matched Egyptian children. Serum levels of osteocalcin, parathy-
tack against the glomerular filter is probably involved. Mutations in genes      roid hormone (PTH) and 25 OH cholicalciferol (25-HCC) and urinary
coding for the glomerular proteins may make the filter susceptible to this       deoxypyridinoline (Dpd) were measured in 14 patients out of the studied
attack. Our results indicate that MCNS is not assoaciated with any specific      children and 12 healthy age- and sex-matched controls. Osteopenia was
mutation in the nephrin gene. However, it seems that mutations and poly-        observed in 13 patients (30.95%), 7 of them had non-severe osteopenia
morphims are common in NPHS1 and the number of nucleotide changes               (Z-score between -1and -2.5) and 6 had severe osteopenia (Z-score more
influences the severity of the disease.                                          negative than -2.5). Compared to controls, patients showed significantly
                                             Free Communication June 12         lower 25-HCC [median= 17.5, range 11-33.3, vs 66, range 61-69 ng/ml;
                                                                                P= 0.0001] and higher urinary Dpd [medin= 81.1, range 76.5-174.9 vs 47.1,




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                                                                                range 29.0-58.1 nmol/l; P= 0.001]. However no significant differences were
 M796 SPECTRUM OF PODOCYTE GENE MUTATIONS                                       obseved in serum osteocalcin and PTH between patients and controls. No
          ASSOCIATED WITH NEPHROTIC SYNDROME                                    correlation was observed between Z-score and total dose of corticosteroid,
                                                                                sex and number of relapses. In conclusion, ostepenia was evident in about
Gian Marco Ghiggeri1 , Gianluca Caridi1 , Roberta Bertelli1 , Marco Di
                                                                                one third of children with PNS. This ostepenia is associated with increased
Duca1 , Monica Dagnino1 , Francesco Emma2 , Andrea Onetti Muda3 ,
                                                                                evidences of bone resorpitive markers and normal osteoblastic markers.
Francesco Scolari4 , Nunzia Miglietti5 , Gianna Mazzucco6 , Luisa Murer7 ,
                                                                                The low dose of 25 HCC points to the need of vitamine D supplemetation
Laura Massella2 , Gianfranco Rizzoni2 , Francesco Perfumo1 . 1 Nephrology
                                                                                in these patients.
Dept. - Lab. of Physiopathology of Uremia, G. Gaslini Institute, Genoa,
Italy; 2 Nephrology Unit, Bambino Gesù Hospital, Rome, Italy; 3 Dept. of
Experimental Medicine and Pathology, La Sapienza University, Rome,
                                                                                 M798 EFFECT OF AGE AT ONSET ON THE PROGNOSIS OF
Italy; 4 Nephrology Unit, Spedali Civili, Brescia, Italy; 5 Dept. of
                                                                                          CHILDHOOD STEROID SENSITIVE NEPHROTIC
Pediatrics, University, Brescia, Italy; 6 Dept. of Biomedical Sciences and
                                                                                          SYNDROME
Human Oncology, University, Turin, Italy; 7 Dept. of Pediatrics,
University, Padova, Italy                                                       Ali Derakhshan1 , Ghamar Hashemi1 , Mohammad Hossein Fallah Zadeh1 ,
                                                                                Gholamreza Bardestani1 . Pediatric Nephrology, Shiraz University of
We screened for podocin mutations 179 children with sporadic nephrotic
                                                                                Medical Sciences, Shiraz, Fars, Iran; Pediatric Nephrology, Shiraz
syndrome, 120 with steroid resistance, 59 with steroid dependence/frequent
                                                                                University of Medical Sciences, Shiraz, Fars, Iran; Pediatric Nephrology,
relapses. Fourteen steroid resistant patients presented homozygous muta-
                                                                                Shiraz University of Medical Sciences, Shiraz, Fars, Iran
tions that were associated with early onset of proteinuria and variable renal
lesions: 11 FSGS, 1 MCNS, 1 with IgM deposit and 1 with mesangial               Minimal change nephrotic syndrome (MCNS) is the most common from
C3 deposition. Single mutations of podocin were found in 4 steroid resis-       of idiopathic nephrotic syndrome (INS) in childhood. Although most of
tant and in 4 steroid dependent; 5 patients had the same mutation (P20L).       the children with INS respond to steroid, but a majority of them have
Among these, 2 had steroid/cyclosporin resistance, while 2 had steroid de-      subsequent relapses. In this study the effect of age at onset on the outcome
pendence and 1 responded to cyclosporin. The common variant R229Q               of steroid-responsive nephrotic syndrome (NS) is evaluated.
of podocin, recently associated with late-onset focal segmental glomeru-        Medical records of all children with INS (1-10 years old) and initial re-
losclerosis, had an overall allelic frequency of 4.2% vs 2.5% in controls.      sponse to steroid who had been followed for at least 7 years were reviewed.
To further define the implication of R229Q we characterized a familial           We considered age of onset, sex, number of relapses, pathology report and
case with two nephrotic siblings presenting the association of the R229Q        immunosupressive therapy other than steroid. They were studied in 3 age
with A297V mutation that were inherited from healthy mother and father          groups: 1-3 years, 4-6 years and 7-10 years at the onset of NS. Sixty-one
respectively. Immunohistochemistry with anti-podocin antibodies revealed        children (46 boys and 15 girls) were included in this study. There were
markedly decreased expression of the protein in their kidneys. All carri-       23, 24 and 14 children in the above mentioned age groups respectively.
ers of heterozygous coding podocin mutation or R229Q were screened for          Kidney biopsy had been done for 46 children of whom 76.5% had MCNS.
nephrin mutation that was found in heterozygosity associated with R229Q         Cyclophosphamide, chlorambucil and cyclospurin had been used for 18.4
in 1 patient. Finally, podocin loss of heterozygosity was excluded in one       and 1 of them respectively.
heterozygous child by characterizing cDNA from dissected glomeruli.             Children with onset of NS at 1-3, 4-6 and 7-10 years of age had (mean
These data outline the clinical features of sporadic nephrotic syndrome due     ± SD) 7 ± 5.92, 4.54 ± 3.74 and 1.57 ± 1.81 of relapses respectively.
to podocin mutations (homo- and heterozygous) in a representative pop-          The duration of disease was also 5.52 ± 4.15, 5.37 ± 3.57 and 1.92 ±
ulation with broaden phenotype, including patients with a good response         1.54 years respectively. Regarding the number of relapses and duration of
to drugs. The pathogenetic implication of single podocin defects ’per se’       disease, the differences between the 7-10 years age group and the other 2
in proteinuria must be further investigated, in view of the possibility that    groups were statistically significant (P< 0.05).
detection of a second mutation could have been missed. A suggestive al-         We concluded that age at onset is a reliable predictor of the course and
ternative is the involvement of other gene(s) or factor(s).                     prognosis of childhood steroid responsive NS.
                                               Free Communication June 12
Monday, June 9                                                    E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                257

 M799 NPHS1 WITH MUSCULAR DYSTONIA AND ATHETOSIS                                  and onset of nephrotic syndrome was 7.5 years. He had no family history
                   1                   1                      2                   and parental consanquinity. He also had homozygote R138X mutation and
Hanne Laakkonen , Tuula Lonnqvist , Marja Ala-Houhala ,
                                                                                  resistance to all therapies. He is still on hemodialysis.
Matti Nuutinen3 , Christer Holmberg1 . 1 Hospital for Children and
                                                                                  Podocin (NPHS2) gene heterozygote mutations were found in 2 of 12
Adolescents, University of Helsinki, Helsinki, Finland; 2 Department of
                                                                                  patients. The onset of the nephrotic syndrome was 9.5 years and 1 year in
Pediatrics, Tampere University Hospital, Tampere, Finland; 3 Department
                                                                                  these two boys. They also had no family history and parental consanguinity.
of Pediatrics and Adolescence, Oulu University Hospital, Oulu, Finland
                                                                                  They had heterozygote R138X and L169P mutations respectively. Renal
Congenital nephrotic syndrome of the Finnish type (NPHS1) is an autoso-           functional detoriation was not developed during their 39 and 45 months
mal recessively inherited disease. It is caused by a mutation in the nephrin      follow up.
gene (NPHS1). There are two main mutations of NPHS1, Fin-major and                Conclusion: This first NPHS2 gene mutation study in Turkey supported
minor, which both cause lack of nephrin and absence of the slit diaphragm         that NPHS2 mutations in SRNS patients were frequent in our population.
between the podocytes. This leads to severe proteinuria, nephrotic syn-           Patients with homozygote NPHS2 mutations have poor prognosis and pro-
drome, infections and death in infancy without renal transplantation.             gression to the ESRD is faster in this group. We think that rapidly end-stage
Between 1984 and 2002 six of the 68 NPHS1 patients (8,8%) diagnosed               renal disease progression is related to combination of two different muta-
at our institutions had similar neurological symptoms, four boys and two          tions in our first case.
girls. We now report this new clinical entity.                                    Note: The NPHS2 mutation results of the rest of SRNS patients with FSGS
The patients were born prematurely after 35+3 weeks (range 32+5 – 37+2            histology will be completed in a short time and presented in the congress.
weeks). Their mean height was 46± 3 cm and weight 2271± 543 g. Pla-
cental weight was 980± 220 g (= 43± 5% of birth weight). In four cases
the amniotic fluid was green. Proteinuria was detected on day 19 (range             M801 DOES THE TYPE OF STEROID RESISTANCE HAVE
1-56 days) and the diagnosis was made at 23 days (range 3-58 days). The                     PROGNOSTIC VALUE IN STEROID RESISTANT NEPHROTIC
NPHS1 mutations (N=5) were major/major in four and one major/minor                          SYNDROME IN CHILDREN?




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mutation.
                                                                                  S. Gulati, A. Saha, R.K. Sharma, R.K. Gupta, A. Gupta, A.P. Sharma.
All patients showed a severe dyskinetic cerebral palsy (CP) – like syn-
                                                                                  Nephrology & Pathology, Sanjay Gandhi Post Graduate Institute of
drome with dystonic features and athetosis. The symptoms of this CP-like
                                                                                  Medical Sciences, Lucknow, U.P., India
syndrome developed during the first year of life and were diagnosed at an
age of 3-14 months, typically at 8-11 months. Four patients died at an early      There is no study till date that has evaluated the prognostic significance
age, two of them during dialysis, one 23 days and another four months after       of the type of steroid resistance (SR) in idiopathic nephrotic syndrome
renal transplantation. Two patients are alive. One is 5, the other 9 years old.   (INS) in children. Ths study was conducted to compare the clinicopatho-
Both have severe motor defects, need a wheelchair and other devices to            logic spectrum and outcome of INS in children with SR who were initial
manage their everyday duties. Both are mentally active and social.                nonresponders (INR) as compared to subsequent nonresponders (SNR).
We have studied the clinical and neuroradiological picture and mitochon-          A retrospective analysis was done of 135 children with INS, who were
drial gene mutations in these children.                                           referred to our institute over the last 12 years who were SR. They comprised
All six patients have similar nephrological symptoms as all other NPHS1           15% of all children with in (135/900). Steroid resistance was defined as
patients. In addition, they have a similar neurological syndrome mimicking        no response to 4 weeks of standard prednisone therapy (60 mg/m2 /day).
dyskinetic CP with severe muscular hypotonia and vigorous dystonic and            This was classified into 2 subtypes: INR – if the child continued to have
athetoid movements. The reason for the neurological defect is still unclear.      proteinuria since onset and SNR if the child had a steroid responsive course
However, the prognosis of these patients seems to be poor and their active        followed by development of secondary steroid resistance. All these children
treatment should be critically decided on an individual basis.                    were subjected to kidney biopsy. They were treated with a variety of drugs
                                                                                  (oral or intravenous cyclophosphamide, cyclosporine, dexamethasone and
                                                                                  prednisone). Children with secondary causes of nephrotic syndrome and
 M800         PODOCIN (NPHS2) GENE MUTATION IN TURKISH                            those with inadequate biopsies were excluded. We compared the clinical,
              CHILDREN WITH STEROID RESISTANT NEPHROTIC                           biochemical, histopathologic subtypes and outcome in the 2 groups. Of the
              SYNDROME                                                            135 children, the majority 93/135 (68%) were INR. The mean age of onset
                                                                                  of symptoms in INR was significantly greater than in SNR (9.4 ± 5.05 vs
Afig Berdeli1 , Erkin Serdaroglu2 , Sevgi Mir2 , Alphan Cura2 . 1 Molecular
                                                                                  6.7 ± 4.99 yrs, p = 0.004). The clinical and biochemical features at onset
Medicine Laboratory, Ege University Medical School, Izmir, TR, Turkey;
2                                                                                 were similar in the 2 groups. Focal segmental glomerulosclerosis was the
  Department of Pediatric Nephrology, Ege University Medical School,
                                                                                  commonest histopathologioc subtype in both INR (55/93) as well as SNR
Izmir, TR, Turkey
                                                                                  (25/42). However all the children with Membranous NS (n=6) as well as
Objective: The aim of the present study was to determine the frequency            those with Mesangiocapillary glomerulonephritis (n=2) were INR. After a
of podocin (NPHS2) mutation and to investigate the fibrosis tendency with          mean followup of 45.5 ± 26.6 months, 18/135 (13.3%) children developed
PAI-1, AGT, ACE I/D, ATR-1 gene polymorphisms in Turkish children                 Chronic renal insufficiency. The mean serum albumin on followup was
with steroid resistant nephrotic syndrome (SRNS).                                 significantly greater in SNR as compared to INR (3.2 ± 1.0 vs 2.8 ±
Material and Methods: 188 children who referred to the three medical              0.9 g/dl, p=0.04). A significantly greater number of SNR children were in
centers in Ege Group between January 2002 – December 2002 and diag-               remission as compared to INR (27/42 vs 37/93, p=0.014).
nosed as idiopathic nephrotic syndrome were included the study. Fifty-two         We conclude that children with INS who are SNR have a better outcome
of them were diagnosed as SRNS according to the ISDKC criteria. Their             than those who are INR.
male/female ratio was 31/21 and the median time for the onset of nephrotic
syndrome was 21±33 months. Forty-two of the cases were diagnosed focal
segmental glomerulosclerosis (FSGS) with renal biopsy. Patients genomic            M802 DO RAS GENE (ACE AND AT1) POLYMORPHISMS AFFECT
DNA was extracted from EDTA blood. NPHS2 mutations were analyzed                            THE RISK AND OUTCOME OF FOCAL SEGMENTAL
using by PCR and direct DNA sequencing. NPHS2 mutations and PAI-1,                          GLOMERULOSCLEROSIS?
AGT, ACE I/D, ATR-1 gene polymorphisms were studied in 12 of 42 FSGS
                                                                                  Nesrin Besbas1 , Mehmet Alikasifoglu2 , Seza Ozen1 , Fatih Ozaltin1 ,
cases.
                                                                                  Rezan Topaloglu1 , Ergul Tuncbilek2 , Aysin Bakkaloglu1 . 1 Pediatric
Results: Podocin (NPHS2) gene homozygote mutations were found in 2
                                                                                  Nephrology, 2 Clinical Genetics, Hacettepe University Faculty of
of 12 patients. The first case was a girl and had no family history for
                                                                                  Medicine, Ankara, Turkey
nephrotic syndrome and parental consanquinity. The onset of the nephrotic
syndrome was 2.5 years old. She had resistance to all therapy modalities          Polymorphisms in the angiotensin converting enzyme (ACE) gene have
and end-stage renal disease developed when she was 4 years old. She is            been associated with an adverse outcome in a number of renal diseases.
still on peritoneal dialysis treatment now. She had homozygote R138X              Focal segmental glomerulosclerosis (FSGS) is characterized by nephrotic
and heterozygote 419delG NPHS2 mutations. The second case was a boy               syndrome and a high incidence of progressive renal disease in childhood.
258      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                         Monday, June 9

We have aimed to compare the frequency of two polymorphisms in the                Additional clinical studies with larger samples of patients are required to
renin angiotensin system (RAS) genes in childhood FSGS with that in               confirm these observations.
healthy controls and with minimal change disease (MCNS). We also eval-
uated the correlation between ACE polymorphism and the course of FSGS
according to declining renal function. The I/D polymorphism of the ACE             M804 A DECREASE OF THE PERMEABILITY OF THE
gene and the A1166C polymorphism of angiotensin II type 1 receptor                          GLOMERULAR BARRIER RATHER THAN HYPOVOLEMIA
(AT1) gene were identified in 28 boys and 25 girls (mean age 3.9± 3.5                        CAUSES ACUTE RENAL FAILURE IN THE NEPHROTIC
years). The mean age of the patients with FSGS (n: 32) and MCNS (n:21)                      SYNDROME
were 4.1± 3.3 and 3.6± 3.9 years, respectively. The ACE genotypes among
                                                                                  Johan Vande Walle1 , Reiner Mauel1 , Ann Raes1 , Joke Dehoorne1 ,
FSGS patients were DD 31.3%, DI 56.3% and II 12.5%. The genotype
                                                                                  Kristof Vandekerckhove1 , Raymond Donckerwolcke2 . 1 Pediatric
distributions in the ACE gene was not different than the MCNS patients
                                                                                  Nephrology, UZGent, Gent, Belgium; 2 Pediatrics, AZ Maastricht,
nor healthy controls (p>0.05). However, the frequency of DD genotype
                                                                                  Maastricht, Netherlands
tended to be higher in FSGS as compared to MCNS and controls (19% and
22%, respectively). The AT1 genotypes among the FSGS patients were                Acute Renal Failure (ARF) in idiopathic Nephrotic syndrome (with Min-
AA 50%, AC 46.9% and CC 3.1%. The genotype distributions were not                 imal Lesions) is according to the classical theory attributed to a hypo-
different than that in MCNS and controls (p>0.05). We have not found              volemia, secundary to the hypo-albuminemia. If ARF is secondary to a
any correlations with these polymorphisms and gender, age onset, blood            hypovolemia, we expect a decreased RPF, an increased filtration fraction
pressure. Although we have not been able to shown any correlation be-             (FF), a negative correlation between colloid osmotique pressure (COP)
tween ACE polymorphism and impaired renal function at early evaluation,           and FF, a positive correlation between vaso-active hormones and FF and
a correlation may become evident at follow-up of these patients. Further-         an amelioration of GFR par albumin perfusion.
more, we were unable to find an association again, when the patients were          The aim of the study was to document this theoretical considerations in
grouped according to early onset (<2 year) and older. Polymorphisms in            patients with Minimal Lesions nephrotic syndrome with and without acute




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the RAS gene polymorphisms were not risk factors for the development of           renal failure.
FSGS in this single-center study group. Larger groups are needed to reach         Methods: clearance studies with inuline, creatinin and PAH (continuous
conclusions.                                                                      flow)
                                                                                  Results: 36 patients with Minimal Lesions NS and creatinine < 1
                                                                                  mg% were studied: we observed a decreased FF (16±5%), a nor-
 M803 POLYMORPHISMS OF THE RENIN-ANGIOTENSIN SYSTEM                               mal GFR (118±31 ml/min/1.73m2 ) but a rather high RPF (740±257
           (RAS) GENES IN CHILDREN WITH STEROID DEPENDENT                         ml/min/1.73m2 ), a positive correlation between colloid osmotique pres-
           AND STEROID RESPONSIVE NEPHROTIC SYNDROME                              sure (COP) and FF (p<0.01) and a positive correlation between vaso-active
                                                                                  hormones and FF (p<0.01).
Ludmila Podracká1 , J. Salagovic2 , T. Baltesova1 , J. Zidzik2 , B. Fleisher2 ,
                                                                                  In 10 patients with serum creatinine between 1.5 and 5 mg% renal function
A. Boor1 . 1 Department of Pediatrics, Medical School, Safarik University,
                                                                                  was studied during basal conditions and during an albumin perfusion: we
Kosice, Slovak Republic; 2 Department of Biology, Medical School,
                                                                                  observed an extremely low filtration fraction, (<10%) in presence of a
Safarik university, Kosice, Slovak Republic
                                                                                  normal or only slightly decreased RPF despite high renin-aldosteron-levels
Polymorphism of the genes of the RAS (including the I/D polymorphism              and low colloid osmotic pressure. Intraveneous albumin-infusion did not
of the angiotensin-converting enzyme gene, ACE I/D, the M235T poly-               ameliorate GFR.
morphism of the angiotensinogen gene, AGT as well as the A1166C poly-             Conclusion: All these observations do not fit with the hypovolemia theory.
morphism of the type 1 angiotensin II receptor gene, AT1) have been               Hypovolemia would stimulate vaso-active hormones, leading to increased
implicated in the progression of various renal diseases. However, there are       filtration fraction, and albumine-perfusion should increase GFR. The ex-
not enough data on the possible relationship between RAS gene polymor-            tremely low filtration fraction in presence of rather normal renal plasma
phisms and the response to steroid treatment in children with nephrotic           flow, even in patients with acute renal failure can only be explained by
syndrome (NS). The purpose of this study was to evaluate the impact of the        a decreased permeability of the basal membrane, possibly related to the
ACE I/D, AGT M235T and AT1 A1166C polymorphisms on the response                   fusion of the podocytes.
to steroid treatment in children with NS.
35 children (17 girls, 18 boys, mean age 12.5 years) with nephrotic syn-
drome followed-up over a 10 years period were enrolled into the study. The         M805 CHILDHOOD THIN GBM DISEASE: REVIEW OF 22
patients were divided into two groups: Group 1 consisted of 10 children                     CHILDREN WITH FAMILY STUDIES AND LONG-TERM
with steroid-resistant NS, while Group 2 was made up by 25 children with                    FOLLOW UP
steroid-dependent NS. Renal biopsy was performed in all patients. Histo-
                                                                                  Carla Carasi1 , William G. van’t Hoff1 , Lesley Rees1 , R. Antony Risdon2 ,
logical findings Group 1 revealed focal segmental glomerulosclerosis in 5,
                                                                                  Richard S. Trompeter1 , Michael J. Dillon1 . 1 Department of Nephrology,
membranous nephropathy in 3 and minimal change disease (MCD) in 2
                                                                                  Great Ormond Street Hospital for Children NHS Trust, London, United
children, while MCD has been found in all 25 subjects belonging to Group
                                                                                  Kingdom; 2 Department of Histopathology, Great Ormond Street Hospital
2. The ACE I/D, the AGT M235T as well as the AT1 A1166C polymor-
                                                                                  for Children NHS Trust, London, United Kingdom
phisms were evaluated in all subjects by polymerase chain reaction.
The frequency of DD genotypes of ACE polymorphism was found to have               Thin glomerular basement membrane (GBM) disease, also called familial
increased in children with resistence to steroids in comparison to the second     basement membrane nephropathy or familial benign hematuria is generally
group (30% vs. 13%). In this group of patients, we also observed increased        known to have a good renal prognosis. However, renal failure has been
value of proteinuria (9,0 vs. 7,5g/d) and significantly (p=0,04) increased         occasionally reported in adults and the overlap with Alport’s syndrome
serum creatinine levels (133,6 vs. 62,9umol/l) in comparison to group 2. In       implies that a good prognosis cannot be guaranteed.
Group 1, the presence of the D allele seemed to be associated with progres-       In order to shed light on long term prognosis of this condition we have eval-
sion of nephropathy (three subjects had D/D and three had I/D genotype),          uated retrospectively 22 children (12 M, 10 F) with persistent hematuria
while the I/I genotype was present in all patients without progression of the     and biopsy proven thin GBM. Mean follow up was 7 years (range 2-17),
renal disease. Neither the AGT M235T nor the AT1 A1166C polymorphism              mean age at onset 7 years (range 1.5-15). The biopsy was performed,
appeared to have any significant influence on the steroid responsiveness of         when non-glomerular causes were excluded, a mean of 3.8 years after on-
NS, the level of proteinuria and/or serum creatinine.                             set. Indications for biopsy were: microscopic hematuria for more than 2
Our preliminary results indicate an association of the ACE I/D polymor-           years, hematuria with proteinuria, recurrence of macroscopic hematuria,
phism with steroid responsiveness in children with NS. Moreover, the ACE          family history of renal failure and/or neural deafness. Parameters evalu-
D/D genotype was linked also to the progression of the disease. On the con-       ated were: hematuria, proteinuria, renal function, family history, audiologic
trary, this study could not show any significant influence of the other two         and ophthalmologic evaluations. The reason for the first investigation was
polymorphisms on the response to steroid treatment in children with NS.           macroscopic hematuria in 10 patients (45,4%), urinary infections or urinary
Monday, June 9                                                E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                  259

symptoms in 5 (22,7%), school screening in 3 (13,6%), incidental finding         tion of CMT and FSGS forms a distinct entity. CMT-patients presenting in
in 2 (9%), family history in 1 (4,5%), abdominal pain in 1. Proteinuria was     the first decade of life should have screening for proteinuria.
present in 13 patients (59%) at onset. Renal function at onset was normal
in all but one patient (acute renal failure due to post infective nephritis).
Ophthalmologic evaluation was normal in all, while audiologic evaluation         M807 URINARY GLYCOSAMINOGLYCAN EXCRETION IN
was normal in all but one, who had minimal sensory-neural hearing loss.                   CHILDREN WITH NEPHROTIC SYNDROME
Light microscopy and immunofluorescence findings were essentially unre-
                                                                                Nurcan Cengiz1 , Aysun Karabay Bayazit2 , Aytul Noyan2 , Yasemin Uslu1 ,
markable in all, while electron microscopy showed thinning of the GBM
                                                                                Mithat Buyukcelik2 , Hasan Dursun2 , Ali Anarat1 . 1 Pediatric Nephrology,
but no characteristics of Alport’s syndrome. Family history showed iso-
                                                                                Baskent Univ., Sch. of Medicine, Adana Hospital, 2 Pediatric Nephrology,
lated hematuria in 8 (36,3%), hematuria with renal failure or deafness in
                                                                                Cukurova Univ., Sch. of Medicine, Balcali, Adana, Turkey
9 (40,9%). The mode of inheritance appeared to be autosomal dominant
in most of these families, except for 2 in which could be either autosomal      Glycosaminoglycans (GAG) are present within the glomerular basement
dominant or X-linked. 4 patients (18%) showed a decline of renal function       membrane. Elevated urine GAG has been suggested as a marker of
after 8, 6, 9 and 12 years of follow up respectively and one of these also      glomerulonephritis. In this study, we evaluated urine GAG excretion in
developed hearing impairment. None developed hypertension. All but one          children with nephrotic syndrome. We measured the concentration of urine
of these 4 had proteinuria since the onset.                                     GAGs by the modified dimethylmethylen blue method. Thirty-seven chil-
In contrast to previous reports, our findings suggest that the long term         dren with steroid sensitive nephrotic syndrome (SSNS), 21 children with
prognosis in children might not be always benign especially if there is a       steroid resistant nephrotic syndrome (SRSN) and 25 healthy control subject
family history of renal impairment or deafness. Both thin GBM disease and       were included in the study. The urine GAG/Cr ratios in patients with SRNS
Alport’s syndrome appear to result from defects in collagen type IV coding      (mean ± SD, 109.18 ±78.46) and in nephrotic period of SSNS (mean ±
genes and may represent two extremes of the spectrum of collagen type IV        SD, 125.74± 101.10) were significntly higher than in the control subjects
gene disorders. A detailed family history is essential in these children and    (mean ± SD, 33,36±23,99) . In the nephrotic period of SSNS, patients




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we strongly suggest a careful follow up over a protracted period because        excreted significantly more urinary GAG than in the remission. No corre-
of the risk of late onset renal failure. Further studies are needed with more   lation was found between the urinary GAG/Cr and urinary protein/Cr. We
children and longer follow up plus genetic analysis to clarify these issues.    concluded that the loss of GAGs from the glomerular basemant membrane
                                                                                may be related to the pathogenesis of the nephrotic syndrome.

 M806 CHARCOT-MARIE-TOOTH DISEASE AND FOCAL
          SEGMENTAL GLOMERULOSCLEROSIS                                           M808     SPECIFIC IGE AND TREATMENT AT FREQUENT
                    1                            2                    3                   RELAPSING AND STEROID-DEPENDENT MINIMAL
Christoph J. Mache , Michaela Auer-Grumbach , Afschin Soleiman ,
                                                                                          CHANGE NEPHROTIC SYNDROME (MCNS) IN CHILDREN
Christian Windpassinger2 , Klaus Wagner2 , Ekkehard Ring1 . 1 Pediatric
Nephrology, University Children’s Hospital, Graz, Austria; 2 Institute of       Nadezhda D. Savenkova, Irina V. Batrakova, Karina A. Papayan, Natalya
Medical Biology and Human Genetics, Karl-Franzens-University, Graz,             A. Lisovaya. Department of Pediatric Nephrology, St.-Peterburg Pediatric
Austria; 3 Institute of Clinical Pathology, University Medical School,          Medical Academy, St.-Petrburg, Russian Federation
Vienna, Austria
                                                                                ISKDC, APN (1981-1991) recommend standardized treatment of MCNS
Charcot-Marie-Tooth disease (CMT) comprises a clinically and geneti-            in children. The aim of our research was to assess the efficiency of treatment
cally heterogeneous group of disorders affecting the peripheral nervous         and to determine specific IgE to allergens in blood serum.
system. So far 18 patients with the combination of CMT and focal seg-           Among 210 patient with MCNS 100% had initial corticosteroid sensitive-
mental glomerulosclerosis (FSGS) have been reported in the literature. We       ness, 160 (76,2%) of this patients had subsequent relapses or frequent re-
report two additional unrelated patients with this rare association.            lapses, 50 patients (23,8%) had the long-term-remission without relapses.
The male patient with early childhood-onset of CMT and deafness pre-            Steroid dependence and resistance in 70 children with MCNS were associ-
sented with hypertension, proteinuria, and chronic renal failure (CRF) at       ated with atopic in 60% of cases. Increasing in blood specific IgE to alimen-
16 years. CRF rapidly progressed to end-stage renal failure at 17 years.        tary, household allergens and allergens of grasses and trees had 42 (60%)
The female patient developed symptoms of CMT during primary school.             children with frequent relapsing steroid dependent MCNS. The alkylating
At 28 years renal disease with proteinuria and CRF was detected. Her re-        agents (chlorambucil in the dose 0,15-0,3 mg/kg/day) have been used in
nal function further deteriorated during the following year. At diagnosis       70 patients with steroid dependence, steroid resistance and steroid toxicity
of renal disease, both patients had a renal biopsy with histological exam-      during 2-3 months and in some cases 6 months. At 3 of such patients hav-
ination including electron microscopy. Electrophysiological studies of pe-      ing frequent and steroid dependent relapses after Chlorambucil was used
ripheral neuropathy and acoustic evoked potential studies were performed.       Cyclosporine A (Neoral sandimmun) in the dose 2,0-2,5 mg/kg/day during
Molecular genetic analyses included a search for intrachromosomal du-           3-6 months. 25 (35%) of cytostatic treated patients with increased specific
plication/deletion of the peripheral myelin protein 22 (PMP22) gene as          IgE in blood subsequently (during 2 years) had relapses.
well as sequencing of the genes encoding for PMP22, myelin protein zero         Children with increased IgE in blood have frequent relapses and steroid
(MPZ), and connexin 32 (Cx32).                                                  dependence after cytostatic therapy. Therefore management of MCNS with
Renal histologies were consistent with FSGS. On electron microscopy,            increased specific IgE in blood in children should be differentiated: diet,
both renal biopsy specimens showed mesangial sclerosis, irregularities of       regimen, exception of contact with allergens and standard glucocorticoid
the glomerular basement membrane with focal dissociation of the lam-            therapy relapses, under the indications – cytostatic therapy.
ina rara interna, and partial flattening of the podocyte foot processes. In
both patients the electrophysiological findings were compatible with a de-
myelinating motor and sensory neuropathy in the upper and lower limbs.           M809 GROWING INCIDENCE OF FOCAL AND SEGMENTARY
Acoustic evoked potentials revealed marked slowing in the male patient,                   GLOMERULOSCLEROSIS AS A CAUSE OF
but were within the upper limit of normal in the female patient. In both pa-              STEROID-RESISTANT NEPHROTIC SYNDROME IN
tients molecular genetic analyses excluded altered gene dosages affecting                 RUSSIAN CHILDREN
the PMP22 gene as well as mutations in the genes encoding for PMP22,
                                                                                Alexey Tsygin1 , Tamara Sergeeva1 , Tatiana Voznesenskaya1 ,
MPZ, or Cx32.
                                                                                Tatiana Pankratenko1 , Vladimir Varshavsky2 , Anna Timofeeva1 .
The clinical presentation of our patients was characterized by early-onset      1
                                                                                  Nephrology, Institute of Pediatrics, RCCH RAMS, Moscow, Russian
symptoms of a demyelinating peripheral neuropathy and the delayed and
                                                                                Federation; 2 Pathology, Moscow Medical Academy, Moscow, Russian
insidious onset of FSGS with proteinuria and rapidly progressive CRF. The
                                                                                Federation
ultrastructural appearances of FSGS were quite comparable and had been
observed previously as well. The similarities to other published cases and      Our results are based upon the single reference center analysis of percu-
the exclusion of frequent CMT-causing mutations suggest that the associa-       taneous guided kidney biopsies performed for 55 children from Central
260      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                        Monday, June 9

Russia admitted during the last 4 years with a history of steroid resistant      Among the 252 patients who suffered from PNS and followed up by our
nephrotic syndrome (39) or untreated asymptomatic proteinuria more than          clinic between January 1980 and May 2001, 159 fulfilled the criteria to be
2.0 g/m2 /24h (16). Light, immunofluorescent and electronic microscopy            included in the study, which were: initial diagnosis and treatment by our
were used in all cases.                                                          department, age older than 1 year at the time of diagnosis and follow-up
The predominant pathology finding appeared to be a focal and segmentary           period at least 6 months. Demographic features, clinical and laboratory
glomerulosclerosis (FSGS)- 26 cases (47,3%). That included 3 cases of            findings, response to treatment and rates of relapses as well as histopatho-
familial nature. Mesangial proliferation was found in 9 (16,3%), minimal         logical findings in these patients were noted.
change glomerulopathy in 5 (9,1%), mesangiocapillary glomerulonephritis          Of the 159 children (104 males) mean age at the time of first admission was
(MCGN) in 4 (7,3%). Membranous nephropathy was demonstrated in 6                 3.9±2.7 years. Follow-up period ranged from 6 months to 16.4 years. In
(10,9%) children being HBv-associated in 3 and a feature of lupus nephritis      addition to the findings of nephrotic syndrome, systolic (16%) and diastolic
in 1 child. Each of acute proliferative GN, sclerosing GN, renal amyloidosis     (26%) hypertension, oliguria (9%), haematuria (25%) and increased serum
due to Familial Mediterranean fever, extracapillary GN and type VI lupus         creatinine (19%) were noted during the initial examination. 149 (93.8%) of
nephritis were found in 1 (1,8%) case.                                           the patients responded to standard steroid regimen. Among the 123 patients
Comparing to the years 1975-1995, we observe a marked increase in preva-         who had been followed longer than 2 years, 24% and 60% were character-
lence of FSGS as a cause of childhood steroid-resistant nephrotic syndrome       ized by no relapses at all or infrequent relapses, respectively; while 16%
from 18,7% to 47,3% nowadays in Central Russia whereas a frequency of            suffered from frequent relapses or were steroid dependent. When the pa-
MCGN decreased significantly. The similar was reported in several other           tients were classified according to the first admission date either before
countries. The reason of that growth may have genetic and/or environmen-         or after 1990, there was not any significant difference considering steroid
tal basis but it still has to be defined.                                         sensitivity or frequency of relapses. Histopathological diagnoses were as
                                                                                 follows in 26 patients who underwent kidney biopsies: MCD: 7, diffuse
                                                                                 mesangial proliferation: 17, FSGS: 2.
 M810 AMILORIDE POTENTIATES EDEMA REMOVAL BY                                     These findings suggest that clinical course and response to steroid treatment




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           FUROSEMIDE IN NEPHROTIC CHILDREN                                      is similar with historical cases and frequency of FSGS has not increased
                                                                                 during the recent years in Turkish children with PNS.
Vincent Guigonis1 , Sylvie Nathanson1 , Alain Doucet2 ,
Georges Deschênes1 . 1 Néphrologie Pédiatrique, Hôpital
Armand-Trousseau, Paris, France; 2 FRE 2468, CNRS, Paris, France
                                                                                  M812 BONE MINERAL DENSITY IN CHILDREN WITH NEPHROTIC
According to the prevention of renal sodium retention and ascites forma-                   SYNDROM TREATED WITH CORTICOSTEROIDS
tion by amiloride at the early phase of PAN nephrosis, the aim of this
                                                                                 Mojgan Mazaheri, Taher Isfahani, Abbas Madani, Hasan Moradinejad,
study was to evaluate the effect of amiloride in nephrotic children with
                                                                                 Parvin Mohseni. Nephrology, Children Medical Center Hospital, Tehran,
edema. Thirteen patients, 6 girls and 7 boys aged 4.5-19 years (m = 11.5),
                                                                                 Iran
with nephrotic syndrome and severe edema were enroled in 2 groups (A
and B) after a control period. A low-sodium diet (0.5 to 0.8 mmol/kg/day)        Aim of the present research was evaluation of bone mineral density (BMD)
was given to all patients. Proteinuria remained unchanged throughout the         & risk of osteoporosis in 39 children (30 male & 9 female) aged 5-17yrs
study in both group. In group A (n = 6), urine Na/creatinine significantly        (mean age:10.02±3.3yr)with nephrotic syndrom who recieved corticos-
increased from 1.2 ± 1.1 before treatment to 9.7 ± 8.0 mmol Na/mmol              teroids.We used medilink standard for sex- matched&agematched con-
creatinine with amiloride alone (p<0.05), and to 21.9 ± 13.3 mmol/mmol           trols. The patients were divided in two groups on the basis of duration
when furosemide was associated (p<0.01). The mean body weight de-                of treatment (more than 2.5yr & less than 2.5 yr).They had normal re-
creased from 65.1 ± 28.9 kg to 64.4 ± 28.2 kg (p<0.05) after 2 days              nal function.we excluded children with immobilization,history of fracture
of treatment with amiloride, and to 62.4 ± 28.7 after 2 days of treatment        or chronic disease. None of them had recieved any drugs for osteoporo-
with the amiloride-furosemide association. Results obtained with sodium          sis.Mesurement of BMD was performed by dual energy Xray absorptiom-
balance were similar. In group B, urine Na/creatinine rose from 1.7 ± 1.3        etry (DXA)at lumbar spine (L1 -L4 ),femoral neck,&distal end of radius.
(basal conditions) to 8.1 ± 5.3 mmol/mmol with furosemide (p<0.05),              Results were expressed as grams hydroxylapatite divided by the projected
and to 15.4 ± 7.7 mmol/mmol (p<0.05 compared to furosemide period)               area in square centimeters (g/cm2 ) and as Z score for determination of
when amiloride was associated with furosemide. The mean body weight              osteoporosis risk.BMD in 86.4% of patients at lumbar spine (mean BMD
decreased from 50.5 ± 22.3 kg to 49.4 ± 22.3 kg (NS) after 2 days of treat-      0.68±0.11g/cm2 ),80.1% at femoral neck (mean BMD 0.8±0.09)&100%
ment with furosemide alone, and to 47.5 ± 21.8 kg (p<0.01 compared to            at distal radius (mean BMD 0.21±0.07g/cm2 )were lower than nor-
the end of the furosemide period) after 2 days of the furosemide-amiloride       mal.Lumbar spine Zscore in 64.8% of patients & femoral neck Zscore
association. Results of sodium balance were similar. Patients had no edema       in 18.1% & distal radius Zscore in 100% of patients was lower than
at the end of 12/13 furosemide-amiloride courses. The mean final weight           -2,meaning osteoporosis risk is 4 times of normal children.There was
loss was 6 ± 2.9 kg (n = 13) in a mean delay of 8.2 ± 3.3 days. No adverse       no relation between cummulative steroid dose & densitometric determi-
effect was noted except cramps in 2 patients.                                    nants.There was positive relation between age at the begining of disease &
In conclusion, this study demonstrated that amiloride enhances urinary           distal radius BMD (P<0.04).There was inverse relation between duration
sodium excretion improves furosemide potency and allows edema removal            of steroid treatment & distal radius Zscore (P<0.004).
in nephrotic patients.                                                           We concluded that all of patients had abnormal bone density at least in one
                                                                                 region.Distal end of radius earlier & also more than other region showed
                                                                                 decreasing bone density.So we can use radius densitometry as the first
 M811 HAVE THE CLINICAL COURSE AND RESPONSE TO                                   screening test to diagnose osteoporosis. We recommend prphylactic regi-
           STEROID TREATMENT CHANGED IN CHILDHOOD                                men for prevention of osteoporosis in these patients.
           PRIMARY NEPHROTIC SYNDROME?
Ece Sule Aslan, Lale Sever, Salim Caliskan, Ozgur Kasapcopur,
                                                                                  M813 ANTIPROTEINURIC AND RENOPROTECTIVE EFFECTS OF
Nil Arisoy. Paediatric Nephrology, University of Istanbul, Cerrahpasa
                                                                                           COMBINATION THERAPY WITH AN
School of Med., Istanbul, Turkey
                                                                                           ANGIOTENSIN-CONVERTING ENZYME INHIBITOR (ACEI)
It has been well defined that minimal change disease (MCD) is responsi-                     AND AN ANGIOTENSIN RECEPTOR BLOCKER (ARB) FOR
ble from 85% of childhood primary nephrotic syndrome (PNS) and almost                      CHILD PATIENTS
90% improve with standard steroid treatment. On the other hand, in recent
                                                                                 Mitsuru Okada, Kazuro Yagi, Hidehiko Yanagida, Tsukasa Takemura.
years many studies indicate that the incidence of focal segmental glomeru-
                                                                                 Department of Pediatrics, Kinki University School of Medicine,
losclerosis (FSGS) has increased both in adult and paediatric patients. This
                                                                                 Osaka-Sayama, Ohno-Higashi, Japan
study was conducted in order to investigate the clinical features, histopatho-
logical findings and response to steroid treatment in PNS patients.               (Purpose of the study) Previous studies have concluded that angiotensin-II
Monday, June 9                                               E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                   261

(Ang-II) play an important role in progressive renal diseases through al-       M815 MULTICENTER TRIAL OF MYCOPHENOLATE MOFETIL
teration of glomerular hemodynamics and direct stimulation of glomerular                 (MMF) IN CHILDREN WITH STEROID DEPENDENT (SD) OR
cell proliferation, thus administration of ACEI is an effective treatment                FREQUENT RELAPSING (FR) NEPHROTIC SYNDROME
for preventing glomerular injuries. However, ACEI may not completely                     (NS). REPORT OF THE SOUTHWEST PEDIATRIC
suppress the synthesis of Ang-II, because Ang-II can be produced via                     NEPHROLOGY STUDY GROUP
non-ACE dependent pathways, such as chymase. Recent studies have sug-
                                                                               Ronald Hogg1 , Lisa Fitzgibbons1 , Joy Bruick1 , Martin Bunke2 ,
gested that combination therapy with ACEI and ARB may be effective
                                                                               Bettina Ault3 , Noosha Baqi4 , Howard Trachtman5 , Rita Swinford6 .
for preventing progression of chronic glomerular diseases in adult series,     1
                                                                                 Clinical Research, Medical City Dallas Hospital, Dallas, TX, United
while evidence for the efficacy of ACEI for treatment of chronic glomeru-
                                                                               States; 2 Medical Transplant Liaison Medical Affairs, Roche Laboratories,
lar diseases in pediatric patients is not available yet. Thus, the present
                                                                               Nutley, NJ, United States; 3 Pediatric Nephrology, University of Tennesee,
study was undertaken to evaluate whether this combination therapy pro-
                                                                               Memphis, TN, United States; 4 Pediatric Nephrology, SUNY Brooklyn,
vides an additive antiproteinuric effect and renoprotection in children with
                                                                               Brooklyn, NY, United States; 5 Pediatric Nephrology, Schneider Children’s
chronic glomerulonephritis. (Methods) A total of 20 patients were en-
                                                                               Hospital, New Hyde Park, NY, United States; 6 Pediatric Nephrology, UT
rolled in this study (male: 12, female: 8, mean age: 15.4±2.7 y, mean
                                                                               Houston, Houston, TX, United States
duration of illness: 7.5±1.3 y). Criteria for patient selection were biopsy-
proven mesangial proliferative glomerulonephritis (IgA-positive: n=10,         Children with SDNS and FRNS often develop adverse effects (AEs) from
IgA-negative: n=5) and other chronic glomerular diseases (FSGS: n=3,           prednisone (P). Attempts to induce long-term remission in such patients
Henoch-Schonlein purpura nephritis: n=1, Immunotactoid glomerulopa-            (pts) with cytotoxic agents (e.g., cyclophosphamide) and calcineurin in-
thy: n=1) with normal blood pressure. All patients showed non-nephrotic        hibitors (e.g., cyclosporin A) have had varying levels of success. Concerns
proteinuria ranging from 0.57 to 2.5 g/d (0.82±0.20 g/d) with normal renal     about long-term AEs with these agents prompted us to evaluate MMF in pts
function (Ccr>70 ml/min). ACEI (enalapril: 5-10 mg/day) was given for          <21 years with SDNS and FRNS. In this multicenter, prospective study,
more than 12 weeks before administration of ARB (losartan: 25-50 mg/d).        14 centers enrolled 33 pts who were in remission at entry, fulfilled standard




                                                                                                                                                                Downloaded from http://ndt.oxfordjournals.org/ by guest on May 1, 2013
Combination therapy was maintained for 1.5 years and more. (Results)           criteria for SDNS or FRNS, and had not received CsA, azathioprine, lev-
Combination therapy achieved significant reduction of proteinuria from          amisole, FK506, or MMF. The pts received MMF in a liquid formulation
the basal value of 0.82±0.20 to 0.32±0.40 g/d 1 to 3 months after combi-       (provided by Roche Labs) at a dose of 600mg/m2 BID for 24 weeks. It was
nation therapy was started (p<0.05). Systolic and diastolic blood pressures    then tapered over 4 weeks and discontinued. A tapering dose of alternate
were unchanged throughout the study (pre: 115±14/66.7±8.3 mmHg, af-            day P was given during the first 16 weeks of MMF therapy. Pts were mon-
ter: 109±13/60.9±7.2 mmHg), as were creatinine clearance, hematocrit,          itored for up to 6 months post MMF therapy. Urine protein was monitored
and serum concentrations of protein and potassium. Serum concentration         at home by dipstick. Treatment failure was defined as a relapse of NS, i.e.,
of uric acid tended to decrease from 6.2±1.13 to 4.77±1.39 mg/dL after         presence of edema, or ≥2+ proteinuria for ≥3 days, plus central lab urine
administration of ARB. Adverse effects including mild fainting, headache       protein/creatinine ratio ≥1.0 on first morning urine or serum albumin <3.0
and oropharyngial aphtoid ulcer were noticed in four patients, but these       g/dl. If a pt had proteinuria at home but central lab studies did not confirm
symptoms were temporary and disappeared after reduction of the dosage          relapse, the pt continued MMF. One pt was excluded following a protocol
of ARB. (Summary) The finding in this study indicate that combination           violation after 2 days of therapy. The other 32 pts had the following features
therapy with ACEI and ARB is the safe and has both additive antiprotein-       at the time of study entry: Age: = 6.8 ± 2.7 years; Range = 2-15 years; 56%
uric effect and renoprotective effect for children with chronic glomelular     Male; 44% Female: Race/ethnicity = 50% White; 25% African American;
diseases.                                                                      25% Other. Entry Classification: 81% FRNS; 19% SDNS. Number of Pre-
                                                                               Entry Relapses: 4.3 ± 2.3 per year. Estimated GFR at entry was 138 ±
                                                                               42 ml/min/1.73m2 . All pts had a normal serum albumin at entry. Although
 M814 SWITCH FROM CYCLOSPORINE A (CyA) TO                                      adverse events were uncommon, one pt stopped MMF because of an ab-
          MYCOPHENOLATE MOFETIL IMPROVES KIDNEY                                solute neutrophil count <1000/m3 . 24 of 32 pts (75%) stayed in remission
          FUNCTION IN CHILDREN WITH NEPHROTIC SYNDROME                         throughout the 6 months of MMF therapy. 10 of these pts stayed in re-
          AND CyA-INDUCED NEPHROTOXICITY                                       mission during the entire 6 months post MMF period, whereas 12 relapsed
                                                                               after stopping MMF; 2 are still in the study. 8 of 32 pts (25%) relapsed
Tim Ulinski, Bruno Ranchin, Marie-Helene Said, Bernadette Parchoux,
                                                                               while on MMF (5 relapsed while on both P and MMF during the first 16
Pierre Cochat. Dept. of Pediatrics, Hopital Edouard-Herriot, Lyon, France
                                                                               weeks of study, the other 3 relapsed while on MMF alone, during the next
Nephrotoxicity is a well known adverse effect of cyclosporine A (CyA)          12 weeks of study.) We conclude from this study that MMF is an effective
treatment in children with steroid-dependent (SD) and steroid-resistant        agent for maintaining remission in NS patients who receive treatment for
(SR) nephrotic syndrome (NS).                                                  at least 6 months and can effectively decrease the AEs that occur with P in
We analysed 7 children (age: 3.3 to 15.7 years, 2 girls) with SD or SR NS      such patients. Long-term studies of MMF in pts with FRNS or SDNS are
who experienced a significant decrease of their GFR under CyA treatment         warranted.
estimated from Schwartz formula. Mycophenolate mofetil (MMF) was                                                              Free Communication June 12
introduced progressively until usual doses of 1g/1.73m2 twice daily. CyA
treatment was stopped after introduction of MMF and oral prednisone was
reduced if possible. After a mean follow-up period of 261±183 days no           M816 SINGLE CENTER EXPERIENCE WITH CYCLOSPORINE IN
adverse effects of MMF such as diarrhoea or haematological anomalies                     106 CHILDREN WITH IDIOPATHIC FOCAL SEGMENTAL
occurred in our patients. After switch from CyA to MMF those children                    GLOMERULOSCLEROSIS
with SD NS remained in remission with negative proteinuria and those with
                                                                               Ihab M. Mahmoud, Fathy M. El-Bassuony, Amr A. El-Huseiny, Nabeel
SR NS did not present any significant changes of their residual proteinuria.
                                                                               A. Hasan, Mohamed A. Sobh. Nephrology, Urology and Nephrology
Proteinemia did not change significantly for any of the analysed children.
                                                                               Center, Mansoura, Dakahlia, Egypt
GFR increased from 76.3±26.0 to 112.2±10.0 mL/1.73m2 per min (P =
0.005). Oral steroid treatment could be reduced from a median [range]          We retrospectively reviewed the data of 106 nephrotic children due to
prednisone dose of 0.625 [0.26-2.94] pre-MMF to 0.125 [0.0-1.1] mg/kg          primary focal segmental glomerulosclerosis (FSGS) who received cy-
per day (P = 0.026) after reaching stable MMF daily dosage. Arterial blood     closporine (CsA) between 1993 and 2002. Indications of CsA therapy were
pressure decreased moderately after CyA withdrawal; but the difference         steroid resistance (n=45) and –dependence with steroid toxicity (n=61).
did not reach statistical significance.                                         Fifty four patients received cyclophosphamide prior to CsA therapy. CsA
We conclude that a switch from CyA to MMF seems to be safe for children        starting dose was 6 mg/kg/day to be readjusted to maintain a whole blood
with SD or SR NS regarding side effects as well as disease control, at least   trough level of 80-150 ng/ml (TDX). The drug was received for 6-48
in the short term. Interruption of CyA treatment leads to rapid ameliora-      (22.1±11) months. The observation period was 5.8±3 years and 6.1±1.9
tion of kidney function in these children. Prospective controlled long term    years before and after CsA treatment respectively. Complete remission,
studies are needed to evaluate the role of MMF in such glomerular diseases.    partial remission and resistance to CsA were observed in 71.7%, 7.5% and
262      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                        Monday, June 9

20.8%, respectively. CsA-sensitive and –resistant patients differed only in     Results: The mean CsA dose was 4.3 mg/kg/day (range 2.8-5.1) and mean
the percentage of steroid responsiveness being 66.7% in the former group        CsA plasma concentration was 280.7 ng/ml (range 197-346). No chil-
and 22.7% in the latter, p<0.0001. Logistic regression analysis identified       dren had significant increase in serum creatinine or worsening creatinine
steroid resistance as the only predictor of resistance to CsA (odds ratio       clearance during CsA treatment. NS relapses were significantly decreased
12.9, p=0.03). Hypertension, renal impairment (> 30% rise of serum cre-         with CsA treatment (4.6/yr versus 1/yr). Glomerular pathology showed 15
atinine), gingival hyperplasia and hypertrichosis occurred in 12.3%, 6.6%,      minimal change lesions, 3 cases of focal segmental glomerulosclerosis.
22.6% and 51.9% respectively. With the exception of hypertrichosis, side        Minimal to mild chronic tubulo-interstitial (CT I) lesions were present in
effects were significantly more frequent among CsA-resistant children. We        11 children. To asses CsA toxicity, isometric tubular vacuolization, some
were able to stop steroids in 91 patients, of whom 31 patients relapsed. Out    minor vascular changes (vacuolization of smooth muscle cells, individ-
of 20 patients for whom CsA was intentionally discontinued while in re-         ual muscle-cell necrosis), typical CsA-associated arteriolopathy (circular
mission, 16 patients relapsed. Of these, four patients (25%) were resistant     nodular hyalinosis of the media) and stripped interstitial fibrosis were eval-
to a second course of CsA. At the last follow up, one child had developed       uated. Clear-cut lesions diagnostic of CsA toxicity were never found; in 5
end-stage renal failure and 3 had chronic renal insufficiency. In conclu-        cases, changes suggestive of CsAN (2 pts) or consistent with CsAN (3pts)
sion, CsA is effective in the treatment of children with idiopathic FSGS        were observed.
but with a high relapse rate. Renal dysfunction and hypertension, which
may be drug-induced or natural disease progression, are the most serious        Grading CTI lesions            No patients         CsA toxicity (No patients)
complications; therefore, a close monitoring is essential.
                                                                                Absent                              7                            0
                                                Free Communication June 9       Minimal (<10%*)                     9             2 (suggestive) + 3 (consistent)
                                                                                Mild (<30%*)                        2                            0
                                                                                Moderate/severe (>30%*)             0                            0
 M817 EFFICACY AND TOLERANCE OF LONG-TERM PULSE                                 * percentage of renal cortex
          METHYLPREDNISOLONE THERAPY IN CHILDREN WITH




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          SEVERE IDIOPATIC NEPHROTIC SYNDROME                                   Conclusions: long-term CsA treatment is confirmed to be effective in pre-
                                                                                venting NS relapses and avoiding steroid toxicity in children with steroid-
Paraschiva Chereches-Panta, Sorin Man, Mirela Popa, Mircea Nanulescu.
                                                                                dependent NS. Renal function is not a reliable indicator of CsAN. Our ex-
IIIrd Pediatric Clinic, University of Medicine, Cluj Napoca, Romania
                                                                                perience suggests that, with doses and plasma concentration of CsA above
Pulse methylprednisolone therapy (PMT) has been described as a success-         mentioned, CsAN has a low incidence and if present is generally mild.
ful treatment regimen in nephrotic syndrome resistant to oral corticos-         Renal biopsy should be performed to monitor patients with long-term CsA
teroids.                                                                        treatment.
We reviewed 7 consecutive steroid-resistant nephrotic syndrome patients
(mean age 11±3.7 years) that received PMT for a duration of 12 to 18
months. All children revealed focal segmental glomerulosclerosis on kid-         M819 REMISSION OF CYCLOSPORIN-STEROID-RESISTANT
ney biopsy. PMT was started after a mean period of clinical course of                       NEPHROTIC SYNDROME USING MULTIPLE
19.6±6.7 months (between 4 and 42 months). All children received oral                       IMMUNOSUPPRESSION
steroids during PMT and in 4 of 7 patients cyclophosphamide was added.
                                                                                Vincent Guigonis1 , Tim Ulinski1 , Françoise Audat2 , François Lefrere2 ,
The mean period of follow-up was of 47.1±24.1 months (between 12 and
                                                                                Philippe Jouvet3 , Albert Bensman1 , Georges Deschênes1 . 1 Pediatric
73 months). Five patients achieved complete and sustained remission after
                                                                                nephrology, Hôpital Trousseau, Paris, France; 2 Hematology, Hôpital
a duration of PMT between 4 and 22 weeks. In 2 patients nephrotic range
                                                                                Necker, Paris, France; 3 Pediatric Intensive Care Unit, Hôpital Necker,
proteinuria persisted. Both of these 2 patients presented high blood pressure
                                                                                Paris, France
and gross haematuria. In these 2 patients PMT was stared at 24 months and
respectively at 42 months after the onset of disease. Renal failure occurred    Nephrotic proteinuria in minimal change disease (MCD) is due to a cir-
in both of these 2 patients, one having ESRD. None of the 7 patients had        culating factor from immunologic source and is usually steroid sensitive.
elevated serum creatinine at the time when PMT was started. Two patients        End stage renal failure frequently occurs when steroids and ciclosporin are
presented transient slight elevation of serum creatinine during PMT. One        not effective. We report here two cases with a successful outcome under an
patient has hypertension and in two patients mild cataracts occurred.           immunosuppressive treatment which has been proposed for MCD relapse
Further studies are necessary to determine the value of PMT for children        after kidney graft.
with severe idiopathic nephrotic syndrome.                                      Case 1: a 2-year old girl presented with nephrotic syndrome in whom pro-
                                                                                teinuria initially resolved under 60 mg/m2 of oral prednisone then relapsed
                                                                                before the decrease of steroids and remained unchanged after 4 weeks of
 M818 LONG-TERM CYCLOSPORINE TREATMENT IN CHILDREN                              oral prednisone (60 mg/m2 ), 3 pulses of methylprednisolone (1 g/1,73 m2 )
          WITH STEROID-DEPENDENT NEPHROTIC SYNDROME:                            and 4 months of high doses of ciclosporin (10 mg/kg/d, T0:140, T2: 1300
          A CLINICAL AND HISTOLOGIC ANALYSIS                                    ng/ml). A kidney biopsy confirmed minimal changes lesions. A treatment
                                                                                strategy derived from the protocol used during relapses of nephrotic syn-
Stefano Rinaldi1 , Antonella Sesto1 , Paola Barsotti2 , Tullio Faraggiana2 ,
                                                                                drome after kidney graft (Cochat, Ped Nephrol, 1993) was undertaken in-
Gianfranco Rizzoni1 . 1 Division of Nephrology, Bambino Gesù Children’s
                                                                                cluding IV ciclosporin (plateau: 500 to 600 ng/ml), 3 pulses of methylpred-
Hospital and Research Institute, Rome, Italy; 2 Department of Pathology,
                                                                                nisolone (250 mg/m2 ) followed by oral prednisone (60 mg/m2 ), cyclophos-
University “La Sapienza”, Rome, Italy
                                                                                phamide (150 mg/kg), pefloxacin (10 mg/kg/d) and plasma exchanges (n
Cyclosporine (CsA) is an effective therapy for children with long last-         = 21; 1.5 plasma volume each). Remission of nephrotic syndrome was ob-
ing nephrotic syndrome (NS). However long-term treatment can result in          tained after the 10th plasma exchange at day 12. Pefloxacin was stopped at
chronic CsA nephropathy (CsAN). For this reason, the clinical and his-          day 17, plasma exchange at day 51, cyclophosphamide at day 86 and pred-
tologic analysis of 18 children (12M/6F) with steroid-dependent (15 pts)        nisone at the 10th month. At 14 months of follow-up, the patient was still
and partially steroid-sensitive (3 pts) NS treated with CsA was reviewed        in remission (proteinuria = 0; serum albumin 33g/L) and a normal serum
retrospectively.                                                                creatinin (50 µmol/L) while receiving ciclosporin 5 mg/kg/d.
Patients and methods: CsA was initiated at mean age of 7.9 years (range         Case 2: a 2-year old boy presented with nephrotic syndrome which was
2.5-14.4) after 3.4 years (range 0.3-12.6) of NS. All patients had previously   initially steroid-dependent. Despite a treatment by oral cyclophosphamide
received treatment with levamisole and/or cytotoxic agents.The following        (130 mg/kg), it turned out steroid-resistant, and no change in proteinuria
parameters were evaluated: serum creatinine, creatinine clearance,steroid       were observed after a 4-month period of ciclosporin (14 mg/kg/d, T0= 190
therapy, number of relapses/year, CsA dosage and mean CsA plasma con-           ng/ml) and a 9-month period of mycophenolate mofetil (1200 mg/m2 /j).
centration obtained from the AUC according to Kahan. A renal biopsy             A similar treatment strategy than reported in case 1 was performed 26
was performed after a mean of 4.1 years (range 2.2- 6.2) after the start of     month after the diagnosis of steroid-resistance. Proteinuria decreased from
CsA.                                                                            more than 50 mg/kg/d to 21 mg/kg/d at day 36 and disappeared at day
Monday, June 9                                                 E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                    263

99. Pefloxacin was stopped at day 46, plasmatic exchanges at day 54 and           A 5 year-old female presented with nephrotic syndrome in December 1998.
cyclophosphamide after a cumulative dose of 50 mg/kg/d. At 6 month of            She was given steroid therapy according to the ISKDC protocol. She was
follow up, the patient was still in remission (proteinuria = 0; serum albu-      corticosensitive but soon became steroid dependent. Renal biopsy was
min 37.4 g/L) and a normal serum creatinin (35 µmol/L) while receiving           performed; the light microscopy and immunofluorescence study revealed
cyclosporin (10mg/kg/d) and prednisone 0.5 mg/kg every other day.                minimal change nephrotic syndrome. She was given cyclophosphamide
Aggressive immunosuppression led to a protracted remission of protein-           2mg/kg/BW for 12 weeks. The effect was short lasting and she became
uria in 2 patients with ciclosporin-steroid resistant idiopathic nephrotic       again steroid dependent. She developed cushingoid aspect, striae and hir-
syndrome.                                                                        suitism. Since July 2002 she has been receiving levamisole 50 mg every
                                                                                 other day. Steroid therapy was tapered and withdrawn. She has been very
                                                                                 stable without edema and no proteinuria. There have been no side effects
 M820 PLASMA EXCHANGE AND IMMUNOSUPPRESSION – AN                                 due to levamisole. Five months after introduction of levamisole, a mild goi-
           INDIVIDUALLY TAILORED APPROACH TO THE                                 ter was found on a systematic examination at school. The ultrasound of the
           TREATMENT OF PRIMARY AND RECURRENT FOCAL                              thyroid revealed non-homogenous structure of the parenchyma. She was
           SEGMENTAL GLOMERULOSCLEROSIS IN CHILDREN                              euthyroid: TSH 1.39 microU/ml (norm. 0.15-4.5), T4 122 nmol/l (norm.64-
                                                                                 160). The thyroid antibodies were significantly increased: anti-TPO 1093
Catherine O’Brien1 , Mary McGraw1 , E. Jane Tizard1 , Edwin Massey2 ,
                                                                                 (norm<60 U/ml), anti-TG>2000 (norm<60 U/ml). Thus, our patient has
Moin Saleem1 . 1 Children’s Renal Unit, Bristol Hospital for Children,
                                                                                 autoimmune thyroiditis associated with minimal change nephrotic syn-
Bristol, United Kingdom; 2 Apheresis Unit, National Blood Service,
                                                                                 drome (biopsy proven). There are no clinical and biological indices for the
Bristol, United Kingdom
                                                                                 presence of other components of the autoimmune polyglandular syndrome.
Focal segmental glomerulosclerosis (FSGS) accounts for approximately             In conclusion, children with frequent relapsing or corticodependent
10% of idiopathic nephrotic syndrome of childhood. Over 50% of these             nephrotic syndrome should have periodic assessment of the thyroid status.
children will progress to end stage renal failure and ultimately require         Thyroid disorders may be due to: (i) iodine deficiency due to prolonged




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transplantation. However, recurrent disease in the transplant kidney will        salt restriction and loss of hormones with heavy proteinuria (ii) associated
occur in about 40% of these patients. Plasma exchange, usually with addi-        autoimmune thyroid disease (iii) thyroid cancer due to previous chemother-
tional immunosuppression, is well described as a treatment option in post        apy for nephrosis. Our patient has favourable effect of levamisole in relation
transplant recurrence. Reports of the use of plasma exchange for FSGS            to the nephrotic syndrome. Although levamisole may inhibit lymphocyte
of the native kidney are scarce. We describe our experience of individu-         infiltration of the thyroid in experimental settings, there are no clinical data
ally tailored treatment regimes for FSGS in the native kidney and in post        to support its effect on the course of Hashimoto thyroiditis.
transplant recurrent disease.
We report 6 children (4 boys, 2 girls; age range 6-16 years), 3 with FSGS in
the native kidney and 3 with post transplant recurrent FSGS. Each received        M822 OBESITY RELATED PROTEINURIA AND PROGRESSION OF
at least 6, single volume, plasma exchanges over a 14-day period with                       RENAL DISEASE IN CHILDREN OF EXTREME LOW BIRTH
albumin replacement using a Gambro BCT Spectra. Additional individu-                        WEIGHT
alised immunosuppressive regimes were administered; these included high
                                                                                 Carolyn Abitbol1 , Maria Rodriguez2 , Brenda Montane1 ,
dose intravenous steroid treatment in 5 of the 6 children. A total of 146
                                                                                 Jayanthi Chandar1 , Gaston Zilleruelo1 . 1 Pediatrics, University of
sessions of plasma exchange, combined with immunosuppression, were
                                                                                 Miami/Holtz Children’s Hospital, Miami, FL, United States; 2 Pathology,
administered over 25 patient months. All 3 children in the post transplant
                                                                                 University of Miami/Holtz Center, Miami, FL, United States
recurrent FSGS group achieved full remission with resolution of protein-
uria. In the group with FSGS of the native kidney 1 child achieved full          Background: Epidemiological studies have identified low birth weight as
remission (resolution of proteinuria), 1 partial remission (reduced protein-     a predisposing factor for the development of insulin resistance, obesity, and
uria and improvement in oedema), and 1 showed no clinically significant           diabetic nephropathy in adulthood. Recent reports associate proteinuria and
response. Overall three children who initially responded had further recur-      obesity with the histopathological findings of focal glomerular sclerosis
rences, all again entered remission (resolution of proteinuria) with a further   and glomerulomegaly. The purpose of our study was to examine the long-
induction course of plasma exchange and have subsequently received on-           term outcome of extremely low birth weight (ELBW) premature infants
going maintenance plasma exchange combined with immunosuppression.               who were referred to the Pediatric Nephrology service for specialty follow-
The frequency and duration of this maintenance phase was again adapted           up.
according to response. Whilst 5 patients (83%) experienced line complica-        Patients/Methods: Twenty-one infants with severe prematurity (Gesta-
tions, including line infections and displacement, these complications did       tional Age 25±2 weeks) and ELBW (mean 687±125 grams) were fol-
not result in the abandonment of treatment in any case.                          lowed over an 18 year period. Those with progressive renal disease (Renal
This single centre experience of an individually tailored approach to the        Disease (RD) (n=10) were compared to those with normal renal function
treatment of FSGS in children, provides further evidence of a role for           (No Renal Disease (NoRD) (n=11). Current age of surviving patients off
plasma exchange in the treatment of post transplant recurrent disease and        dialysis (n=16) is 7.5±4.6 years. Of the 10 patients with RD, 2 have steroid
adds to the paucity of reports of the use of plasma exchange in FSGS in the      resistant nephrotic syndrome, 3 have chronic renal insufficiency and 5 have
native kidney.                                                                   progressed to end stage renal disease (ESRD). Body mass index (BMI)
                                                                                 calculated as weight in kilograms/height in meters2 (kg/m2 ) was used as
                                                                                 a measure of overweight (>85%centile) and obesity (>95%centile). Mea-
 M821 AUTOIMMUNE THYROIDITIS IN A CHILD WITH
           CORTICODEPENDENT NEPHROTIC SYNDROME TREATED
           WITH LEVAMISOLE
Velibor Tasic1 , Meri Angjeleska2 , Nadica Ristoska-Bojkovska1 ,
Gordana Petrusevska3 , Zoran Gucev1 . 1 Pediatric Nephrology and
Endocrinology, Clinic for Children’s Diseases, Skopje; 2 Dept. of
Pathophysiology and Nuclear Medicine, School of Medicine, Skopje;
3
  Dept. of Pathology, School of Medicine, Skopje, The Former Yugoslav
Republic of Macedonia
Hashimoto thyroiditis is frequently described as a part of autoimmune
polyglandular syndrome. The association with minimal change nephrotic
syndrome is rarely described. Herein we report a pediatric patient who was
diagnosed to suffer from autoimmune thyroiditis four years after the onset
of nephrotic syndrome.
264       E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                                     Monday, June 9

surements were recorded at the most recent encounter or prior to entering                     M824 MICTURATING CYSTOURETHROGRAM IN INFANTS WITH
the ESRD program.                                                                                       FETAL HYDRONEPHROSIS
Results: BMI was significantly greater in the RD group as compared to
                                                                                             Maria Candida Bouzada, Eduardo Oliveira, Andrea Rodrigues,
the NoRD group (91±14%centile vs 64±33%centile; respectively, p =
                                                                                             Ricardo Parreiras, Ricardo Gonçalves, Alamanda Pereira. Department of
0.026). Elevated BMI > 85%centile for age carried a Relative Risk = 5.3;
                                                                                             Pediatrics, Federal University of Minas Gerais, Belo Horizonte, Minas
p=0.03 for renal disease. Proteinuria correlated significantly to progres-
                                                                                             Gerais, Brazil
sion of renal disease (r=0.6;p=0.003). BMI also correlated with degree of
proteinuria (r=0.8; p=0.004). Kaplan-Meier estimates of % Renal Survival                     A micturating cystourethrogram (MCU) is required to exclude vesi-
showed that patients with BMI >85%centile for age developed renal dis-                       coureteral reflux (VUR) in infants with prenatally detected pyelectasis.
ease at a significantly earlier age than non-obese patients. Renal biopsy                     If mild hydronephrosis is present or a normal appearing urinary tract is
specimens available in 3 patients showed focal glomerulosclerosis and                        observed in two successive neonatal ultrasound scans (US), the MCU
glomerulomegaly. Two additional whole renal specimens were also avail-                       will rarely be abnormal. Seventy-five neonates with a diagnosis of fetal
able and demonstrated a lower radial glomerular count than expected for                      hydronephrosis were followed up. US were done at the end of the first
age.                                                                                         week, at 6 months and at 1 year of age. Scintigraphy was indicated if
Conclusions: In summary, obesity and proteinuria were prominent features                     moderate/severe dilatation or progressive hydronephrosis was present. All
of children with extreme prematurity and low birth weight who showed                         patients were submitted to MCU and only 5 (6.6%) showed abnormal find-
evidence of progressive renal disease in childhood.The challenge remains                     ings. The results are presented in the table.
to determine the physiologic link between proteinuria and obesity.
                                                                                             Table. Abnormal MCU and outcome of US scan
                                             Free Communication June 10
                                                                                             MCU                                      Postnatal     6 months,    1 year,
                                                                                                                                      APD (mm)      APD (mm)    APD (mm)

 M823 VALUE OF ANTEROPOSTERIOR RENAL PELVIS                                                  Normal        70




                                                                                                                                                                             Downloaded from http://ndt.oxfordjournals.org/ by guest on May 1, 2013
                                                                                             Abnormal       5       VUR 5**          14        0       0            0
            DIAMETER DIAGNOSED BY PRENATAL ULTRASOUND AS
                                                                                                                                      0        0       0            0
            AN INDICATOR OF UROPATHY                                                                                                  6      5.4      5.7           0
                                                                                                                                     14      6.8      5.8          5.3
Maria Candida Bouzada, Eduardo Oliveira, Andrea Rodrigues,                                                                            –       16       17          11.2
Livia Fagundes, Alamanda Pereira, Henrique Leite. Department of                                                   Ureterocele 1     13.8     18.2     11.3          –
Pediatrics, Federal University of Minas Gerais, Belo Horizonte, Minas                        Total:        75
Gerais, Brazil                                                                               *APD: anteroposterior pelvis diameter **renal unit
Although renal pelvis dilatation is the most common congenital condition                     Four neonates had VUR (unilateral in 3 and bilateral in 1) and 1 ureterocele.
detected by fetal ultrasound, its evaluation and management are the sub-                     After delivery, the one with bilateral VUR presented a normal APD that
ject of enormous controversy. Seventy infants with prenatally diagnosed                      persisted up to the end of the first year of life, and the other 3 showed a
hydronephrosis were prospectively followed up from October 1999 to July                      tendency to a reduction in APD. Normal US scans after delivery do not
2002. An inclusion criterion was an anteroposterior pelvic diameter (APD)                    rule out the possibility of uropathy but judicious criteria should be applied
of 5 mm or wider during the third trimester of gestation. Ureteropelvic junc-                because MCU is not risk-free for neonates.
tion obstruction, vesicoureteral reflux, posterior urethral valves, megau-
reter, ureterocele and others were considered to be significant uropathies.
Postnatal follow-up included clinical evaluation as well as ultrasonography,                  M825 OUTCOME OF POSTERIOR URETHRAL VALVES TREATED
voiding cystourethrography and renal diuretic scan when indicated. Of the                               AT A SINGLE UNIT - MULTIFACTORIAL ANALYSIS OF
140 renal units evaluated, 103 presented with APD of 5 mm or more.                                      PREDICTORS OF OUTCOME
Uropathy was diagnosed in 23 infants (33%) and in 28 renal units (20%).
                                                                                             Leigh Haysom, Fiona Mackie, Robert H. Farnsworth, Andrew
The sensitivity, specificity, positive predictive value and negative predictive
                                                                                             R. Rosenberg, Gad Kainer. Department of Paediatric Nephrology, Sydney
value of APD were determined at different cut-offs and are presented in
                                                                                             Children’s Hospital, Randwick, NSW, Australia
the table.
Sensitivity, specificity, positive predictive value and negative predictive value of APD      Although it is stated that up to 50% of boys born with posterior urethral
                                                                                             valves (PUV) will progress to end stage renal failure (ESRF), the renal
Cutt-off   Sensitivity %,        Specificity %,         PPV %,               NPV %,           outcome is difficult to predict. To determine which factors may be used to
point (mm)    95%CI                 95%CI              95%CI                95%CI
                                                                                             more accurately predict outcome, we retrospectively analysed data from 48
7          92.6 (74.2 - 98.7) 18.4 (10.8 - 29.3)   28.7 (19.8 - 39.6)   87.5 (60.4 - 97.8)   consecutive patients between 1987-2002 who were followed for a median
10          81.5 (61.3 - 93) 60.5 (48.6 - 71.3)     42.3 (29 - 56.7)    90.2 (77.8 - 96.3)
15           59.3 (39 - 77)    92.1 (83 - 96.7)    72.7 (49.6 - 88.4)   86.4 (76.6 - 92.7)   of 93 months (10-228). Only seven boys (15%) have reached ESRF (me-
20         51.9 (32.4 - 70.8) 100 (94 - 100)        100 (73.2 - 100)     85.4 (76 - 91.7)    dian age of 60 months; range 4-140). The only factor predictive of outcome
25          44.4 (26 - 64.4)    100 (94 - 100)      100 (69.9 - 100)    83.5 (73.9 - 90.2)   was the nadir of serum creatinine within the first year after surgical relief
30         25.9 (11.9 - 46.6) 100 (94 - 100)        100 (56.1 - 100)    79.2 (69.4 - 86.5)   of obstruction (130 vs 44 micromol/L in the ESRF and non-ESRF groups
                                                                                             respectively; p<0.0001). Not predictive of outcome was age of diagnosis,
A threshold of 7 mm showed 92.6% sensitivity and 18.4% specificity for                        urinary incontinence at age five, antenatal detection of renal abnormality,
the diagnosis of significant uropathy. Above the cut-off of 20 mm the                         oligohydramnios and respiratory distress at birth. Other findings were a
specificity and the positive predictive value were 100%. In other words,                      strong association between PUV and urinary tract infection, vesicoureteric
using a threshold of 20 mm or greater there were no false-positive cases.                    reflux, undescended testes, dislocated hips and bilateral inguinal hernias.
Nevertheless, as expected, sensitivity decreased with increasing threshold.                  Early urinary diversion appeared to confer no benefit to outcome (5/7 ver-
Considering APD as a screening test, it is recommended that the cut-off                      sus 18/41 diverted in the ESRF and non-ESRF groups respectively), and
that maximized the true-positive rate should be selected for the purpose                     these boys on average had 3.2 additional operations over and above valvo-
of postnatal investigation. Therefore, fetuses with APD of 7 mm or more                      tomy.
should be submitted to detailed postnatal evaluation and should be followed
up.
Monday, June 9                                               E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                  265

 M826 GLOMERULAR FILTRATION RATE IN BOYS WITH HISTORY                          which can be used to differentiate glomerular and nonglomerular diseases
          OF SUCCESFUL RESECTION OF POSTERIOR URETHERAL                        in children with asymptomatic hematuria even in the absence of significant
          VALVES - THE VALUE OF CYSTATIN C MEASUREMENT                         proteinuria.
Marcin Tkaczyk1 , Anna Rogowska-Kalisz1 , Jolanta Lukamowicz2 ,
Michal Nowicki1 . 1 Department of Nephrology and Dialysis, Polish
                                                                                M828 OCCULT SPINAL DYSRAPHISM IN ENURETIC CHILDREN
Mother’s Memorial Hospital Research Institute, Lódz, Poland;
2
  Laboratory Unit, Polish Mother’s Memorial Hospital Research Institute,       Nese Karaaslan Biyikli1 , Aida Semic Jusufagic1 , Tufan Tarcan2 ,
Lódz, Poland                                                                   Harika Alpay1 . 1 Pediatric Nephrology, Marmara University Faculty of
                                                                               Medicine, Istanbul, Turkey; 2 Urology, Marmara University Faculty of
Cystatin C now is regarded as more sensitive and age-independent marker
                                                                               Medicine, Istanbul, Turkey
of renal function impairment. Thus we assessed plasma concentration of
cystatin C in presumably healthy boys after successful electroresection of     Clinical and laboratory findings of enuretic children seen at Pediatric
posterior urethral valves (PUV) in order to reveal early changes of renal      Nephrology outpatient clinic were reviewed. There were 165 children, 99
function.                                                                      girls (60%) and 66 boys (40%).The median presantation age for girls and
Twelve boys (aged 18-55 months) at least 12 months after the resection of      boys were 4.4 and 8.3 years respectively. Primary enuresis was recorded in
PUV and 10 healthy age-matched controls were studied. The absence of           108 (65.5%) and secondary one in 57 (34.5%) patients. Monosymptomatic
PUV or vesico-ureteral reflux was confirmed by ultrasound and micturating        (MNE) versus polysymptomatic (PNE) enuresis incidence was recorded
cystogram.                                                                     in 25 (15%) and 140 (85%) patients respectively. Positive family history
Serum and urine cystatine C concentrations were measured by im-                was present in 85 (51.5%) cases. Urinary tract infection was diagnosed
munonephelometry (Dade-Behring, Germany). Additionally, serum urea,            in 51 (31%) of 165 patients. Fifty seven VCUG studies were analyzed of
creatinine, blood pressure and albuminuria were measured. GFR was cal-         which 12 showed VUR. In order to identify the group of children with
culated from serum creatinine according to the Schwartz formula.               neuropathic bladder dysfunction, we performed urodynamic (UD) studies




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Serum concentration of cystatin C was higher in children after PUV resec-      in those having PNE symptoms. Of 50 UD studies 45 (90%) patients had
tion (0,92±0,12 mg/l) than in healthy controls (0,76±0,14 mg/l, p<0.01).       UD abnormalities being associated with the small capacity bladder (n=2),
In contrast to this finding, no difference in either serum creatinine or cal-   detrussor instability (n=16), or both of them (n=27). Lumbosacral MRI
culated GFR was detected (123,7±13,2 vs. 129,6±25,5 ml/min/1.73 m2 ,           evaluation was done in 46 cases of which 10 showed spinal cord abnormal-
p=0,6). Systolic and diastolic blood pressure was similar in the study group   ity (21%) as spina bifida occulta in 4, tethered cord in 4, syringomyelia in 1
and controls (systolic 93±10 vs 91±9 mmHg and diastolic 65±8 vs 62±7           and diastamatomyelia in 1. Five (50%) patients with spinal cord abnormal-
mmHg respectively). 24-h albumin excretion was comparable to controls          ity who do not have any sacral markings were 3 spina bifida patients and 2
(50,5±93,2 mg vs 34,4±26,2 mg in 24-h urine collection).                       tethered cord patients. On the other hand 15 patients (9%) out of 165 had
The study shows that children after successful resection of posterior ure-     sacral markings, of whom 11 had lumbosacral MRI studies. Among these
thral valves may exhibit subtle impairment of renal function that may be       11 patients, 6 (54.5%) cases had abnormal results with sacral dimple in 2
detectable only when more sensitive markers are applied.                       tethered cord patients and 1 syringomyelia patient, sacral hypopigmenta-
                                                                               tion in 1 tethered cord patient, sacral hairy patch in 1 diastomatomyelia
                                                                               patient.
 M827 DIAGNOSTIC VALUE OF URINARY MICROALBUMIN IN                              In conclusion, MRI detected occult spinal dysraphism in 21% of chil-
          CHILDREN WITH ASYMPTOMATIC HEMATURIA                                 dren with voiding dysfunction who have normal neurological examination
                                                                               which indicates the importance of investigating occult spinal dysraphism.
Larisa Prikhodina1 , Vladimir Dlin1 , Gennady Raba2 , Olga Morozova1 ,
                                                                               On the other hand patients who have sacral markings need early investiga-
Maya Ignatova1 . 1 Nephrology, Institute of Paediatrics & Child’s Surgery,
                                                                               tion to prevent further morbidity and mortality.
Moscow, Russian Federation; 2 Lab. of Pathology, Institute of Paediatrics
& Child’s Surgery, Moscow, Russian Federation
Asymptomatic hematuria is still remains one of the most difficult symptom        M829 CONSERVATIVE MANAGEMENT AND FOLLOW-UP OF
of kidney diseases for differential diagnostics because the degree of hema-              MULTICYSTIC DYSPLASTIC KIDNEY DETECTED BY FETAL
turia doesn’t reflect topical lesion of nephron’s damage. As known, mi-                   SONOGRAPHY
croalbuminuria (mA) (67kDa) is a sensitive marker of glomerular diseases.
                                                                               Eduardo Oliveira, Jose Silverio Diniz, Eli Rabelo, Jose Penido Silva,
In this study we investigated the significance of urinary mA in children with
                                                                               Isabela Pezutti, Maria Candida Viana. Department of Pediatrics, Federal
asymptomatic microscopic hematuria and normal renal function. We exam-
                                                                               University of Minas Gerais, Belo Horizonte, Minas Gerais, Brazil
ined forty children at a mean age of 10.15±0.23 [range 3.0-16.0] yrs with
asymptomatic hematuria persisting for more than 12 months. They were di-       Multicystic dysplastic kidney (MCDK) is the cystic anomaly most fre-
vided into2 groups: 1st , 18 patients (pts) with nonglomerular hematuria due   quently identified antenatally by prenatal echography, presenting an inci-
hypercalciuria or hyperuricosuria and 2nd , 22 pts with glomerular hema-       dence estimated at 1:4300 live births. The previously recommended sur-
turia who had biopsy proven mesangio-proliferative glomerulonephritis.         gical approach was justified by the possible complication associated with
The degree of hematuria in these pts (36.2±9.1 and 47.9±9.2 red blood          MCDK such as hypertension and malignant transformation. Currently this
cells/ml, respectively) was the same. Proteinuria was minimum and distin-      approach has been replaced with periodic clinical follow up, providing a
guished between pts of these groups (0.03±0.007 and 0.27±0.01 g/24h,           better understanding of the natural history of the anomaly. The objective of
respectively; P=0.038). The value of mA was measured in second spot            the present study was to describe the clinical course of conservative man-
morning urines by enzyme-linked immunosorbent assay (Randox Labora-            agement of MCDK. Between 1989 and 2001, 43 children (23 boys) with
tories, UK). The mA data were expressed as ratios to urinary creatinine        MCDK detected by prenatal ultrasonography were conservatively managed
(Cr) concentration by Jaffe reaction.                                          and prospectively followed. All patients were submitted to radioisotope
Mean [range] of the urinary mA/Cr was elevated significantly in pts with        scans and a micturating cystogram. Follow-up ultrasound examinations
glomerular hematuria than in cases of nonglomerular hematuria (14.0±2.8        were performed at 6-month intervals during the first two years and an-
[8.5-19.5] vs. 1.6±0.4 [0.8-2.5] mg/mmol, respectively; P<0.001). Fur-         nually thereafter. Clinical (weight, height, blood pressure) and laboratory
thermore, we established the minimal value of the mA/Cr, which allows          (urea, creatinine, urinalysis, and urine culture) data were systematically
differentiated glomerular and nonglomerular hematuria by using analysis        recorded. Mean age at diagnosis during the prenatal period was 31 weeks
of the logarithmical distribution of individual values of the mA/Cr. The       of gestation (range 20 to 41 weeks). Birth weight ranged from 1650 to 3830
urinary mA/Cr more than 6.0 mg/mmol is typical for pts with glomerular         g (mean, 2900). The affected kidney was palpable during the neonatal pe-
hematuria. The urinary mA/Cr less than 1.0 mg/mmol is characteristic for       riod in 21 patients (49%). Scintigraphy showed exclusion of the affected
pts with nonglomerular hematuria. These ranges of the urinary mA/Cr were       kidney in all cases. The ultrasound scan confirmed the diagnosis of uni-
significant different between pts of groups (the the chi-square test=38.6;      lateral MCDK disease in all cases and suggested mild hydronephrosis in
P<0.0001). Determination of urine mA/Cr is a simple, non-invasive test,        3 contralateral units and ureteral dilatation in 1 case. Vesicoureteral reflux
266      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                       Monday, June 9

into the contralateral ureter was found in only two patients (grade II in       ment and clinical follow-up are safe and desirable for the subgroup with
both). In the laboratory evaluation at admission, mean creatinine was 0.48      mild/moderate pelvic dilatation and preserved renal function.
mg/dl (range, 0.2 to 1.1) and mean urea was 22.5 mg/dl (range, 5 to 73).
During the neonatal period no child presented hypertension, with an aver-
age systolic pressure of 85 mmHg and a diastolic pressure of 45 mmHg.            M831 EFFECT OF INTERFRENTIAL CURRENT ON ENRESIS IN
Follow-up ranged from 4 to 135 months (median 36). No episodes of uri-                    CHILDREN
nary tract infection occurred during this period. Two children developed
                                                                                Mohammad Hossein Fallahzadeh1 , M. Ebrahimian1 , F. Fallahzadeh1 ,
transitory hypertension and all maintained normal growth percentiles. All
                                                                                A. Piri1 . Pediatric Nephrology, Shiraz University of Medical Sciences,
children included in the study were submitted to serial ultrasound exam-
                                                                                Shiraz, Fars, Iran; Pediatrics, Shiraz University of Medical Sciences,
ination, which demonstrated complete involution of the affected unit in 6
                                                                                Shiraz, Fars, Iran; Physical Therapy&Rehabilitation, Shiraz University of
(14%) cases. All children showing complete involution had been submit-
                                                                                Medical Sciences, Shiraz, Fars, Iran; Physical Therapy&Rehabilitation,
ted to at the least two ultrasounds, both of which confirmed the absence
                                                                                Shiraz University of Medical Sciences, Shiraz, Fars, Iran
of MCDK. The median age at complete involution of the kidney was 36
months (range 9 to 123). Partial involution of the affected kidney was ob-      Different treatment modalities has been recommended for enuresis as a
served in 28 (65%) cases and in 3 children (7%) there was no change in size.    common childhood problem.To our knowledge effect of interferential cur-
Ultrasound showed increase MCKD size in the other 6 (14%) cases. No             rent (IF) on enuresis has not been studied previously. There is some infor-
clinical or echographic findings of malignant transformation were observed       mation on the effect of electrotherapy in the treatment of this disorder. In
during follow-up. All children have maintained normal growth. In conclu-        this study 40 patients who were unresponsive to other modalities of treat-
sion, the natural history of MCDK is usually benign and serial ultrasound       ment were selected for IF therapy (3 times/wk, each for 15 minutes and to a
examinations show that affected kidneys frequently present involution with      total of 12 sessions). Half of the children had used medications previously,
time.                                                                           with no response. None of them had used enuresis alarm. Age of the chil-
                                                                                dren were 5 – 15 years (Mean=9.2) and half of them were female. Totally




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                                                                                complete response was observed in 6 patients (all were female) and relative
 M830 OUTCOME OF APPARENT URETEROPELVIC JUNCTION                                response (at least 50% reduction in the number of wet nights) in 21 of them
          OBSTRUCTION IDENTIFIED BY INVESTIGATION OF FETAL                      (67.5% had relative or complete response). The condition remained un-
          HYDRONEPHROSIS                                                        changed in 23 of the responsive patients up to 8 weeks after completing the
                                                                                treatment. There was no significant difference between clinical response
Eduardo Oliveira, Guilherme Apocalypse, Eli Rabelo, Jose Silverio Diniz,
                                                                                in the groups who had used or not used medications previously. Only one
Viviane Marino, Carlos Simal, Maria Candida Viana, Guilherme Silva.
                                                                                of the 8 patients with secondary enuresis was unresponsive, while in 32
Pediatrics, Federal University of Minas Gerais, Belo Horizonte, Minas
                                                                                patients with primary enuresis 12 were unresponsive to IF therapy. The
Gerais, Brazil
                                                                                response had no relation to age and no complication was reported. We can
Ureteropelvic junction obstruction (UPJO) is the most common cause of fe-       conclude that IF is effective in reducing enuresis and with more experience
tal urinary tract dilatation. Before the spread of antenatal ultrasonography,   in the future, it may be recommended as a modality of treatment, especially
children with UPJO usually presented with urinary tract infection, abdom-       in girls.
inal pain, and flank mass on palpation. At the time, standard management
consisted of surgical correction by a dismembered pyeloplasty. Recently
there has been a dramatic increase in the detection of hydronephrosis in         M832 THE CLINICAL IMPORTANCE OF ULTRASOUND
utero and consequently most apparent UPJO cases have been diagnosed                       DIAGNOSIS OF RENAL CALYCEAL MICROCALCIFICATION
in asymptomatic neonates. In this setting, the management of UPJO is                      IN CHILDREN
difficult because the diagnosis of significant obstruction cannot always be
                                                                                Ilmay Bilge, Aydan Sirin, Sevinc Emre, Banu Sadikoglu, Alev Kadioglu,
established with certainty in neonates and infants. The treatment of chil-
                                                                                Aysegul Sucu. Pediatric Nephrology, University of Istanbul, Istanbul
dren with unilateral hydronephrosis with apparent UPJO has remained a
                                                                                Medical Faculty, Istanbul, Turkey
controversial topic for more than a decade because the diagnosis of sig-
nificant obstruction cannot always be established with certainty in asymp-       The data about the clinical importance and natural history of children with
tomatic neonates and infants. We report the outcome of prenatally detected      renal calyceal microcalcification (RCM) detected by ultrasonography (US)
UPJO managed according to a more conservative protocol. The records and         are limited. For the increasing frequency of this entity during childhood,
imaging studies of 77 consecutive neonates with UPJO identified by fetal         we aimed to evaluate the clinical presentation, laboratory findings and
hydronephrosis were reviewed. A nonoperative approach was attempted             prognosis of children in whom RCM detected by US in our department.
in patients with mild/moderate pelvic dilatation, renal units with good         Our study was based on the data of 97 children (49 M, 48 F) with a mean
function as ascertained by DMSA scan and a non-obstructed pattern on            age of 20,6 ± 27,6 months (1 to 62 months) at the time of US diagnosis.
DTPA. Otherwise, the patients were managed surgically by pyeloplasty.           RCM was defined as an hyperechogenic spot less than 3 mm in diame-
Both groups were prospectively followed and the imaging studies were            ter in renal calyces detected by US during the study. All US evaluations
performed before and after the initial approach and at one-year intervals       were performed using Siemens Sonoline Elegra with a 7,5 MHZ trans-
thereafter. Of the 77 infants (85 units), 39 were submitted to surgery (33 to   ducer. The indication of US was recurrent abdominal pain in 26 patients
pyeloplasty and 7 to nephrectomy) and 38 were conservatively managed.           (26.8%), microscopic hematuria and/or dysuria in 28 patients (28.9%), re-
During follow-up, 9 (24%) of 38 patients in the non-operated group pre-         current urinary tract infection in 18 patients (18.6%) and routine control
sented renal function deterioration and 3 presented with urinary infections     or the evaluation for non-urinary symtoms in 25 patients (25.8%). There
and were submitted to pyeloplasty. Of the 39 patients surgically managed,       were 21 patient (21.6%) who had at least one normal US before. Thirty-
76% presented improvement of hydronephrosis and 90% showed a nonob-             eight children (39.2%) had a positive family history for urinary stone dis-
structed pattern on diuretic renography. Of the 33 units conservatively         ease. Hypercalciuria was detected in 63 children (64.9%) and 21of them
managed, 30% presented improvement of the washout pattern, 67% per-             (33.3%) had also hypocitraturia and/or hyperoxaluria. Hyperuricosuria was
sisted with an intermediate pattern, and 3% presented a deterioration of        established in 7 patients (7.2%). In 27 of all patients (27.8%), there was
the diuretic response. The differential renal uptake, as measured by DMSA       no detectable etiological reason for RCM. The mean follow-up time was
scan, remained stable in the three groups analyzed (conservative, initial       19.8±15.7 mo (range:1 to 84 months). Seventy-two patients (74.2%) were
pyeloplasty, and delayed pyeloplasty). There was a minimal improvement          re-evaluated at least 6 mo after the first RCM diagnosis. Of 72 patients who
in the units submitted to pyeloplasty with impaired renal function at base-     were re-evaluated during the study period, 11 of them (15.3%) developed
line (< 40%). Mean renal uptake was 28.6% at admission and 33.9% at the         renal calculi more than 4 mm in diameter, and RCM showed a regression
end of follow-up was. In conclusion, there is a wide spectrum of pelvica-       in diameter in 13 patients (18.1%), progression in 17 patients (23.6%) and
lyceal dilatation and UPJ stenosis. Surgical intervention in a subgroup of      remained unchanged in 14 patients (19.4%). RCM was not detectable in 17
patients with severe hydronephrosis and impaired function may possibly          patients (23.6%) on control US.
improve or preserve renal parenchyma. Conversely, conservative manage-          The increasing frequency of the diagnosis of renal calyceal microlithia-
Monday, June 9                                                 E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                  267

sis observing in children might be related to more sensitive sonographic         worsened. Three of these boys already had definite surgical correction with
equipment. Early detection of RCM may allow recognition of children at           closure of supravesical diversion and ureterocystoneostomy performed at
greatest risk for urinary stone disease. Our results show that renal calyceal    3 - 4 years of age. Renal dysplasia was found in 7 boys (bilateral in 6),
microcalcification may be present even in children without hematuria or           one non-functioning kidney required nephrectomy at 3 years of age. Four
hypercalciuria. We suggest that repeated US evaluations may be required          boys are stable in moderate CRF after 6 - 7 years of observation (GFR
not only in patients with hypercalciuria, but also in those with recurrent ab-   > 45 ml/min/1.73 m2 ). Two boys with a normal serum creatinine during
dominal pain, urinary tract infections, microhematuria not associated with       the first year of life have bilateral renal dysplasia and probably will enter
hypercalciuria and positive family history for stone disease.                    CRF during childhood. Febrile urinary tract infections occurred in 7 boys
                                                                                 during follow-up despite antibiotic prophylaxis. One obstructive candida-
                                                                                 mycetoma had to be removed surgically. Bladder function is well preserved
 M833 URINARY N-ACETYL-BETA-D-GLUCOSAMINIDASE LEVELS                             in 6 boys after 6 - 9 years of observation.
           AS AN INDEX OF RENAL TUBULAR FUNCTION IN                              Conclusions: Prenatal diagnosis of boys with PUV cannot prevent CRF
           CYANOTIC AND ACYANOTIC CONGENITAL HEART                               in many cases. Fetal interventions may convert a probably lethal condition
           DISEASE                                                               into a chronic renal disease. Neonatal and long-term therapy are a nephro-
                                                                                 logical and surgical challenge. Early diagnosis contributes to preserve renal
Pinar Isik Agras1 , Esra Baskin1 , Murat Derbent1 , Figen Ozcay1 ,
                                                                                 and bladder function, prevents additional renal damage, and seems to be of
Derya Aldemir2 , Kursad Tokel3 , Umit Saatci1 . 1 Pediatric Nephrology,
                                                                                 definite benefit for these severely compromized patients.
Baskent University, Ankara, Turkey; 2 Biochemistry, Baskent University,
Ankara, Turkey; 3 Pediatric Cardiology, Baskent University, Ankara,
Turkey
                                                                                  M835 SUPRA NORMAL RENAL FUNCTION IN UNILATERAL
The objective of this study was to investigate the renal tubular function in               HYDRONEPHROSIS. COMPARATIVE STUDY WITH MAG3
younger patients with congenital heart disease and compare the findings in                  AND DMSA SCINTIGRAPHY IN CHILDREN




                                                                                                                                                                 Downloaded from http://ndt.oxfordjournals.org/ by guest on May 1, 2013
cyanotic and acyanotic groups. Twenty patients with acyanotic, 24 patients
                                                                                 François Bouissou1 , Anne Julian2 , Eric Ouhayoun2 , Jacques Moscovici3 ,
with cyanotic congenital heart disease and 12 healthy children were en-
                                                                                 Christine Azema1 , Stéphane Decramer1 . 1 Néphrologie Pédiatrique,
rolled. Hemoglobin, thrombocyte count, serum sodium, creatinine in blood
                                                                                 Hôpital des Enfants, Tpulouse, France; 2 Médecine Nucléaire, CHU
and sodium, microalbumin, creatinine, Beta-2 microglobulin and NAG lev-
                                                                                 Purpan, Toulouse, France; 3 Urologie Pédiatrique, Hôpital des Enfants,
els in urine have been determined in simultaneous samples. The mean age
                                                                                 Toulouse, France
was 4.9±3.6 (0.2-12), 3.2±3.5 (0-11) and 3.7±2.8 (0-12) years in acyan-
otic, cyanotic and control groups, respectively. Groups were similar in age      Background: Hyperfunction of hydronephrotic kidney, when compared
and sex (p>0.05). There were no statistically significant difference in the       with controlateral kidney, has been well documented by MAG3 scintigra-
urinary Beta-2 microglobulin, urinary Beta-2 microglobulin/creatinine, uri-      phy. Its significance is not well understood: artefact or pathophysiological
nary microalbumin, FeNa and glomerular filtration rate between the groups         adaptation ? The outcome of this hyperfunction after surgery is not well
(p>0.05) The median urinary NAG/creatinine was significantly higher in            documented.
the cyanotic group (p<0.05).                                                     Aim of the study: To compare and follow up MAG3 and DMSA scintig-
The results of this study performed on patients younger than those studied       raphy in unilateral hydronephrosis in children.
previously, demonstrated that tubular injury in patients with cyanotic con-      Patients and methods: 204 chidren were prospectively enrolled (age:
genital heart diease is detectable as early as the first decade of life before    35m, 0-235m). 93 were followed up (1to 5 y), 77 out of 93 underwent
the development of glomerular injury and urinary NAG enzymuria is a              pyeloplasty. A total of 333 MAG3, 120 DMSA (late acquisition, 18h after
useful marker in detecting tubular injury.                                       IV injection) scintigraphies were analyzed. A cut off value >4% between
                                                                                 the two kidneys was used to consider renal hyperfunction of the obstructive
                                                                                 kidney.
 M834 IS PRENATAL DIAGNOSIS OF BENEFIT IN BOYS WITH                              Results: Supranormal renal function at diagnosis was found in 53% of
           POSTERIOR URETHRAL VALVES?                                            MAG3 and only 20% of DMSA (p<0.001).After surgery the proportion
                                                                                 decreased to 41% of MAG scans (p< 0.003), but remained stable for
Ekkehard Ring1 , Christoph J. Mache1 , Martin Häusler2 , Barbara Pertl2 ,
                                                                                 DMSA scintigraphies. If we consider DMSA scan as the gold standard
Michael Riccabona3 , Peter Vilits4 . 1 Department of Pediatrics,
2                                                                                to estimate separate renal function, the proportion of hyperfunctionning
  Department of Obstetrics, 3 Department of Radiology, 4 Department of
                                                                                 kidneys (difference MAG versus DMSA >4%) was higher: 64% at diag-
Urology, University Hospital, Graz, Austria
                                                                                 nosis and dramatic decrease after surgery, 14% (p<0.001). Hyperfunction
Posterior urethral valve (PUV) is the most severe urinary tract obstruction      was more frequent in patients< 1 year old (60% versus 49%, NS)
in boys leading to chronic renal failure (CRF) in many cases. Prenatal           Conclusion: DMSA scan (proximal tubular uptake, late acquisition after
diagnosis enables neonatal detection, immediate therapy after birth, and         IV) and MAG3 (fitration, tubular secretion, immediate acquisition after IV)
fetal interventions in selected cases. We analyzed our boys with prenatal        do not give superposable results in hyronephrosis..
diagnosis of urinary tract malformation (UTM) and postpartal diagnosis of        DMSA is more predictive of late renal function than MAG3.
PUV for neonatal presentation, need for surgical interventions in addition       Hyperfunction detected by MAG3 is partially reversible after surgery and
to catheter drainage of the bladder and valve resection, and for long-term       could be considered as an adaptative phenomenum and/or as an artefact
renal function to evaluate the contribution of early diagnosis to the outcome    (isotope accumulation at the early phase). However some patients kept
of patients.                                                                     persistant hyperfunction, suggesting a possible long standing positive effect
During a period of 10 years we had 278 consecutive patients with pre-            due to a partial obstructive uropathy.
natal diagnosis of UTM, and 9 boys (3.2%) had PUV. This final diagno-
sis was already suspected prenatally in 7 boys who had severe bilateral
hydroureteronephrosis after birth. Two boys with just moderate changes            M836 FEMALE URETHRAL MEATAL STENOSIS - MYTH OR
on prenatal and postpartal ultrasound had diagnosis after voiding cys-                     FACT?
tourethrography performed during the first weeks of life. Two boys with
                                                                                 Barbara Socik1 , Tomasz Jarmolinski1 , Mariusz Antoszewski2 ,
oligohydramnios had fetal interventions. One patient had vesico-amniotic
                                                                                 Stanislaw Paradowski2 . 1 Department of Nephrology and Dialysis, District
shunt at 19 weeks of gestation. The other presented with fetal urinary as-
                                                                                 Children’s Hospital, Szczecin, Poland; 2 Department of Pediatric Surgery,
cites, bilateral forniceal rupture, and left-sided urinoma at 30 gestational
                                                                                 District Children’s Hospital, Szczecin, Poland
weeks. He had peritoneo-amniotic shunt as pulmonary function was ques-
tionable and prohibited premature delivery. Seven boys had drainage of the       The existence of female urethral meatal stenosis (UMS) evokes contro-
bladder immediately after birth. Two neonates had urinoma resolving spon-        versies. The diagnosis is based on urethral calibration performed by some
taneously within 3 weeks. Four boys had bilateral supravesical diversion         urologists when symptoms and signs of infravesical obstruction are ev-
at 1 - 5 weeks of life as renal function deteriorated or urinary obstruction     ident. Normal structure of external urethral orifice in prepubertal period
268      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                                Monday, June 9

allows introduction of a dilator which diameter is (21 + years of age) Fr.        M838 ASSOCIATION BETWEEN GENES CONDITIONING LOW
The aim of our study was to present clinical picture of UMS and to answer                   RENIN ANGIOTENSIN SYSTEM ACTIVITY (RAS) AND
the question about its role in female urinary tract pathology.                              SEVERE HYPODYSPLASTIC CONGENITAL NEPHROPATHY
22 patients (pts) with UMS aged 2 mo-11 yr (mean 7,7 ± 3,0 yr) were
                                                                                 Federica Lombardo1 , Licia Peruzzi1 , Alessandro Amore1 ,
hospitalized at our departments in 2001-2002. The causes of referral, re-
                                                                                 Monica Chiesa1 , Roberta Bretto2 , Mirko Ferrigno2 , Cristina Zambelli2 ,
sults of abdominal ultrasonography (USG), micturating cystourethrogra-
                                                                                 Maria Chiara Russo2 , Emilio Merlini3 , Leandra Silvestro2 ,
phy (MCU) and urodynamic study (UDO) with special regards to possible
                                                                                 Gabriella Restagno4 , GianCarlo Mussa2 , Rosanna Coppo1 . 1 Nephrology
signs of infravesical obstruction, indications to urethral calibration, kidney
                                                                                 Dialysis Transplantation, Regina Margherita Children’s Hospital, Torino,
function on diagnosis of UMS, cystoscopic bladder appearance, renal scars
                                                                                 Italy; 2 Pediatric Clinic, University, Torino, Italy; 3 Pediatric Surgery,
in DMSA scan and the clinical effect of meatal dilatation were analyzed.
                                                                                 Ospedale Maggiore della Carità, Novara, Italy; 4 Clinical Pathology,
The causes of admission to hospital were: recurrent urinary tract infections
                                                                                 Regina Margherita Children’s Hospital, Torino, Italy
(UTI) with dysfunctional voiding (DV) in 9 pts, recurrent UTI in 4, recur-
rent UTI and nocturnal enuresis (NE) in 2, DV in 2 and, in individual cases,     Hypo-dysplastic renal dysgenesis, a frequent cause of chronic renal fail-
recurrent UTI with NE and nephrolithiasis of solitary kidney, first episode       ure in childhood, can have a familial nature, suggesting a role for genetic
of acute pyelonephritis, erythrocyturia, abdominal pain and hydronephro-         conditioning factors. Among the many genes currently under study, a par-
sis. Symptoms had lasted 0,5-8 years before diagnosis of UMS. Residual           ticular interest is focused on the RAS genes for their important role in
volume (RV) in the bladder after micturition was found in USG in 5 pts,          nephrogenesis, as they are among the major regulators of apoptosis and
thickening of bladder wall in 9 (4 pts with RV among them) and upper             cellular proliferation in the developing kidney. A previous investigation on
urinary tract dilatation in 5 (3 with RV). MCU revealed vesico-ureteric          the impact of ACE I/D gene polymorphism on congenital renal malfor-
reflux in 8 pts (bilateral in 6 and reflux to solitary kidney in 1) and blad-      mations (Ped Nephrol 2001) failed to prove any association, however the
der diverticulum in 1. Abnormal voiding pattern in UDO was found in 10           group of children investigated was not homogeneous, including also reflux
pts. Severe disturbances of bladder function (“pseudoneurogenic bladder”)        nephropathies and other urological abnormalities. Moreover, the patients




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were discovered in 3 pts. Indications to calibration were as follow: clinical    were compared with controls not matched for the geographical area of
(C) and radiological (R) features of infravesical obstruction in 7 pts, C, R     origin.
and urodynamic (U) signs of obstruction in 6, C and U in 4, C in 2 and R in      Aim of this study was to investigate the polymorphisms of the genes encod-
2. In one case UMS was found unexpectedly during cystoscopic evaluation          ing for angiotensin converting enzyme (ACE:D/I), angiotensin I receptor
for erythrocyturia. Two girls had impaired renal function on admission.          (ATR1:C/A) and angiotensinogen (AGT:C/T) in a highly selected series
Chronic cystitis was diagnosed by cystoscopy in 8 pts. In 5 pts signs of         of 19 children (15 M/4 F) with severe bilateral renal dysplasia and global
chronic pyelonephritis were found in DMSA scan. Clinical improvement,            renal function <50% than normal. Above dysplasia, 7 children presented
defined as decreased frequency of UTI and/or disappearance of obstruc-            with associated complex malformative uropathies. During the follow-up
tion signs and/or better voiding, was achieved in all 14 girls who were          6/19 children evolved to ESRF with requirement of substitutive dialytic
observed for the period longer than 3 months after dilatation. 10-yr-old girl    therapy in pediatric age. Data on polymorphisms were compared to 50
with “pseudoneurogenic bladder” and severe reflux nephropathy went into           healthy subjects of equal sex and similar geographic origin.
end-stage renal failure (ESRF) in spite of clinical improvement.                 The table reports gene frequencies (in %) in congenital hypodysplasia and
We concluded that observation of serious consequences of UMS and their           in controls, Odd’s ratio (OR) and confidence limits of selected genotypes
disappearance after dilatation of the stenosis may support the old thesis        in patients versus controls:
about the role of meatal stenosis in urinary tract pathology in girls. Repeti-
tion of controlled prospective study on this area seems to be substantiated.
                                                                                                         ACE (%)               AT1R (%)                AGT (%)
                                                                                                          II ID DD            AA AC CC                 TT TC TC
                                                                                 Hypodysplasia            21 58 21              47 42 10                10 63 26
 M837 CONGENITAL DEFECTS OF THE URINARY TRACT (UT) IN                            Controls                 10 56 34              56 32 12                16 52 32
           CHILDREN AGED 0-3 YEARS                                               OR (conf limit)    II: 2.4 (0.57-10.12)   AA: 0.7 (0.25-2.04)    TT: 0.63 (0.12-3.21)
Grazyna Krzemien, Agnieszka Szmigielska, Maria Roszkowska-Blaim,
Izabella Kostro, Monika Karpinska. Department of Pediatric Nephrology,
                                                                                 These data show a possible association, never reported in the literature,
Medical University of Warsaw, Warsaw, Poland
                                                                                 among genotypes considered able to negatively modulate the RAS and
We performed clinical evaluation of children with UT defects diagnosed           severe renal hypodysplasia.
during the first 3 years of life. The study included 363 children aged 1 week     These data are in agreement with the observations in man and in exper-
to 34 months (mean 5.6 ± 6.2 months) treated in our Department of Pedi-          imental models of a primary role of angiotensin II defects in abnormal
atric Nephrology in 1997-2002. Twenty-two percent of the subjects were           nephrogenesis, such as dysplasia in fetal kidneys due to ACE inhibitors
newborns, 62% were infants and 16% were children aged more than 1 year.          treatment during pregnancy and severe kidney malformations in mice with
Indications for UT imaging studies were as follows: UT infection in 246          deletions of the RAS genes.
(68%) children, abnormal prenatal ultrasonograhy (USG) in 26 (7%) chil-
dren, abnormal routine abdominal USG during hospitalization in 84 (23%)
children, other coexisting congenital defects in 4 (1%) children, and vesi-       M839             BLADDER SHAPE IMPACT ON THE ULTRASONIC
coureteric reflux (VUR) in patients’ siblings in 3 (1%) children. The most                          MEASUREMENT OF BLADDER VOLUME IN CHILDREN
commonly found UT defect was VUR, diagnosed in 217 (60%) children,
                                                                                 Andrea Cvitkovic Kuzmic1 , Boris Brkljacic2 , Davor Ivankovic3 . 1 Dept. of
followed by subpelvical ureteric stenosis in 61 (17%) children, ureteral
                                                                                 Pediatric Nephrology, Children’s Hospital Zagreb, Zagreb, Croatia;
duplication in 27 (7%) children, and paravesical ureteric stenosis in 19         2
                                                                                   Dept. of Radiology, University Hospital Dubrava, Zagreb, Croatia;
children. Abnormal UT USG was found in 216 (60%) children, including             3
                                                                                   Dept. of Medical Statistics, University of Zagreb, Zagreb, Croatia
only 21% with VUR but no other UT defect. Thirty-five (10%) children,
including 12 (6%) children with II-V grade VUR and other coexisting UT           Aim of the study was to evaluate the impact of the bladder shape on the
defects were referred for surgical treatment. Among 205 (94%) children           accuracy of US estimation of bladder volume in children, and to calculate
with VUR managed medically, follow-up micturition cystourethrography             formulas for US estimation of volume for different bladder shapes. US was
after 7-21 months (mean 13.5 ± 2.3 months) was performed in 112 (55%)            performed in 146 children (94 girls, 52 boys, age 9 months - 17 years,
children. Resolution of I-IV grade VUR was found in 73 (65%) children,           median 7.9 years) referred for urodynamic investigation. Three bladder
medical management was continued in 32 (29%) children, and 7 (6%) chil-          diameters were measured, and bladder shapes categorized as round, el-
dren were referred for invasive treatment. Routine abdominal USG allowed         lipsoid, cuboid, triangular, and undefined. Urine was collected, and total
earlier diagnosis of UT defects in 23% of children. Spontaneous resolution       bladder volume calculated. Correction coefficients (k) were calculated for
of VUR during the mean follow-up of 13.5 months was found in 65% of              the whole sample and for each bladder shape using linear regression anal-
children                                                                         ysis. Our results showed that 23% of children had round bladder shape,
Monday, June 9                                                 E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                   269

25% cuboid shape, 21% ellipsoid shape, 25% triangular shape, and 6% un-           In vitro study Decreasing concentrations of SonoVue (5-0.5 ml, equivalent
defined shape. Correction (regression) coefficient (k) for the whole sample         to 40-4 µl of CM; one vial = 40 µl) were diluted in 250 ml normal saline-
was 0.66 (S.E.=0.011); for round bladder 0.561 (S.E.=0.013), cuboid blad-         filled containers. The presence of CM microbubbles were daily evaluated
der 0.923 (S.E.=0.012), ellipsoid bladder 0.802 (S.E.=0.006), triangular          using the Esatune (Esaote, Italy) and the H21 (Hitachi, Japan) US scanners
bladder 0.623 (S.E.=0.007), for undefined shape 0.749 (S.E.=0.048). Mean           with the fundamental, HI and CnTI (that characteristically visualizes only
percentage errors when utilizing different correction coefficients were sig-       the CM) modalities. The endocavitary persistence of this CM was surpris-
nificantly lower compared to errors when utilizing k=0.5 applied to our            ingly long (at least 14 days also for the most diluted concentrations), and
data.                                                                             depended on the US modality and the mechanical index (MI) used, being
Conclusion: Bladder shape has considerable impact on the accuracy of US           maximal with the CnTI modality at a low MI.
estimation of bladder volume in children. Correction coefficients should be        In vivo study 30 Patients (19 M, 11 F: 17 aged 2-12 months, 8 aged
used for volume measurements of different bladder shapes.                         1-3 years, 4 aged 4-6 years, one adolescent boy) performed CUS due
                                                                                  to pyelonephritis (17 cases), antenatal hydronephrosis (9 cases), or other
                                                                                  causes (4). After informed written consent of both parents or tutors who
 M840 WHICH PARAMETERS ARE RELEVANT TO PREDICT THE                                collaborated in the assitance, 0.8-4 µl of SonoVue were administered into
           RISK OF RENAL DETERIORATION IN WATCHFUL WAITING                        the bladder through a 4Fr catheter along with normal saline, according
           FOR URETEROPELVIC JUNCTION (UPJ) OBSTRUCTION?                          to the bladder capacity. All the bladder filling and voiding were studied
                                                                                  with the HI and CnTI modalities, and the images of the urinary tract were
Stefan Conrad2 , Markus Kemper3 , Thilo Eggert2 , H. Huland2 , Dirk
                                                                                  recorded. After discussion of the diagnostic conclusions, the parents were
E. Mueller-Wiefel1 . 1 Pediatric Nephrology, University Children‘s
                                                                                  requested to answer a Questionnaire about the quality of the information
Hospital, Hamburg, Germany; 2 Urology, University Hospital, Hamburg,
                                                                                  and communication received, the understanding of the US images, their
Germany; 3 Pediatric Nephrology, Children‘s Hospital, Zürich,
                                                                                  emotional experiences, and the perceived suffering of their children.
Switzerland
                                                                                  No adverse effect due to the procedure was observed. CUS was very well




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Introduction and objectives: Watchful waiting is an accepted treatment of         accepted by parents and by their children. Understanding of the US im-
congenital UPJ obstruction, but is associated with a risk of irreversible renal   ages as well as communication and information were considered good or
damage. Many urologists therefore rely on potential prognostic parameters         excellent. CUS was performed with very low (0.4-1µl) doses of CM; the
such as washout in isotope renograms or ultrasound features to define              use of two different US modalities (HI and CnTI) reduced the diagnostic
subgroups of patients with elevated risk that will then undergo either closer     interference of the artifacts. The mean time of CUS was 25’.. VUR was
observation or immediate pyeloplasty. These parameters however are rarely         detected in 18 out of 60 kidney-ureter units (30%): 3 grade I, 8 II, 6 III,
prospectively validated. We therefore analyzed the true prognostic potential      and 1 IV. 43% of children (54.5% of the F and 36.8% of the M), all aged
of commonly used prognostic parameters using data from a prospective              less than 5 years, had VUR. According to the CUS indications, 41% of
study on watchful waiting in patients with UPJ obstructions.                      cases with pyelonephritis and 44% with hydronephrosis had VUR. CUS
Methods: Children with newly diagnosed asymptomatic UPJ obstruction               demonstrated a proximal urethral dilatation in a girl aged 5 years with a
were followed expectantly, if split renal function was normal and no addi-        functional obstruction, confirmed with the urodynamics, and a rare case of
tional pathology was present. Follow-up consisted of clinical examinations,       proximal urethral stricture in an adolescent boy, confirmed with cystoscopy.
standard and duplex Doppler sonography and isotope renograms at 3, 6, 18          Il conclusion, the long-lasting endocavitary echo enhancement offered by
and 30 months. Delayed pyeloplasty was performed in case of functional            very low doses of sulphur hexafluoride can significantly contribute to a
deterioration. The ability of furosemide washout characteristics, ipsilateral     good visualization of the refluxing urinary tract with CUS, improving its
and contralateral renal lengths and pelvic diameters and resistive indices        diagnostic possibilities and reducing the costs of the examination.
at study entrance to predict subsequent renal deterioration was analyzed
using multiparametric logistic regression.
Results 48 children with UPJ obstruction were followed for a mean of 24            M842         PRO- AND ANTI-INFLAMMATORY CYTOKINES IN
months. Of these, 4 kidneys deteriorated and underwent delayed pyelo-                           CHILDREN WITH VESICO-URETERAL REFLUX
plasty. Directly prior to deterioration, furosemide washout was reduced
                                                                                  Svetlana Paunova1 , Alla Kucherenko2 , Ivan Smirnov2 , Elena Olchova3 ,
and pelvic diameter was increased significantly in these kidneys compared
                                                                                  Olga Anochina1 . 1 Chair of pediatrics, Russian State Medical University,
to those with stable function in contrast to renal length and resistive in-
                                                                                  Moscow, Russian Federation; 2 Dpt of Pathophysiology, RAMS Research
dices. When entering the study, however, renal pelvic diameter was the
                                                                                  Institute, Moscow, Russian Federation; 3 Dpt of Ultrasound Investig.,
only parameter with significant prognostic impact on future renal function
                                                                                  St.Vladimir’s Childrens’ Hospital, Moscow, Russian Federation
(odds ratio 1.183 per mm diameter, 95% confidence interval 1.011 to 1.385,
r2=0,22, intercept 6,169, p<0,05), while furosemid washout, renal length          In order to reveal the role of pro- and anti-inflammatory cytokines in renal
and resistive index were without any prognostic significance.                      scar formation in children with chronic pyelonephritis (CP) and vesico-
Conclusions: Of numerous prognostic parameters commonly used to de-               ureteral reflux (VUR) 123 patients with impaired urodynamics aged from
fine the individual risk of renal deterioration in UPJ obstruction, only renal     3 to 14 years were examined at the beginning of the study and after 6
pelvic diameter is of proven although limited prognostic significance when         months follow-up.
studied prospectively, while the prognostic value of furosemide washout           In all of them the 24 hours urine excretion of interleukins 8, 10 (IL-8, 10)
is neglectable. New prognostic parameters, potentially detecting molecu-          and tumor necrosis factor-a (TNF-a) has been measured by immunoenz-
lar mechanisms of atrophy, are necessary to individualize the treatment of        ime method. All the children underwent color Doppler ultrasound (CDUS)
congenital UPJ obstructions.                                                      examination of kidneys. Three groups of patients were determined: 1) with
                                                                                  VUR and normal X-ray and ultrasound image of kidneys (n = 94), 2)
                                                                                  with CDUS detected reflux nephropathy (RN) (n = 12), 3) with IVU con-
 M841 DEVELOPMENTS IN THE ULTRASOUND IMAGING OF                                   firmed reflux nephropathy (n = 17). Patients with CP without VUR (n =
           VESICO-URETERAL REFLUX IN CHILDREN                                     10) were used as controls. As a result, all the patients demonstrated signif-
                                                                                  icantly elevated daily urine excretion of IL-8, 10 and TNF-a in comparison
Maurizio Bosio. Paediatric Nephrology and Ultrasonography, Italian
                                                                                  with controls (p<0,05) at 1st investigation and mild increasing of IL-8
Diagnostic Centre, Milan, Italy
                                                                                  and TNF-a concentration after 6 months of follow-up. In children with-
Contrast-enhanced cystourethrosonography (CUS) is a diagnostic imaging            out X-ray and ultrasound signs of renal scarring IL-10 urine concentration
modality with high accuracy in detecting and grading vesico-ureteral re-          increased approximately in 1,2 times (from 56,3±2,2 pg/ml to 63,0±1,7
flux (VUR) in children. Further important technical improvement could be           pg/ml, p<0,05). In opposite, representatives of 2 and 3 groups showed
offered by a contrast medium (CM) long-lasting in the endocavitary flu-            identical cut-back of IL-10 level in 24 hours urine within 6 months after
ids, and easily recognized by the actual US technologies.AIM of this pilot        1st investigation (from 70,5±2,3 pg/ml to 54,4±3,1*pg/ml-2nd group, from
study was to evaluate in vitro and in vivo the endocaviatry properties of a       44,3±1,5 pg/ml to 36,5±4,4*pg/ml-3rd group, *p< 0,05)
new CM, sulphur hexafluoride (SonoVue Bracco, Italy).                             To conclude, the similar dynamics of IL-10 urine level in patients with
270      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                       Monday, June 9

X-ray confirmed RN and CDUS sings of renal scar formation suggests              office BP measurements. Recently data for ABPM in children have been
using it as an early marker of renal tissue damage. The diminishing of anti-   published. The largest such series (Soergel et al 1997) provides limits of
inflammatory cytokine may reflect the protraction of covered inflammation         normal ABPM derived from mid-European children.
in renal parenchyma in remission of chronic pyelonephritis.                    The aim of this study was to determine the effect on interpretation and
                                                                               clinical decisions of reporting ABPM records using 95th centile limits from
                                                                               both office derived (USA Task Force Blood Pressure 1996) and European
 M843         BLOOD PRESSURE AND RENAL FUNCTION IN 19 YEAR                     ABPM data.
              OLD ADULTS AFTER INTRA UTERINE GROWTH                            We used the SpaceLabs 90207 in a standardized fashion. 57 ABPM records
              RETARDATION AND PREMATURE BIRTH                                  from patients 5 to 18 years of age at our Paediatric Nephrology Clinic were
                                                                               reported using 95th centile limits from both sets of normative data (limits
Mandy G. Keijzer-Veen1,2 , Martijn J.J. Finken2,3 , Jeroen Nauta1 , Friedo
                                                                               for sleep periods in the Task Force group were set at 10% less than the 95th
W. Dekker2 , Elysee T.M. Hille4 , Jan Maarten Wit3 , Bert J. van der
                                                                               centile for awake BP). Records were reported by paediatric nephrologists
Heijden1 . 1 Pediatric Nephrology, Erasmus Medical Center Rotterdam -
                                                                               who were blinded as to the normative data used to set the 95th centile
Sophia Children’s Hospital, Rotterdam, Netherlands; 2 Clinical
                                                                               limits. For each record, the nephrologist determined whether the child was
Epidemiology, Leiden University Medical Center, Leiden, Netherlands;
3                                                                              hypertensive and decided upon management.
  Pediatrics, Leiden University Medical Center, Leiden, Netherlands;
4                                                                              Using Task Force derived limits 45 of 57 records were interpreted as
  Prevention and Health, TNO, Leiden, Netherlands
                                                                               demonstrating hypertension. Using the European ABPM derived limits
According to Barker’s hypothesis infants exposed to intrauterine growth        3 of these 45 (6.7%) were thought not to show hypertension and a further
retardation (IUGR) are predisposed to develop parameters for Syndrome X        4/45 (8.9%) were felt to be indeterminate.
in adult life. In addition, some studies indicate that impairment of kidney    The median systolic BP load for all records using Task Force limits, was
development and alterations in Renine Angiotensin System (RAS) activity        higher than when the European limits were set (64% vs. 47%, Wilcoxon
is associated with IUGR. It has been suggested that impaired fetal kidney      p<0.0001) as was the daytime systolic load (72% vs. 44% p<0.0001), but




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development plays an important role in hypertension and (end-stage) renal      not for the night-time systolic load (60% vs. 57% p>0.4).
disease in adult life. Furthermore, the influence of premature birth on renal   A 24 hour systolic load greater than 50% was found in 33 records in the
function and blood pressure in adult life has not been studied yet. The        Task Force limits set, compared to 27 in the set using the European limits
objectives of this study are to determine renal function and blood pressure    (χ 2 , p>0.26). The corresponding numbers were 38 and 26 for daytime (χ 2 ,
in 19 year old subjects born prematurely, with or without IUGR.                p=0.025) and 34 and 36 (N.S) for the night time period.
A multicenter prospective follow-up study of a subset of the POPS cohort       The combined results of this study demonstrate that using different ’nor-
(Project Of Prematures and Small for Gestational age infants) at 19 years      mative data’ to set limits for ABPM in childhood may lead to differing
of age was performed in the Netherlands. Our study population contained        clinical interpretation. The data set of Soergel et al tend to provide higher
all subjects born in 1983 with a gestational age (GA) < 32 weeks (n=676).      blood pressure limits, resulting in a reduction of calculated blood pressure
Subjects were divided in groups correcting BW for GA. Subjects with a          load.
BW percentile < P10 were defined as small for gestational age (SGA)             These findings suggest that there is a need to determine which of the
and subjects with a BW percentile >= P10 were defined as appropriate            published ABPM data allow a more accurate diagnosis of hypertension
for gestational age (AGA). Data of 243 subjects concerning blood pressure      that will correlate with pathophysiological effects and long term outcomes.
and body mass index (BMI) were obtained. Blood and urine samples were
obtained to measure renal function (creatinine clearances, urinary albumin-
and sodium excretion) at the age of 19 years. Laboratory results were           M845 VARIABILITY IN INTERPRETATION OF PAEDIATRIC
available for 153 subjects.                                                              AMBULATORY BLOOD PRESSURE MONITORING
Preliminary results show significantly higher systolic, diastolic and mean
                                                                               Sean Kennedy, Gad Kainer, Andrew R. Rosenberg, Fiona Mackie.
arterial blood pressure values in 18 SGA subjects with a BMI >= 20 at
                                                                               Nephrology, Sydney Children’s Hospital, Randwick, Sydney, NSW,
the age of 19 years, compared to 20 SGA subjects with a BMI < 20
                                                                               Australia
adjusted for gender, maternal smoking and maternal hypertension during
pregnancy (linear regression analysis, P value < 0.05). However, blood         Oscillometric 24 hour ambulatory blood pressure monitoring (ABPM) is
pressure values in SGA subjects were still within the normal range (121.6 ±    gaining acceptance in the diagnosis and management of hypertension in
11.1 mmHg for SBP, 65.8 ± 8.3 mmHg for DBP and 85.7 ± 8.1 mmHg for             children. The diagnosis of hypertension using ABPM is based on parame-
MAP). No differences between blood pressure values were seen between           ters derived from successful BP readings and partly on the judgment of the
AGA subjects with a BMI < 20 or a BMI >= 20 (n=205).                           reporting physician. The most widely used parameters are mean BP, BP
Creatinine clearance did not differ significantly between SGA (n=19) and        load, BP index and presence of nocturnal ’dipping’.
AGA (n=134) subjects adjusted for gender and BMI. Also, no differences in      The ’culling’ of readings beyond the extremes programmed into the mon-
urinary albumin excretion, albumin creatinine ratio and sodium excretion       itor is performed automatically. Patient activity diaries, heart rate and
were seen. However, small numbers of renal parameters were available           recording of sleep/awake times are generally provided with the record.
when preliminary data were analysed.                                           The clinician may use this information to manually edit the record before
In conclusion, preterm SGA subjects with a relatively high BMI at the          decision making. The application of judgment makes it possible for the
age of 19 years have a higher systolic, diastolic and mean arterial blood      same data to be interpreted differently by each physician. There are no
pressure in this prospective follow-up study. These subjects may be at         published studies reporting on the variability of interpretation of paediatric
risk for hypertension in later life. Renal parameters have not yet shown       ABPM by different clinicians.
differences between SGA and AGA subjects.                                      The aim of this study was to determine whether experienced paediatric
                                               Free Communication June 11      nephrologists could agree on the diagnosis of hypertension based on
                                                                               ABPM.
                                                                               We used a SpaceLabs 90207 in a standardized fashion. The children aged
 M844 24 HOUR AMBULATORY BLOOD PRESSURE (ABPM) IN                              5 to 18 years, were all seen at our centre for the investigation of sus-
          CHILDREN. DO THE LIMITS WE SET AFFECT CLINICAL                       pected hypertension or for ongoing management of previously diagnosed
          DECISIONS?                                                           hypertension. 102 ABPM records were de-identified before being reported
                                                                               independently by 3 paediatric nephrologists. For the purposes of this study
Sean Kennedy, Gad Kainer, Andrew R. Rosenberg, Fiona Mackie.
                                                                               two questions were asked: a)Is this patient hypertensive? b)Would you alter
Department of Nephrology, Sydney Children’s Hospital, Randwick,
                                                                               management? For each question, the choices for answers were “yes”, “no”
Sydney, NSW, Australia
                                                                               or “indeterminate/undecided”.
ABPM is potentially a more useful tool in the diagnosis and management         The three nephrologists agreed on the answer to both questions for 61
of hypertension in childhood than office BP measurement. Traditionally,         records (59.8%). At least 1 nephrologist disagreed with the others’ diagno-
interpretation of ABPM has been performed using normative data from            sis of hypertension 16 times and decisions on management were opposed
Monday, June 9                                                E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                  271

22 times. The majority of disagreements over diagnosis were because at           M847 ANGIOTENSIN-CONVERTING ENZYME GENOTYPE IN
least one answered “indeterminate” (23%).                                                 CHILDREN AND YOUNG ADULTS WITH DIABETES
This study demonstrates the need for consensus on standardization of in-                  MELLITUS TYPE 1 AND FAMILIAL HISTORY OF
terpreting ABPM in childhood.                                                             HYPERTENSION AND CARDIOVASCULAR DISEASES
                                                                                Terezie Šuláková1 , Alena Kloudová5 , Josef Gut2 , Vlasta Janštová1 ,
                                                                                Jana Cerná1 , Andrzej Ciechanowicz8 , Grazyna Adler8 , Lenka Kocinová2 ,
 M846 CHANGES OF ARTERIAL BLOOD PRESSURE IN THE TEST
                                                                                Nad’a Filaková3 , Helena Strmenová4 , Andrzej Brodkiewicz7 ,
          WITH PHYSICAL LOAD IN TYPE 1 DIABETIC CHILDREN
                                                                                Jaroslav Hubácek6 . 1 Dpt of Paediatrics, University Hospital, Ostrava,
Tatiana Nastausheva, Irina Kuznetsova, Lilia Stakhourlova,                      Czech Republic; 2 Dpt of Paediatrics, Regional Hospital, Ceská Lípa,
Alexandra Nastausheva, Anatoly Shvirev. Pediatric Department, Medical           Czech Republic; 3 Dpt of Pediatrics, City Hospital, Ostrava, Czech
Academy, Voronezh, Russian Federation; Regional Children Hospital,              Republic; 4 Dpt of Paediatrics, Regional Hospital, Havírov, Czech
Voronezh, Russian Federation                                                    Republic; 5 Dpt of Immunology and Allergology, Institute of Public
                                                                                Health, Ostrava, Czech Republic; 6 Lab Athero Res, IKEM, Praha, Czech
The aim of this work is to determine the changes of arterial blood pressure
                                                                                Republic; 7 Dpt of Paediatrics, Pomerian Medical University, Szczecin,
(ABP) in the test performed with physical load - veloergometry (V) in
                                                                                Poland; 8 Dpt of Pathobiochemistry and Molecular Biology, Pomerian
type1 diabetic children.
                                                                                Medical University, Szczecin, Poland
97 children having type1 diabetes mellitus were examined, aged 9 to 17
years old, with the disease history from first revealed to 15 years duration.    It is suggested that the insertion/deletion (I/D) polymorphism of the
All the children were decompensated without ketoacitosis; they were nor-        angiotensin-converting enzyme (ACE) is an independent risk factor for hy-
motensive and did not have proteinuria. All the children were divided into      pertension (HT) and a coronary artery disease. It is also known that familial
7 groups taking into account the disease history for I type diabetes: from      predisposition to essential HT and cardiovascular diseases (CVD) increases
the first revealed to 10-13 years duration. The glucose level, HbA1c did         the risk of diabetic nephropathy. The aim of our study was to compare the




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not show significant difference in the groups examined: 10.1±3.8 mmol/l          distribution of single genotypes and alleles of I/D ACE polymorfism in 2
in the 1st year to 12.1±3.0 mmoll/l having the 10-13th year of the disease      groups of Czech diabetic children: with or without the presence of positive
and 12.3±8.3% to 16.3±1.7% correspondingly.                                     familial history of HT and CVD.
The control group consisted of 25 healthy children of the same age. All the     We analysed the distribution of the ACE genotype in 153 children, adoles-
children underwent veloergometry with the physical load on the bicycle          cents and young adults from the Czech Republic (111 from Ostrava, North
ergometer “Jagger” ER 900 with the use of the cardiomonitor ESAOTE              Moravia, 42 from Ceská Lípa, North Bohemia). The patients were 77 boys
BIOMEDICA “Acta plus”. The test PWC was applied. The values of sys-             and 76 girls (aged 15.78 4 ± 8.845, mean ± SD). The distribution of the
tolic (S), diastolic (D) and mean (M) arterial blood pressure (BP) were         ACE I/D genotype was: II = 39 (25.49%), ID = 72 (47.06%) and DD 42
assessed during the maximal load.                                               (27.45%). Allele frequencies were I = 150 (49%), D = 156 (51%). The
In the control group of children normotesive type of hemodynamic re-            distribution of the genotype did not differ from the genotype distribution
sponse to load was observed in 19 children (76.9%), hypertensive type was       in the population of the Czech Republic.
marked in 4 (16.0%) children, and hypotensive type - in 2 (8,0%) children.      The patients were divided into 2 groups. Group 1 included 66 (47.83%) pa-
In diabetic patients hypertensive type was observed 2.4 times as often (in 37   tients with negative CVD familial history (including HT), group 2 included
children, that makes 37.8%). The frequency rate of hypertensive responses       72 (52.17%) patients with a positive CVD and HT familial history. We did
to the load increased as the disease duration grew (0% at the beginning,        not succeed in getting the familial history in 15 patients. The genotype
31.3% on the second year of the disease, 53.3% in the disease duration of       distribution in group 1 was I = 17 (12.32%), ID = 38 (27.54%) and DD =
6-7 years and 62.5% in the disease history of 10-13 years).                     11 (7.97%), and in group 2: II = 16 (11.59%), ID = 31 (22.46%) and DD
Systolic type of hypertensive response was revealed in the control group        = 25 (18.12%). We found that the relation between the positive CVD and
in boys only (25.0%). In diabetic patients hypertensive type of reactions       HT familial history and the DD genotype was not statistically significant,
during veloergometry was observed in boys and girls equally: 34.5% and          or on the border of the statistical significance (ch2 = 5.9352, p = 0.0514,
30.9%. Except for systolic type of reactions found in 14 patients (14.3%),      on the p level 0.05). The relation between the presence of D or I allele and
there was diastolic type - in 16 patients (16.3%) and mixed type - in 7         the positive or negative familial history in the groups was: group 1 (I = 55,
patients (7.1%). Diastolic type of reactions prevailed in girls: 11 (11.2%),    D = 11), group 2 (I = 16, D = 56) (ch2 = 51.4829, p = 0.0000). We found
systolic - in boys: 10 (10.0%). Significant increase of DBP and MBP to           significant over-representation of D allele in the group 2 and I allele in the
the load was found in patients with the disease history of 2-3 years dura-      group 1.
tion compared with the control group: 71.2±21.6 mmHg, p<0.05, 102.7             Conclusion: Significantly higher D and lower I allele frequencies were
±15.9 mm Hg, p<0.05, correspondingly in the control group - 63.3±17.0           present in the children and young adults with diabetes mellitus type 1 and
mmHg and 93.4±14.5 mmHg. The correlation between DBP, MBP and                   with the positive familial history. Connection between the CVD and HT
the duration of diabetes was found during the maximal load: 0.29, p<0.01        familial history and D allele could be useful for detecting patients with
and 0.31, p<0.01 correspondingly and between DBP,MBP and the daily              high risk of diabetic nephropathy.
insulin dose (r=0.28, p<0.01, r=0.3, p<0.01 correspondingly).
Thus it was determined that hypertensive reactions to the physical load
were met more often in type I diabetic children compared to healthy chil-        M848 EVIDENCE FOR A GLOBAL ACTIVATION OF CIRCULATING
dren. Children, particularly, girls with the disease duration from 2-3 years              RENIN ANGIOTENSIN SYSTEM IN PEDIATRIC DIABETIC
represent the risk-group for the arterial hypertension development.                       PATIENTS
                                                                                Ana Cristina Simoes e Silva, Augusto Cesar Soares dos Santos Junior,
                                                                                Mariana Guimaraes Penido, Patricia P.C.A. Salgado. Departamento de
                                                                                Pediatria, Universidade Federal de Minas Gerais, Belo Horizonte, Minas
                                                                                Gerais, Brazil
                                                                                The diabetic nephropathy is a common complication found in patients
                                                                                with diabetes mellitus type 1. There are several factors associated with
                                                                                the progression to diabetic nephropathy such as patient’s glycemic control,
                                                                                genetic pattern and the Renin-Angiotensin System (RAS) profile. The aim
                                                                                of this study was to evaluate the profile of circulating RAS in pediatric
                                                                                patients with diabetes mellitus type 1, comparing to the same data obtained
                                                                                from healthy control subjects.
                                                                                We studied 26 pediatric diabetic patients with mean age of 13.7 years and
                                                                                32 control subjects. All patients were clinically evaluated at a Pediatric
272         E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                     Monday, June 9

Center. Blood samples for plasma renin activity (PRA) and angiotensin            correlation (r=0.48, p<0.01) between glycated hemoglobin (HbA1c) and
peptides measurements were obtained in all subjects after the sign of an         glomerular filtration (CCr).
agreement term. The samples for PRA were iced collected in tubes contain-        In conclussion, oxidative stress is present in young DM1 patients with
ing EDTA 7.5%. Plasma samples for Angiotensin peptide measurements               good glycemic control, without subclinical microangiopathy, even in early
were iced collected using a solution peptidase inhibitors and were extracted     stages of disease. This increase was more evident in DM1 children and
using Bond-Elut cartridges. After extraction, plasma samples were resus-         adolescents with glomerular hyperfiltration, suggesting a close relationship
pended and the concentration of Angiotensin I (Ang I), Angiotensin II            between hyperglycemia, oxidative stress and renal dysfunction.
(Ang II) and Angiotensin-(1-7) [Ang-(1-7)] were determined by radioim-
munoassays as previously described (Peptides, 1994).The results were ex-
pressed as mean ± standard error. The non-paired student t test was used          M850 THE RELATION OF SOLUBLE ADHESION MOLECULES TO
for statistical analyzes and the level of significance was set at p<0.05.                   MICROALBUMINURIA IN EGYPTIAN CHILDREN WITH
Regarding clinical aspects, the diabetic patients exhibited an average gly-                INSULIN DEPENDENT DIABETES MELLITUS
cohemoglobin level of 9.5 ± 0.4% and a 7.3 ± 0.7 years of the disease.
                                                                                 Hesham Safouh1 , Hesham Elhefnawy2 , Ghada Anwar1 , Nehad Mosaad3 ,
The PRA levels were significantly higher among the diabetic group in
                                                                                 Ibrahim Ibrahim2 . 1 Pediatrics, Pediatric Nephrology Unit, Cairo
comparison to the control subjects (1,81 ± 0,12 vs. 0,40 ± 0,22 ng Ang
                                                                                 University, Cairo, Egypt; 2 Pediatrics, National Institute of Diabetes and
I/ml/h, p<0.05). The same pattern was also found when we compared the
                                                                                 Endocrinology, Cairo, Egypt; 3 Clinical Pathology, Cairo University,
angiotensins plasma concentrations: Ang I – 76.7 ± 9.9 vs 26.4 ± 13.7
                                                                                 Cairo, Egypt
pg/ml; Ang II – 76.4 ± 6.1 vs. 21.4 ± 8.7 pg/ml and Ang-(1-7) 52.2 ± 5.8
vs 16.2 ± 7.9 pg/ml (p< 0,05 for all comparisons).                               The aim of this study was to determine the levels of soluble vascular
The preliminary data indicate an overall activation of the Renin An-             adhesion molecule-1 (sVCAM-1) in diabetic children and their relation
giotensin System in pediatric patients affected by diabetes mellitus.            to microalbuminuria.
                                                                                 The study included 30 children (18 females), 8 – 15 years old (mean =




                                                                                                                                                                 Downloaded from http://ndt.oxfordjournals.org/ by guest on May 1, 2013
                                                                                 12.3 ± 1.6 years) with a duration of diabetes longer than 5 years. There
    M849 OXIDATIVE STRESS AND GLOMERULAR                                         were 10 healthy age- and sex-matched controls. sVCAM-1 measurement
              HYPERFILTRATION IN YOUNG DIABETIC PATIENTS WITH                    was carried out using a solid phase ELISA method. Other lab studies in-
              GOOD GLYCEMIC CONTROL                                              cluded measurement of fasting blood sugar, HbA1c, and urinary albumin
                                                                                 to creatinine ratio.
Roberto Hernández1,2 , Sara Pons1 , Victoria Valls2 , Pilar Codoñer2 ,
                                                                                 SVCAM-1 was significantly elevated in diabetic children versus controls
Juan Marín1 , Jaime Fons2 , Laura Boix2 , M. Carmen Torres2 . 1 Unidad de
                                                                                 (859.5 ± 83.3 vs. 611 ± 93.5 ng/mL, p < 0.001). There was a positive
Diabetes Infantil, Hospital Dr. Peset, Valencia, Spain; 2 Dept. Pediatría,
                                                                                 correlation between sVCAM-1 levels and microalbuminuria (r = 0.786, p
Obstetricia y Ginecología, Universidad de Valencia, Valencia, Spain
                                                                                 < 0.001 and between sVCAM-1 levels and the duration of diabetes (r =
We studied the relationship between glomerular hyperfiltration (CCr > 150         0.469, p < 0.05). There was a positive but non-significant correlation be-
ml/min/1,73m2 ) and oxidative stress in children and adolescents with well-      tween sVCAM–1 and HbA1c levels, fasting blood sugar, body mass index
controlled diabetes mellitus type-1 (DM1) in early stage of disease, without     and mean arterial blood pressure. All the diabetic children with positive mi-
clinical microangiopathy. 13 (8 males) DM1 young patients (mean age 13.5         croalbuminuria also had elevated sVCAM-1 levels and 13 diabetics were
± 5.4 y) with mild glomerular hyperfiltration (CCr>150 ml/min/1.73 m2 ),          positive for sVCAM-1 though negative for microalbuminuria.
normal blood pressure and without clinical retinopathy were compared             In conclusion, sVCAM-1 levels may serve as an early and useful marker
with 16 (9 males), normal CCr, and well-controlled DM1 young patients            for renal involvement in children with diabetes.
(mean age 14.2 ± 4.0 y). 13 healthy sex- (6 males) age-matched (mean
age 13.8 ± 4.6 y) children and adolescent was included as a control group.
Glomerular filtration (CCr) and albumin excretion rate (AER) was mea-              M851 PRELIMINARY EXPERIENCES WITH RECOMBINANT
sured in 24-h urine samples. The antioxidant status in blood was estimated                 URATE OXIDASE (FASTURTEC) FOR PREVENTION OF
by glutathione peroxidase activity (GPx) and reduced glutathione (GSH) in                  HYPERURICEMIA IN CHILDREN WITH LEUKEMIA OR
erythrocytes. The lipid peroxidation was estimated by thyobarbituric acid                  LYMPHOMA
reactive substances (TBARS) and oxidative damage to protein by carbonyl
                                                                                 Edit Bardi1 , Istvan Szegedi1 , Erzsebet Udvardi2 , Csongor Kiss1 ,
group (PCG) levels in plasma. Statistical analysis was carried out with the
                                                                                 Imre Renyi3 . 1 Pediatrics, University of Debrecen Medical and Health
SPSS package. The results (mean ± d.e.) are included in the table:
                                                                                 Scince Center Medical School, Debrecen, Hungary; 2 Sanofi-Synthelabo
                                                                                 Hungary Co. LTD., Budapest, Hungary; 3 1st Pediatrics, Semmelweis
Clinical and biochemical parameters
                                                                                 University, Faculty of Medicine, Budapest, Hungary
                              Control group     Normal GFR     Hyperfiltration
                                 n=13)            (n=16)          (n=13)         Tumor lysis syndrome and renal failure remain important complica-
Age (y)                         13.5±1.7          14.2±4.0         13.5±5.4
                                                                                 tions early in the course of therapy for pediatric Non-Hodgkin’s lym-
Onset diabetes (y)                                 9.6±3.1          8.4±4.3      phoma (NHL) and acute leukemia. Standard prophilaxis and treatment of
Diabetes duration (m)                            55.4±33.2        62.6±37.3      malignancy-associated hyperuricemia has been allopurinol with vigorous
Insulin (IU/kg/day)                              0.93±0.36        0.96±0.33      hydration, urinary alkalization and osmotic diuresis. To improve the control
HbA1c (%)                      5.00±0.46         7.49±0.621       7.65±0.981     of hyperuricemia, we tested a newly developed uricolitic agent, recombi-
CCr (ml/min/1.73 m2 )         112.11±6.73       107.39±15.09   178.38±25.401 2
                                                                                 nant urate oxidase (Rascubinase, Sanofi-Synthelabo, Paris, France), which
AER (µg/min/1.73 m2 )          3.47±2.42         5.82±2.90      37.15±39.9112
GPx (U/g Hb)                  90.25±37.34       103.88±51.86     89.00±25.28     catalizes the oxidation of uric acid to allantoin, a highly water soluble
GSH (µmol/g Hb)                1.63±0.38         1.61±1.06        1.70±0.92      metabolite readily excreted by the kidneys.
TBARS (nmol/ml)               23.45±7.32        33.80±12.92     34.70±10.931     From 8/2002 to 1/2003 12 patients with newly diagnosed leukemia or
PCG (nmol/mg protein)          1.21±0.23         1.57±0.461       1.86±0.631     lymphoma, who either presented with abnormally high plasma uric acid
1
    p<0.05 vs Control group. 2 p<0.05 vs Normal GFR                              concentrations or had large tumor cell burden were treated by 0.2 mg/kg
                                                                                 Rascubinase for consecutive 5 days intravenously. Blood levels of uric
When we compared with the control group, DM1 patients showed a signi-            acid, creatinine, phosphorus, calcium, LDH, complete blood count were
ficative increase (p<0.05) of lipid peroxidation (TBARS = 34.20 ± 11.87           measured every day during RascubinaseÒ treatment and on days 6, 7 and
nmol/ml) and increased (p<0.05) oxidative damage to protein (PCG = 1.64          12 of the chemotherapy. The laboratory findings were compeared retro-
± 0.70 nmol/mg protein) despite good glycemic control (HbA1c = 7.5 ±             spectively by data of 6 other patients, who were treated with allopurinol.
0.8%). The DM1 patients with hyperfiltration, that was accompanied of a           There were no statistically significant differences in the age and male-
mild microalbuminuria in 3 patients (AER < 100 mg/min/1,73 m2 ), showed          female ratios between the 2 groups. The initial mean WBC counts
a no-significant difference with respect to DM1 patients with normal CCr.         was133G/l in the Rascubinase group and 152 G/l in the allopurinol group.
In all DM1 patients (normal or with hyperfiltration) there was a positive         In neither of the patients developed tumor lysis syndrome or required dial-
Monday, June 9                                                  E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                    273
                                                                                   Mode of presentation
ysis inspite of the high tumor cell burden in the Rascubinaseâ group (max
P: 2.33mmol/L, max LDH 9760U/L, max uric acid 1059mmol/L). The uric                Mode of presentation                                     Number of patients
acid level normalised quickly, 4 hours after the 1st Rascubinase infusion.         Incidental finding                                      30 (2 on school medical)
Serum creatinine level was within normal range during the treatment and            Headache ± vomiting                                               16
                                                                                   Cardiac manifestations                                             8
the follow up period (max 89mmol/L).In contrast, 3 patients in the allop-
                                                                                   Failure to thrive                                                  6
urinol group, had elevated creatinine levels (max 144 mmol/L) and one              Encephalopathy                                                     6
of the patients required dialysis. The tumor burden markers in this latter         Facial palsy                                                       4
group were high (max.P 3.33 mmol/L, max. LDH 20560 U/L, max. uric                  Screening (syndrome associated with hypertension)                  3
acid levels (785mmol/L) until days 10-14.                                          Vomiting alone                                                     2
                                                                                   Hemiparesis                                                        2
No toxic or allergic event was registered in connection with the Rascubi-
                                                                                   *CVA/TIA                                                           2
nase treatment.                                                                    Post cardiac surgery                                               2
Rascubinase is a safe and highly effective for the prophilaxis of hyperuri-        Other CNS (’Dizziness’)                                            1
caemia in pediatric patients sufferring from leukemia and lymphoma.                *Cerebrovascular accident/Transient ischaemic attack
This work was supported by Sanofi-Synthelabo, Paris, France.

                                                                                   31 (35.7%) underwent therapeutic intervention: 24 angioplasties with 10
 M852 USE OF CLEAN INTERMITTENT CATHETERIZATION (CIC)                              successful, leading to either improvement or cure of blood pressure (BP)
           IN PREVENTING CHRONIC RENAL DAMAGE IN CHILDREN                          or reduction of requirement for antihypertensive agents (one death linked
           WITH NEUROGENIC BLADDER DUE TO                                          to the procedure occurred); 4 renal artery stentings (2 patients) with nei-
           MYELOMENINGOCELE                                                        ther achieving long-term improvement in BP control. Revascularization of
                                                                                   17 kidneys was undertaken with, in 11, BP improvement. There were 13
Randall Lou1 , Evelyn Valenzuela2 . 1 Pediatric Nephrology, Instituto
                                                                                   nephrectomies that resulted in either cure or improvement in BP control.




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Guatemalteco de Seguridad Social, Guatemala, Guatemala; 2 Pediatrics,
                                                                                   Over this period 5 of these patients died, although not all deaths were
Instituto Guatemalteco de Seguridad Social, Guatemala, Guatemala
                                                                                   attributed to RVD.
Neural tube defects, specially myelomeningocele, are common congenital             RVD in childhood is a relatively uncommon, but important problem with
problems in Guatemala, as well as in other developing countries.                   significant morbidity and mortality, but potential for curative intervention.
One of the complications seen in patients with neural tube defects is the          However only one third of affected children have lesions that are amenable
neurogenic bladder, that leads to dyssynergy, increased intravesical pres-         to intervention and there is varying success from this. Angioplasty may
sure, reflux, and reflux nephropathy. The cause of renal function deterio-           cure hypertension, but some patients need surgical intervention, and others
ration can be explained by this mechanism in close to 50% of our patients          long term medical treatment. The benefit of stenting in paediatric RVD still
with chronic renal failure.                                                        needs to be established.
The aim of the study was to determine the cost effectiveness of CIC, it’s
feasibility and acceptance among Guatemalan families, and to compare the
clinical course of a group of patients before and after the initiation of CIC.      M854 BISPHOSPHONATES FOR TREATMENT OF
Eighteen patients (10 males, mean age 5 years) with neurogenic bladder                        HYPERCALCEMIA SECONDARY TO ACUTE VITAMIN D
due to myelomeningocele were followed for 12 months. The mothers were                         INTOXICATION
trained to perform the hand washing and the catheterization. Before CIC
                                                                                   N. Yildiz1 , A. Fedakar1 , M. Yildiz2 , U.Y. Denizmen1 , M. Erguven1 .
our patients had 5 febrile UTIs/year (range 2-10) and 4 had dilated up-            1
                                                                                     Department of Pediatric Nephrology; 2 Pediatric Endocrinology, SSK
per urinary tract. After CIC, all ultrasounds were normal, 13 (72%) had 2
                                                                                   Goztepe Education Hospital, Turkey
or less febrile UTIs/year (5 were UTI free) and the serum creatinine de-
creased in all. The CIC was well accepted and the complications reported           Vitamin D intoxication is a rare cause of hypercalcemia, which is associated
included: mild irritation of the external genitalia (6) and transit macro-         with severe and prolonged morbidity. Biological half-life of vitamin D is
scopic hematuria (2).We conclude that CIC is feasible and well accepted            long, and hypercalcemia and/or hypercalciuria may persist for months
among Guatemalan population and has shown to be cost effective and a               We report our experience in the use of intravenous and oral bisphospho-
good therapeutic option in preventing the development of chronic renal             nates, for the treatment of severe, prolonged symptomatic hypercalcemia
failure in patients with neural tube defect.                                       with hypercalciuria and nephrocalcinosis secondary to vitamin D intoxica-
                                                                                   tion in two children.
                                                                                   Case 1: 4 month-old girl admitted with fever, lethargy, vomiting and dehy-
 M853 CHILDHOOD RENOVASCULAR DISEASE - 30 YEARS                                    dratation. She was given 300 000 U/day vitamin D, twice in a week, while
           EXPERIENCE                                                              she was taking 400 U/day.
                                                                                   Serum calcium level was 15 mg/dl, phosphorus 3 mg/dl, parathormone
Suzanne Stephens, Vanita Shah, Michael Dillon. Nephrourology, Institute
                                                                                   (PTH) 4.1 pg/ml (9-55), 25-OH-D3 >160 ng/ml (10-68) and 1,25-(OH)2
of Child Health, London, United Kingdom
                                                                                   -D3 level was 187 ng/ml (45-270). Urinary calcium/creatinine ratio was
10% of patients with hypertension, needing treatment, presenting to major          2.1. Renal ultrasonography showed bilateral nephrocalcinosis.
paediatric nephrology centres have renovascular disease (RVD). To estab-           At 50th day of admission symptomatic hypercalcemia and hypercalciuria
lish the spectrum of presentation, anatomical variation, treatment choices         persisted despite the management with prednisone, high intravenous fluid
and outcome in a very large series of children with RVD we conducted               saline intake, furosemide and calcitonin. Therefore, single daily infusions
a retrospective case note review of patients treated in one centre over a          of 1 mg/kg (5.5 mg/day) intravenous pamidronate (Aredia ) was adminis-
30 year period. Patients were identified by review of ward discharge sum-           tered. 18 doses of pamidronate was given because of the persistant hyper-
maries and by the unit database.137 patients were identified and to date            calcemia. Serum Ca level was 13.9 mg/dl after the last dose of pamidronate.
data for analysis was available on 98 patients (61 male).                          Oral alendronate (Fosamax ) was given at a dose of 5 mg/day for ten days
In 18% of these patients a syndrome known to be associated with RVD                and her serum calcium levels was normalized.
was identified. Detailed data on presenting features was available on 82            Case 2: 8 month-old girl was admitted with symptomatic hypercalcemia.
patients, with presentation occurring between the ages of 10 days and 17           She was given 300 000 U/day for three times in a month. Her serum calcium
years with a mean of 6.98 years.                                                   level was 15.6 mg/dl, phosphorus 3.2 mg/dl, PTH 26.3 pg/ml, 25-OH-D3
In addition to these features 6 patients reported visual disturbance.              207.2 ng/ml and 1,25-(OH)2 -D3 level was 35.8 pg/ml (18.7-47.7). Urinary
Arteriography revealed unilateral main and/or intra renal artery disease in        Calcium/creatinin ratio was 1.32. Renal ultrasonography showed bilateral
37 patients, bilateral main and/or intra renal artery disease in 40 patients and   medullary hyperechogenity. On day 6 of hospitalization, serum calcium
multi-site arterial disease (including mid aortic syndrome) in 21 patients.        level was 18.8 mg/dl despite the treatment with prednisone, calcitonin,
11 patients (11%) had cerebrovascular disease (CVD) but only a proportion          forced diuresis and furosemide. Intravenous pamidronate (Aredia ) at a
of the total number of patients underwent CVD investigation.                       dose of 1 mg/kg/day for 4 days was administered and serum calcium level
274      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                        Monday, June 9

was reduced to 13.8 mg/dl. A dose of 10 mg of oral alendronate was               M856 DOES LATE REFERRAL TO A NEPHROLOGIST
successfully used to maintain normocalcema in the patient for 6 days.                     CONSTITUTE A PROBLEM IN CHILDREN STARTING
Serum calcium level was reduced to 10.2 mg/dl and remained in normal                      RENAL REPLACEMENT THERAPY IN POLAND?
range.                                                                                    A NATIONWIDE STUDY
Conclusion;: The administration of bisphosphanates are effective in the
                                                                                Michal Nowicki1 , Anna Jander1 , Marcin Tkaczyk1 ,
treatment of severe hypercalcemia secondary to vitamin D intoxication
                                                                                Maria Roszkowska-Blaim2 , Tomasz Jarmolinski3 , Ewa Marczak4 ,
and the treatment is well tolerated with no significant side effects.
                                                                                Ewa Paluba5 , Jacek A. Pietrzyk6 , Grzegorz Siten7 , Roman Stankiewicz8 ,
                                                                                Krystyna Szprynger9 , Malgorzata Zajaczkowska10 , Jacek Zachwieja11 ,
                                                                                Walentyna Zoch-Zwierz12 , Danuta Zwolinska13 . 1 Pediatric Nephrology,
 M855         CLINICAL ASPECTS OF PLASMAPHERESIS THERAPY
                                                                                Centers, In Lodz; 2 Warszawa; 3 Szczecin; 4 Gdansk; 5 Sandomierz;
              IN CHILDREN – EXPERIENCE WITH SIX HUNDREDS                        6
                                                                                  Krakow; 7 Rzeszow; 8 Torun; 9 Zabrze; 10 Lublin; 11 Poznan; 12 Bialystok;
              SESSIONS                                                          13
                                                                                   Wroclaw, Poland
Jacek Rubik, Ryszard Grenda, Sylwester Prokurat, Katarzyna Jobs,
                                                                                Late referral (LR) of patients with chronic renal failure to a nephrologist
Ewa Smirska, Joanna Latoszynska, Mieczyslaw Litwin, Bogdan Materna.
                                                                                adversely affects their prognosis on renal replacement therapy (RRT). It
Nephrology & Kidney Transplantation, Children’s Memorial Health
                                                                                has been reported that 20-50% of adults started RRT without a prior visit
Institute, Warsaw, Poland
                                                                                to a nephrologist. The aim of this present nationwide survey was to assess
Data concerning 636 plasmapheresis sessions (indications, anticoagula-          the clinical and biochemical status of children at the start on RRT in re-
tion, supplement and complications) in children at age from 3 months            gard to timing of nephrologist referral. Based on questionnaires filled by
to 20 years (mean body weight = 34,2kg ±12 [min. 5kg, max. 75kg])               physicians in 13 (of all 14) pediatric dialysis centers in Poland, we have an-
performed between 1990 and 2002 were analyzed. Number of sessions               alyzed the data of 180 children on chronic dialysis in the years 2001-2002
ranged from 1 (intoxication) to 22 (glomerulonephritis recurrence after         (mean age 14±6 yrs). Patients were classified as early referrals (ER) or




                                                                                                                                                                 Downloaded from http://ndt.oxfordjournals.org/ by guest on May 1, 2013
kidney transplantation) per patient. Indications: Glomerulonephritis – 207      late referrals (LR) depending on whether they started first RRT more than
(32,5%)[recurrence after kidney transplantation – 119, rapidly progressive      1 month after the first referral to a pediatric nephrologist. The percentage of
glomerulonephritis – 74, other types – 14), hemolytic-uremic syndrome –         LR was 21% (37 pts) and ER 79% (143 pts). The LR patients had a signif-
128 (20,1%) [after kidney transplantation – 65, atypical – 63], other im-       icantly higher serum level of urea at the start of dialysis (39±28 mmol/l vs
mune diseases – 123 (19,3%)[systemic lupus erytematosus - 42, Guillain-         28±9 mmol/l; p<0.001), but there were no differences in the serum creati-
Barre syndrome - 34, Wegener’s granulomatosis - 22, myasthenia - 20,            nine. The renal clearance of creatinine (9.4±4 vs 4.1±2.9 ml/min/1.73m2
others - 5], intoxication - 122 (19,2%)[amanita poisoning - 100, poison-        p<0.001) and the residual diuresis (1261±855 vs 901±637 ml; p<0.05)
ing with albumin-bound drugs – 22], acute hepatic encephalopathy – 42           were higher in ER group. These patients had also higher Ht and Hb levels
(6,6%)[acute hepatic failure -33, after liver transplantation – 9] and com-     (28±5 vs 25±7%; p<0.001 and 9.3±1.6 vs 8.4±5.9 g/dl, respectively;
plications in kidney graft recipients – 14 (2,2%)[acute vascular rejection-     p<0.05). ER had higher calcium (2.3±0.5 vs 1.9±1.4mmol/l; p<0.0001)
9, parathormon toxicity - 5]. Within the period 1990-2002 the profile of         and lower serum phosphate levels (1.9±0.6 vs 2.5±1.8 mmol/l; p<0.0001),
treated patients has changed (graph).                                           The Ca x P product did not differ between groups. A higher percentage of
                                                                                LR patients started RRT with a temporary vascular access (67.6% vs 22%
                                                                                in ER; p<0,0001). Their clinical status was worse, i.e. hypertension was
                                                                                found in 75.7% (vs 57.3% in ER), pulmonary edema in 29.7% (vs 5.6%
                                                                                in ER) and they were more often treated in an ICU (24% vs 0.7% in ER).
                                                                                Peritoneal dialysis was the first method of RRT in 59% of ER and 46%
                                                                                of LR (NS). The ER were treated more frequently with calcitriol (84%),
                                                                                phosphate binders (89%) and received dietary recommendations (92%).
                                                                                Only 20% of ER were treated with erythropoietin in the predialysis pe-
                                                                                riod (vs none in LR). The results of this survey show that late referral to
Anticoagulation: till the end of 1999 - unfractionated heparin (divided dose    a pediatric nephrologist is still a great problem in children starting RRT
each 30 min. - mean 103 IU/kg/session), from 2000 single dose of Fraxi-         in Poland. It is associated with poorer clinical condition and more severe
parine (mean 74 aXaU/kg/session). Supplement: till 1995 Ringers solution        metabolic disturbances. Thus, further efforts to improve referral patterns
+ fresh frozen plasma, from 1996 Ringer solution + fresh frozen plasma          are needed.
or 4% albumin solution diluted in 5% dextrose (depending on indications,                                                         Free Communication June 10
contraindication and fibrinogen concentration). There was no death in the
course of plasmapheresis. Complications (rush, abdominal pain, headache,
nausea and vomiting, blepharoedema, drop in arterial pressure, pruritis) –       M857         EFFICACY OF RAMIPRIL MONOTHERAPY IN CHILDREN
22 (3,5%), withholding the ongoing procedure - 35 (5,5%): due to techni-                      WITH CHRONIC RENAL FAILURE
cal problems – 28 (4,4%)[filter damage – 8, membrane occlusion (exces-
                                                                                Elke Wühl, Otto Mehls, Franz Schaefer, ESCAPE Trial Group. Pediatric
sive TMP) –8, machine break-down – 5, filter clotting – 4, problems with
                                                                                Nephrology, University Children’s Hospital, Heidelberg, Germany
vascular access – 3), deteriorating patient’s clinical condition (dyspnoea,
urticaria, hypotension) – 4 (0,6%) and hemolysis - 3 (0,5%).                    Systemic hypertension and glomerular hyperfiltration with resulting pro-
In spite of progress in blood purification techniques plasma exchange is still   teinuria and activation of vasoactive, profibrotic and proinflammatory sys-
effective and safe method of treatment of pediatric patients with immune-       tems have been identified as major causes of nephron damage and progres-
mediated diseases and intoxications.                                            sive chronic renal failure. ACE inhibitors are not only potent antihyperten-
                                                Free Communication June 9       sive agents but also reduce proteinuria, glomerulosclerosis and tubulointer-
                                                                                stitial fibrosis via reduction of the local angiotensin tone in the kidney. Our
                                                                                consortium of 33 pediatric nephrology centers is currently investigating the
                                                                                renoprotective efficacy of ACE inhibition and intensified antihypertensive
                                                                                treatment in more than 350 pediatric patients with chronic renal failure and
                                                                                high normal or elevated blood pressure.
                                                                                Here we report on the effects of six months ramipril monotherapy in a
                                                                                subgroup of 69 children. Mean age was 11.5 (3.6-16.8) years and mean
                                                                                GFR 46 (14-75) ml/min/1.73m2 . More than two third of patients suf-
                                                                                fered from hypo/dysplastic kidney disease. Following a two-month ACE
                                                                                inhibitor wash-out period, ramipril monotherapy was started with an in-
                                                                                crementing dose schedule, aiming at a fixed single daily dose of 6 mg/m2
Monday, June 9                                                 E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                  275

after 8 weeks in all children. At baseline and after 6 months of treatment        with improvement (p<0,02). The fittest model of crf progression in path
ambulatory blood pressure monitoring was performed and urinary protein            analysis included systolic BP and anthropometric parameters.
excretion was measured.                                                           Conclusions: in unselected group of pts with crf of non-glomerular origin
At baseline mean 24-hour systolic (SBP), diastolic (DBP) and mean ar-             and nil to moderate proteinuria the main risk factors of crf progression
terial blood pressure (MAP) were 119 (+1.0 SDS), 72.7 (+1.1 SDS) and              is rapid somatic growth, age and systolic BP. AH and proteinuria even of
88.6 mmHg (+1.4 SDS), respectively. Under ramipril treatment mean SBP,            mild intensity differs significantly groups with progression and stable renal
DBP and MAP decreased by 7.2 (1.0 SDS), 6.5 (1.2 SDS), and 6.5 mmHg               function or improvement. Renoprotective therapy with ACEi is correlated
(1.2 SDS), respectively. Ramipril reduced blood pressure with equal effi-          with significant slowing of crf progression but persistent proteinuria even
cacy during day- and nighttime. Patients with a baseline MAP above the            of mild intensity is risk factor for resistance to renoprotective effect.
95th centile showed the best antihypertensive response with a mean MAP
reduction by 11 mmHg (2.0 SDS), whereas patients < 95th MAP centile
at baseline responded less markedly (-4.3 mmHg = 0.8 SDS). The depen-              M859 RENAL FUNCTIONS IN CHILDHOOD OBESITY
dence of ramipril’s antihypertensive efficacy on the prevailing degree of
                                                                                  Oguz Soylemezoglu, Elif Cingi, Esin Ozgur, Aysun Bideci, Mustafa Unlu.
hypertension was confirmed by a linear correlation of baseline MAP with
                                                                                  Pediatric Nephrology and Endocrinology, Gazi University Hospital,
treatment induced MAP reduction (r = 0.63; p < 0.0001). Urinary pro-
                                                                                  Ankara, Turkey
tein excretion was reduced by ramipril treatment by 49% on average. The
antiproteinuric effect of ramipril was independent of its antihypertensive        Obesity initiates a complex cascade of cardiovascular, metabolic and renal
efficacy. During the first six months after initiation of ramipril monotherapy      disorders such as hypertension, atherosclerosis, diabetes, and chronic renal
GFR (Schwartz Formula) declined by 1.9 ml/min, compared to 3.8 ml/min             failure. Abnormal kidney function plays a role in the aetiology of obesity
in the 6-month pretreatment period. Serum potassium levels increased by           hypertension and this condition is an important risk factor for end-stage
0.43 ± 0.6 mmol/L. In two patients ramipril treatment was discontinued            renal disease. Various abnormalities have been suggested to alter renal
due to symptomatic hypotension or hyperkalemia.                                   function in obesity including hyperinsulinemia and insulin resistance, ac-




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In conclusion, ramipril is a very effective antihypertensive and antipro-         tivation of sympathetic nervous sytem and structural changes within the
teinuric agent in children with CRF-associated hypertension. The blood            kidney. The aim of this study is to evaluate the renal functions of obese
pressure lowering effect is dependent on the degree of hypertension at            children with or without hypertension and also try to investigate the some
baseline, with the best response in severely hypertensive children.               of various factors contributing to hypertension in obese children.
                                               Free Communication June 11         A total of 31 obese children ranging from 6 to 16 years were included
                                                                                  in the study. All overweight subjects had a body mass index above the
                                                                                  95th percentile. No severely obese subject (BMI 40 kg/m2 ) was included.
 M858 RISK FACTORS OF CHRONIC RENAL FAILURE                                       Ambulatory blood pressure measuments were performed in all the ubjects
           PROGRESSION IN CHILDREN WITH CHRONIC,                                  during a school day with a Spacelabs model 90207 monitor Sixteen of the
           NON-GLOMERULAR NEPHROPATHIES AND NIL TO                                study population were found to be hypertensive according to the SBP, DBP
           MODERATE PROTEINURIA                                                   and mean arterial pressure (MAP) values and as expected these parameters
                                                                                  were significantly different from the normotensive group. Most of the sub-
Mieczyslaw Litwin. Dept. of Nephrology & Kidney Transplantation, The
                                                                                  jects had hyperfiltration (CrCl > 130 ml/min 1.73 m2 ) according to their
Children’s Memorial Health Institute, Warsaw, Poland
                                                                                  age and this finding was also prominent when we consider the estimated
Aim of the study: analysis of progression of chronic renal failure (crf),         GFR. Estimated GFR was increased in all the subjects but there were no
effects of modification of risk factors for progression and development of         difference between the hypertensive and normotensive group. Urinary al-
theoretical model describing crf progression in unselected group of chil-         bumin excretion did not differed in two groups when we adjust it also as
dren with chronic, non-glomerular nephropathies and nil to moderate pro-          albumin creatinine ratio (ACR) but 29% of the patients had microalbu-
teinuria.                                                                         minuria.There were no difference in the serum lipid parameters and also
92 children with crf and low-level proteinuria (GFR: 43 ±18                       in the fasting glucose concentrations. Although the fasting insulin levels
ml/min/1,73m2 ; proteinuria 0,57 ±1 g/day), 34 girls and 58 boys, in mean         tend to be high in the hypertensive group there was no significant differ-
age 9,2 ±5,8 yrs. The etiology of crf was congenital uropathy in majority         ence (18,7±14,7 IU/ml versus15±8,5 IU/ml, p>0.05). When we divide the
of pts. There were no pts with glomerulopathies. Study design: cross-             study population according to insulin resistance (IR) and then check the
sectional, retrospective. Inclusion criteria: established diagnosis and end of    different parameters we again did not find significant difference between
urological treatment before inclusion to the analysis. 69 children observed       the various parameters including the MAP and renal vascular resistance.
for longer than 3 yrs were divided in two groups: one with progression of         We found no correlation between the mentioned parameters including the
crf and second with stable course or improvement of kidney function. 43           microalbuminuria, insulin resistance and blood pressure values. Dyslipi-
children were on renoprotective therapy with ACEi.                                demia was not a prominent finding in the study population. Lipid param-
Results: during 3 yrs of observation crf progressed in 39 children and            eters: Cholesterole (166.6±38.2 mg/dl), Triglyseride (101.7± 50.7mg/dl).
in 30 children the course of crf was stable or kidney function improved.          There was no significant difference for MAG3 scintigraphy findings be-
There were no differences in etiology of crf between groups. Children             tween hypertensive and normotensive group.
with progression of crf were older (p<0,08), had faster rate of growth            Our study populatian admitted to the hospital for the first time and we
(p<0,004), higher blood pressure (BP) (p<0,01) and were more often                followed them for a short period. Hyperfiltration of kidneys and in half of
proteinuric (p<0,03). Arterial hypertension (AH) in pts with progressive          the subjects hypertension were the prominent findings. We need to follow
crf was resistant to therapy and these pts needed more intensive treatment.       up these subjects for a long period till the adulthood in order to see the
The main risk factors for progression were older age and increase in body         renal effects of obesity and hypertension including the renal histology and
surface area which explained 30% of variability of crf progression. After         cardiovascular changes.
excluding anthropometric variables from the analysis, the main risk factor
for progression was increase in kidney length. Systolic BP explained 17%
of variability and GFR at 1-st observation, increase in kidney length and          M860 SENSORNEURAL HEARING LOSS IN CHILDREN WITH
change in body surface area together determined 65% of variability of GFR                   END-STAGE RENAL DISEASE
after 3 yrs. ACEi therapy led to improvement of kidney function in 50% of
                                                                                  Seyed Taher Esfahani1 , Abbas Madani1 , Nematollah Ataei1 ,
treated pts but increase in body surface area was the risk factor of resistance
                                                                                  Parvin Mohseni1 , Ziba Ghanbari1 , Abbas Nadimi Tehrani2 . 1 Department
to renoprotective effect and this factor determined 22% of variability of
                                                                                  of Pediatric Nephrology, Tehran University of Medical Sciences, Tehran,
crf progression among pts on renoprotection. In pts treated with ACEi
                                                                                  Iran; 2 Department of Otorhinolaryngology, Tehran University of Medical
kidney function stabilized in contrast to pts not treated with renoprotective
                                                                                  Sciencess, Tehran, Iran
drugs (p<0,007) as well as proteinuria (p<0,05) and BP (p<0,02) lowered
despite higher basal proteinuria and BP. In pts on renoprotection in whom         To determine the prevalence of sensorineural hearing loss (SNHL) in chil-
crf progressed despite treatment proteinuria was persistent in contrast to pts    dren with end-stage renal disease (ESRD) and it’s relationship with sex of
276      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                         Monday, June 9

patients,presence of hypertension,history of ototoxic drugs administration       M862 THE COMPARISON OF SERUM CREATININE AND SERUM
and duration of dialysis,we evaluated 30 children and adolescents suffering                CYSTATIN C FOR DETECTION OF RENAL FAILURE IN
from ESRD who were under hemodialysis therapy with standard pure-tone                      SPINA BIFIDA PATIENTS
audiometry.The ages of patients were 5-17.5yeas (mean 12 years). Patients
                                                                                Sevinc Cabukoglu1 , Harika Alpay1 , Nese Karaaslan Biyikli1 , Omid
with Alport’s syndrome or other heriditary or congenital syndromes in-
                                                                                A. Noshari2 , Guler Topcu3 . 1 Pediatric Nephrology, 2 Nucleer Medicine,
volving the ears were excluded from ths study.                                  3
                                                                                  Biochemistry, Marmara University Faculty of Medicine, Istanbul, Turkey
SNHL was detected in 9 of patients (30%).3 children had unilateral and
6 had bilateral SNHL (15 ears).In 100% of these 15 ears SNHL was in             Glomerular filtration rate (GFR) is considered as the best marker of renal
high frequencies (4000-8000 Hz)in 10 ears (66%) also there was SNHL             function. Although serum creatinine concentration is mostly used as an
in middle frequencies (1000-2000 Hz),and in 7 ears (46.6%) there was            indirect marker of GFR practically it’s not the most accurate method. Cys-
hearing loss in all frequencies (250-8000 Hz).The severity of SNHL was          tatin C, a cysteine proteinase inhibitor has been proposed as an indicator of
mild in7 ears, moderate in 9 ears,severe in 13 ears and profound in 3 ears      GFR since 1985. The aim of this study was to compare the clinical value of
in at least one frequency (many patients had SNHL in more than one fre-         serum cystatin C and creatinine in spina bifida patients whose muscle mass
quencies).There was no significant relationship between SNHL and sex             and related creatinine production is reduced. Serum creatinine, cystatin
of patients,prescence of hypertension,history of ototoxic drugs adminis-        C levels were determined and diethylenetriaminepentaasetic acid (DTPA)
tration,and duration of dialysis (P values were 0.8107,0.3217,o.69,0.087        clearance tests were performed in 43 spina bifida patients. Serum creatinine
respectively).                                                                  and cystatin C levels were compared by using DTPA clearance as the gold
Conclusions; SNHL is common in children and adolescents with ESRD,the           standard method. Patients were divided into 3 groups according to DTPA
origin of this impairment is not obvious,and must be considered [n rehabil-     clearance; Group I patients with GFR 30-49 ml/min/1.73 m2 (n: 5), Group
itation of these children.                                                      II patients with GFR 50-79 ml/min/1.73 m2 (n: 12), Group III patients with
                                                                                GFR greater than 80 ml/min/1.73 m2 (n: 26). Children with GFR lower
                                                                                than 80 ml/min/1.73 m2 were accepted as chronic renal failure patients.




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 M861 NUTRITIONAL ASSESSMENT OF CHILDREN WITH                                   The mean age of the study population was 40 ± 20.8 months (1 – 9 years).
          CHRONIC RENAL FAILURE: USEFULNESS OF                                  Chronic renal failure patients consisted of 17 patients due to DTPA clear-
          BIOELECTRICAL IMPEDANCE                                               ance, only two of them had serum creatinine greater than 1 mg/dl (11%).
                                                                                Serum cystatin C levels of these patients were high in 8 patients (47%).
Carla Simao, Fernando Batista, Zelia Patricio, Margarida Almeida.
                                                                                The upper limit of GFR that correspond to 80 ml/min/1.73 m2 in our study
Pediatric Clinic, Hospital de Santa Maria, Lisbon, Portugal
                                                                                was 0.4 mg/dl for creatinine and 1.1 mg/dl for cystatin C. A highly signifi-
Protein-energy malnutrition is present in a large proportion of pediatric       cant correlation (r=0.84) was found between serum creatinine and cystatin
patients with chronic renal failure (CRF), in nondialysed and dialysed pa-      C. The difference between the correlation coefficients of cystatin C-DTPA
tients and is associated with increased morbidity and mortality. Bioelectri-    (r=-0.50, p<0.001) and creatinine-DTPA (r=-o.52, p<0.0001) was not sta-
cal impedance, more rapid and practical, can be used to evaluate nutritional    tistically significant. To compare the diagnostic values receiver operating
status and body composition. The goals of our study were: 1) to evaluate        characteristic (ROC) analysis was performed and the areas under the curves
the nutritional status of paediatric patients with CRF by using anthropomet-    (AUC) were found similiar.
ric measurements and 2) these results were correlated with a bioelectrical      In this study it is concluded that cystatin C can also be used safely as
impedance analysis.                                                             an indicator of GFR in clinical practice especially in those patints with
We have investigated the nutritional status of 26 pediatric patients (2-19      reduced muscle masses.
years old) with chronic renal failure (Glomerular filtration rate less than 80
ml/min/1,73m2 - Schwartz formula), 16 on predialysis, 5 on hemodialysis
and 5 on peritoneal dialysis. Dietary intake of protein and calories was         M863 SKELETAL DISPROPORTION AND PUBERTAL STATUS IN
calculated from 3-day dietary diaries. Anthropometric indexes included:                    CHILDREN AND ADOLESCENTS WITH CHRONIC RENAL
height (Ht), body-weight (Wt), triceps skinfold thickness (TST) and mid-                   FAILURE
arm circumference (MAC). We used also nutritional index McLaren y Read
                                                                                Nadia Qayyum1 , Heather Maxwell2 , James Beattie2 , Ian Ramage2 ,
and Suckla, body mass index (BMI), Waterlow index, mid-arm muscle
                                                                                Faisal Ahmed1 . 1 Department of Child Health, Royal Hospital for Sick
circumference and mid-arm fat area calculated by using Gruney y Jelliffe
                                                                                Children, Glasgow, United Kingdom; 2 Renal Unit, Royal Hospital for
Normogram. Impedance index included resistance, reactance and % fat
                                                                                Sick Children, Glasgow, United Kingdom
estimated, lean body weight and fat mass.
Patients were divided by anthropometric parameters, into 4 groups: I- se-       The objective of the study was to assess stature, skeletal disproportion and
vere malnutrition; II- moderate/mild malnutrition; III-normal; IV- obese.       pubertal status in a cross-section of children and adolescents with chronic
Using nutritional index Mc Laren y Read and Suckla, 17 patients (65%)           renal failure (CRF) who were attending the renal failure clinic.
were severely or moderately malnourished (23% severe, 42% moder-                Height (HT), sitting height (SH), subischial leg length (SILL), sitting
ate/mild), 7 patients (27%) presented normal nutritional status and 2 (8%)      height/height ratio (SH:HT) and disproportion score (SH SDS minus SILL
were obese patients. Using BIE the value of fat mass calculated in the group    SDS) and Tanner staging were performed in 56 children (M:35) with me-
I were: 3,65 (+ 2,03); group II: 5,45 (+ 3,09); group III: 8,56 (+ 6) and in    dian age 11.4yrs (range, 4.5,18.7) with chronic renal failure. Thirty-one
the group IV: 9,78 (+ 5,6). Fat mass calculated by anthropometry and by         patients had received renal transplants, 6 were on peritoneal dialysis, and
impedance presented a strong correlation, evaluated by Pearson coefficient       19 children with moderately severe chronic renal failure were being treated
(r = 0,94; p < 0,0001). We conclude that bioelectrical impedance analysis       conservatively.
can be successfully used for the nutritional evaluation of CRF patients. Re-    The median HTSDS for the whole group was -1.21 (-2.8, 0.35). The me-
sults obtained with impedance were strongly correlated to those obtained        dian SH:HT ratio in non-transplanted children and in the renal transplant
with standard nutritional evaluation using anthropometric parameters. The       groups were 0.51 (0.49, 0.53) and 0.50 (0.48, 0.53), respectively (p=0.02).
results of fat mass increasing at the same time that increasing indexes and     The median disproportion score of the whole group was –3.2 (–4.8,–1.8).
body percentiles. However bioelectrical impedance remains to be defined          Only 12% of patients had a disproportion score over -2.0, and 46% had a
in pediatric population and in particular its use for prospective evaluation    score of less than -3.0. There was a significant correlation between dispro-
deserves further study.                                                         portion score and SH:HT ratio (r=0.5, p=0.005). SH:HT ratio was nega-
                                                                                tively related to the duration of illness (r=0.4, p=0.005). In the transplanted
                                                                                group, SH:HT ratio was inversely correlated with the age at transplantation
                                                                                (r = 0.4, p = 0.008).
                                                                                Pubertal assessment in 35 children was appropriate for age and only one
                                                                                boy and one girl had clearly delayed puberty. One girl was diagnosed as
                                                                                having precocious puberty.
                                                                                In conclusion, in this cross-sectional study of children with chronic renal
Monday, June 9                                                E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                  277

failure, disorders of puberty were not encountered commonly. However,           tients on dialysis). Patients were selected randomly from CRF and ESRD
these children and adolescents had significant body disproportion, which         children attending the Pediatric Nephrology unit, Cairo University. None
may be due to a disproportionately greater effect of disease and treatment      of the study patients was known to have clinically important pulmonary or
on spinal growth.                                                               chest wall disease. Assessment of the following pulmonary functions was
                                                                                carried out: forced vital capacity (FVC), Forced expiratory flow that occurs
                                                                                during the middle 50% of the effort (FEV25-75), peak expiratory flow rate
 M864 DISPROPORTIONATE GROWTH IN CHILDREN WITH                                  (PEFR), residual volume to total lung capacity ratio (RV/TLC), forced ex-
          CHRONIC RENAL FAILURE                                                 piratory volume in first second to forced vital capacity ratio (FEV1/FVC),
                                                                                and diffusion capacity of carbon monoxide adjusted for hemoglobin con-
Miroslav Zivicnjak1 , Doris Franke1 , Jochen H.H. Ehrich1 ,
                                                                                centration (DLcoAdj). Although patients were symptom free at the time of
Dieter Haffner2 , Uwe Querfeld2 . 1 Pediatric Nephrology, Children’s
                                                                                the study, nevertheless their pulmonary function tests, especially diffusion
Hospital, Medical School, Hannover, Germany; 2 Pediatric Nephrology,
                                                                                capacity, were deranged. The mean spirometric values of FVC, FEV25-75,
Charité University Hospital, Berlin, Germany
                                                                                PEFR, and RV/TLC, as well as DLcoAdj were found to be slightly lower
While the typical pattern of longitudinal growth in chronic renal failure       than predicted in all uremic patients. Statistically significant differences
(CRF) in childhood has been established already in the seventies, anthro-       regarding the same spirometric parameters were found between the non-
pometric data on age related sitting height, arm and leg length are lacking.    dialyzed uremic groups (1,2,and 3) and the ESRD group (4) (p <0.001,
We describe the effects of CRF on body morphology in 290 patients (111          <0.001, <0.01, <0.05, and <0.001 respectively) indicating progressive
females and 179 males, age range 3-22 years) in a prospective study. Mean       deterioration with declining GFR.
age was 11.7± 5 years at initial growth measurements. 149 patients were         Moreover, hemoglobin (Hb) had been positively correlated to FVC,
followed up for at least 1 year, 101 for 2, 67 for 3 and 32 for 4 years. At     FEV25-75, PEFR, and DLcoAdj (p <0.01, 0.01, 0.05, 0.001 respectively).
initial growth measurement, 103 patients had preterminal CRF, 39 were           DLcoAdj was as well positively correlated to albumin level in the stud-
on dialysis, and 148 patients had a functioning renal allograft. Fourty-eight   ied patients (p <0.05). Nevertheless, serum calcium showed significant




                                                                                                                                                                Downloaded from http://ndt.oxfordjournals.org/ by guest on May 1, 2013
percent of children had been treated with growth hormone or were currently      negative correlation to FVC and FEV25-75 (p <0.05). In conclusion, CRF
under treatment.                                                                patients had both ventilatory and diffusion disorders that got worse with de-
Methods: Anthropometric measurements were performed as recom-                   clining GFR, anemia and deranged biochemical profile. These alterations
mended by the International Biological Program. Calculation of Z-scores         were more pronounced in ESRD patients on hemodialysis. Therefore, pa-
(SDS), of the anthropometric parameters was performed from reference            tients with impaired renal functions need to be closely monitored to pre-
limits derived from 5115 school children aged 3 to 19 years.                    vent or at least retard progressive decline in GFR. Moreover, we believe
Results: The analysis revealed age related disproportionate growth in chil-     that more efforts should be made to optimize treatment of anemia and and
dren with a history of conservative treatment of CRF, dialysis and trans-       nutritional deficiencies paricularly in dialysed children. Renal transplanta-
plantation. Growth impairment and disproportion was most obvious in             tion should be advised early in the course of ESRD before the occurance
early childhood (mean leg length –2.53 SDS, mean sitting height – 1.15          of irreversible pulmonary damage.
SDS). Sitting height was preserved best and arm length (-1.43 SDS to -1.92
SDS) oscillated least from childhood to adulthood. The greatest dynamic
changes of body dimensions were observed for legs (mean SDS values in-           M866 ARTERIAL CHANGES IN PEDIATRIC PATIENTS WITH
creasing from –2.53 SDS in early childhood to –1.17 SDS in adolescence).                  END-STAGE RENAL DISEASE (ESRD)
Growth of legs was the best indicator of statural growth. The prepubertal
                                                                                Helena Ziolkowska1 , Michal Brzewski2 , Maria Roszkowska-Blaim1 .
period of growth was marked by catch up (mean height increasing from –          1
                                                                                  Department of Pediatrics and Nephrology, Medical University of
2.18 SDS to –1.50 SDS and mean leg length from –2.3 SDS to –1.58 SDS).
                                                                                Warsaw, Warsaw, Poland; 2 Department of Pediatric Radiology, Medical
Puberty showed “catch down” of growth until the age of 16 years, how-
                                                                                University of Warsaw, Warsaw, Poland
ever, late adolescence was correlated with catch up growth (mean height
increased from age of 16 years to 18 years by 0.35 SDS, mean leg length         The aim of the study was to determine the changes in arterial wall in chil-
by 0.39 SDS).                                                                   dren and young patients with ESRD. Twelve patients (6 girls, 6 boys) aged
Conclusions: The severity of growth failure in children with chronic renal      17 ± 4.7 years, 5 on hemodialysis (HD), 7 on peritoneal dialysis (PD)
failure was age related. The most vulnerable periods of life were infancy       were included in the study. Duration of dialysis therapy was 65.4 ± 34.2
and early childhood, followed by puberty. Growth failure in CRF led to          months. The control group consists of 14 healthy volunteers, aged 19.6 ±
disproportionate growth. The susceptibility to disproportion was also age       6.2 years. Examinations of carotid and femoral arteries were performed in
related. The presented growth patterns of different longitudinal body di-       all children by one radiologist using 12 MHz probe. The ultrasound exami-
mensions should help in the processes of evaluating the beneficial effect of     nations in B-mode and Color Doppler were performed. In all children were
rhGH and of searching for the optimal time of initiation of rhGH therapy.       estimated: total cholesterol (TC), HDL and LDL cholesterol, triglicerides
                                                                                (TG); in children with ESRD intact parathormone (PTH int - both 1-84
                                                                                amino acid native molecule, cyclase activating peptide - CAP and 7-84
 M865 PULMONARY FUNCTION ALTERATIONS IN CHRONIC                                 cyclase inactivating peptide -CIP), CAP, calcium (sCa), phosphorus (sP)
          RENAL FAILURE AND END-STAGE RENAL DISEASE                             and alkaline phosphatase (AP) activity. Calcium-phosphorus product (Ca
          EGYPTIAN CHILDREN                                                     x P), CIP and CAP/CIP ratio were calculated. Different structures (thick-
                                                                                ness, hyperechogenicity, irregularity) of intima-media-complex were found
Neveen Soliman1 , Bahia Moustafa1 , Samiha Samuel1 , Hany Gamal2 ,
                                                                                in arteries examinations in 10 out of 12 patients and none in the control
Azza Ashour1 . 1 Pediatric Department, Cairo University, Cairo, Egypt;
2                                                                               group. Atherosclerotic plaques in carotid artery were found in one patient
  Physiology Department, Cairo University, Cairo, Egypt
                                                                                aged 18 years. The analysed parameters were not significantly different
In chronic renal failure (CRF) and end-stage renal disease (ESRD) patients,     between patients on HD and PD. The TG plasma concentration was higher
mechanical and hemodynamic changes could occur in the lungs without ob-         (169 ± 50 vs 109 ± 48, p < 0.02, Mann-Whitney test) in dialysed patients
vious pulmonary symptoms or signs and might later progress to pulmonary         than in control group. The TC, HDL, LDL concentrations were not differ-
dysfunction. As little is known about pulmonary functional changes in           ent between groups. In dialysed patients PTH int was 224.4 ± 156 pg/ml,
CRF and ESRD children, we studied pulmonary functions in uremic pa-             CAP 108 ± 92 pg/ml, CIP 115.8 ± 70 pg/ml, Ca x P 57 ± 22, AP 189 ±
tients on conservative therapy as well as ESRD children on hemodialysis.        169 U/l. The CAP/CIP ratio was below 1 in 9 (3 on HD and 6 on PD) out of
Moreover, we investigated the relationship between these functions and          12 patients. In young patients with ESRD, lipids and calcium-phosphorus
clinico-laboratory parameters.Fifty patients (39 males and 11females; age       metabolism disturbances caused atherosclerotic changes, which were no-
range 9-18 years, Mean ± SD: 13.3 ± 2.7 years) were enrolled in this            ticed in ultrasound examination of carotid and femoral arteries.
study. They were grouped according to glomerular filtration rate (GFR) into
group1: GFR 74-50 (10 patients), group 2: GFR 49-30 (12 patients), group
3: GFR 29-10 (11 patients), and group 4: GFR <10 ml/min/1.73m2 (17 pa-
278      E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                                                        Monday, June 9

 M867 OUTCOME OF CONSERVATIVELY MANAGED CHRONIC                                 high risk pediatric and young adult patients with ESRD. Serial EBCT scans
          RENAL INSUFFICIENCY BY A INTERDISCIPLINARY                            may also provide a tool for assessing rates of progression of coronary
          PROTOCOL: A LONGITUDINAL STUDY                                        disease and timely therapeutic intervention. Further study is needed to
                                                                                develop normal standards in children and young adults.
Eleonora Lima, Jose Silverio Diniz, Cristina Bouissou, Gilce Rodrigues,
Monica Vasconcelos, Eduardo Oliveira, Patricia Vieira, Flavia Gazolla,
Flavia Silveira. Department of Pediatrics, Federal University of Minas
                                                                                 M869 SERUM ADIPONECTIN (S-ADPN) CONCENTRATION
Gerais, Belo Horizonte, MG, Brazil
                                                                                          INCREASES WITH THE SEVERITY OF CHRONIC RENAL
The objective of the present study was to describe the outcome of pa-                     FAILURE (CRF) IN CHILDHOOD
tients with chronic renal insufficiency (CRI) admitted to the Interdis-
                                                                                Kristina Zepf, Maike Westphal, Dirk E. Mueller-Wiefel. Pediatric
ciplinary Conservative Management Ambulatory (ICMA) from 1990 to
                                                                                Nephrology, University Children‘s Hospital, Hamburg, Germany
2002. Records of 99 patient with CRI admitted to the ICMA were system-
atically reviewed. A diagnosis of CRI was made when glomerular filtration        S-ADPN a secretory humoral protein of the adipose tissue has been shown
rate (GFR) was 80% of the normal value for age. The following variables         to counteract with the inflammatory response of the endothelium by inhibit-
were analyzed: gender, race, age at admission, renal function, hypertension,    ing the adhesion of monocytes to the endothelial cells and the expression
progression to end stage renal disease (ESRD), etiology of CRI (primary         of adhesion molecules in the vessel walls. Since the behaviour of S-ADPN
disease). The primary diseases were divided into 3 groups: Uropathies           in children with CRF is not well understood up to now and the increase of
(group 1), Glomerulonephritis (group 2), and others (group 3). Most pa-         S-ADPN might help to prevent early arteriosclerosis, essentially contribut-
tients were males (51%) and white (61%). Mean age at admission was 8.2          ing to the excessively increased cardiovascular mortality of young adults
y (1m-18y). The most frequent primary diseases were uropathies (51%),           on renal replacement therapy, we investigated S-ADPN in children with
glomerulonephritis (19%), tubular diseases (11%) and others (18%). There        CRF.
was a difference in age at admission, with group 2 having been admit-           S-ADPN was measured by ELISA technique (Biocat), glomerular filtration




                                                                                                                                                                 Downloaded from http://ndt.oxfordjournals.org/ by guest on May 1, 2013
ted later at a mean age of 12 y (p < 0.001). Thirty-eight (38%) patients        rate (GFR) calculated according to the Schwartz formula. A total of 40
presented hypertension at admission, without difference between groups.         children (mean age 12.8 yrs.) with CRF were investigated vs 15 healthy
Mean GFR was 36.4 ml/min (range: 7-82). There was no significant dif-            controls (mean age 9.2 yrs.). 12 children suffered from preterminal renal
ference between groups regarding renal function at admission (p = 0.20).        failure (PTRF; mean GFR 40 ml/min/1.72m2 ), 14 were on regular dialysis
A total of 36 (36%) patients progressed to ESRD and 6 (6%) died during          treatment (3 HD, 11 PD) and 14 had been tranplanted for at least 1 year with
follow-up. Group 2 had a greater risk of progression to ESRD (RR = 2.11,        stable renal function (mean GFR 70 ml/min/1.72m2 ). Statistical evaluation
95% CI = 1.3-3.4, p = 0.014). Mean time of progression to IRCT was              was performed by Mann Whitney U Test.
38 months, and progression was faster in group 2 compared to the others.        Mean S-ADPN concentrations in the group of children with PTRF was 18.9
The group of patients with glomerulopathies was at higher risk to develop       µg/ml, in the group of children on dialysis (D) 29.9 µg/ml and after renal
ESRD during follow-up, with a more rapid progression of renal failure.          transplantation (TPL) 20 µg/ml compared to 11.6 µg/ml in the group of
                                                                                healthy children (C). The differences between the S-ADPN concentrations
                                                                                of the groups were statistically significant (PTRF vs D: p=0.009; PTRF vs
 M868 ELECTRON BEAM TOMOGRAPHY IN END STAGE RENAL                               C: p=0.02; D vs TPL: p=0.02; D vs C: p=0.001; TPL vs C: p=0.006). In
          DISEASE IN CHILDREN                                                   the group of children with PTRF and after TPL (n=26) S-ADPN inversely
                                                                                correlated with GFR.
Peter Varga1 , Eunice John1 , Adisorn Lumpaopang1 , Maria Serrato2 ,
                                                                                Data suggest, that S-ADPN increases with the severity of chronic renal
Enrico Benedetti3 . 1 Pediatrics, University of Illinois at Chicago, Chicago,
                                                                                failure. Further investigations will show if elevated S-ADPN is the result
IL, United States; 2 Pediatrics, Cook County Hospital, Chicago, IL, United
                                                                                of disturbed renal function or adjuvant treatment modalities, which might
States; 3 Surgery, University of Illinois at Chicago, Chicago, IL, United
                                                                                be concluded from the relatively high concentrations in the TPL group.
States
A significant association has been noted between coronary artery calcifi-
cation (Cca) and dyslipidemia in adult patients on chronic hemodialysis.         M870 PARATHYROID HORMONE, GROWTH AND BONE DENSITY
The quantitative Ca score obtained by Electron Beam CT scan (EBCT)                        IN CHILDREN WITH CHRONIC RENAL FAILURE
correlates with the plaque burden on coronary angiography. EBCT has be-
                                                                                Simon Waller1 , Deborah Ridout2 , Thomas Cantor3 , Ping Gao3 ,
come a widely used technique to assess Cca and to stratify risk in adult
                                                                                Lesley Rees1 . 1 Nephro-Urology, Institute of Child Health and Great
patients. There is little data on the status of Cca in children with dyslipi-
                                                                                Ormond Street Hospital, London, United Kingdom; 2 Paediatric
demia and calcium/phosphate metabolic derangement in end stage renal
                                                                                Epidemiology and Biostatistics, Institute of Child Health, London, United
disease (ESRD) on dialysis or after renal transplant.
                                                                                Kingdom; 3 Scantibodies Laboratories Inc., Santee, CA, United States
We reviewed data on 15 patients in our pediatric nephrology clinic with
a mean age of 18 years (10–24years) with ESRD of various etiologies             The management of renal osteodystrophy in children with chronic renal
who were undergoing chronic dialysis or who had renal transplant. All           failure (CRF) is controversial. At Great Ormond Street Hospital the renal
had increased risk factors for coronary artery disease. The total choles-       unit policy is to aim for normal parathyroid hormone (PTH) levels. This
terol for the group was 203.2±58 mg/dl, LDL 146.3±65.5 mg/dl, HDL               study investigates the relationship between PTH, bone density and growth
54.4±14.2 mg/dl and triglycerides 130.6±86.1mg/dl. Phosphate/Ca prod-           in children with CRF.
uct was 51.4+ 20.2. Seven of fifteen patients were obese with a body mass        Between June 2001 and December 2002, 131 patients with a GFR < 60
index (BMI) of >25. IPTH was 551.4±667.8 pg/ml. Hemoglobin was                  mls/min/m2 , not treated with growth hormone, were recruited from CRF
10.8±1.8 gm/dl. All patients were also hypertensive. EBCT was performed         clinics. At each appointment patients were measured and bloods drawn for
at an average of 5 years after diagnosis and 2 years after starting dialysis.   routine biochemistry including three PTH assays. DEXA bone scans were
Six patients were scanned after renal transplant. One patient was scanned       also performed on 58 of these patients.
twice with an interval of two years.There was no significant change in the       Age (mean (range)) at entry was 7.9 (0.3-16.9) years, GFR 34 (7-60); data
Cca.                                                                            was collected for 1.0 (0.5-1.4) years, at 4.9 (2-10) clinic visits. PTH levels
Mean Cca was 129 ±480.14 (range 0–1864). Twenty percent had signif-             varied according to assay. Mean levels were within the normal range (NR),
icant scores by adult standards. These were among the oldest patients in        24.5 pg/mL (1.7-218.8) for Scantibodies CAP PTH (NR 5-39 pg/mL)
our group.There was no correlation between the level of total cholesterol,      and 36.4 pg/mL (5.0-302.0) for Scantibodies Total Intact PTH (NR 14-
HDL, LDL or triglyceride and Cca. There was a tendency towards higher           66 pg/mL) and just above the NR 6.5 pmol/L (0.3-77.6) for Immulite DPC
Cca with increasing age. In the high Cca group there was a more significant      (NR 1.1-5.4 pmol/L). Two thirds of patients were prescribed calcium based
increase in Ca x Phosphate product as well as IPTH. BMI was higher in           phosphate binders (CaCO3 ), at a dosage of 0.88 (0.1-7.2) mmol/kg, and
this group. There was a tendency for lower hemoglobin in this group.            92% were prescribed activated vitamin D, dosage 0.013 (0-0.09) µg/kg.
EBCT may provide a tool for early identification of coronary disease in          The height standard deviation score (Ht SDS) was below normal, –1.06
Monday, June 9                                                E2. Paediatric clinical nephrology, acute and chronic renal failure, ...                  279

(-4.55 to 2.46), (p<0.001), although the change in Ht SDS ( HtSDS) was          folic acid and vitamin B12 failed to show any significant difference in the
normal, 0.03 (-1.16 to 2.27), (p=0.47). There was no relationship between       three studied groups. No significant difference in the basal Hcy level be-
the Ht SDS and age, GFR, PTH level, dosage of calcium carbonate or              tween patients with previous history of vaso-occlusive disease and those
activated vitamin D; however it was positively correlated to serum alkaline     without. Half of the patients on regular HD (group Ia) (n=15) were given
phosphatase (r=0.41, p<0.001). The mean DEXA age-matched Z-score                folic acid as 50 mg of 5-formyl-tetrahdrofolate (the active form of folic
was normal, 0.02 (-2.3 to 2.8) (p=0.89). This correlated with Ht SDS (r=        acid) intravenous once weekly after the dialysis session for 4 weeks. The
-.035, p<0.01) and CaCO3 prescription (r=0.34, p<0.01). However, there          other half (group Ib) (n=15) received in addition to folic acid therapy, vi-
was no relationship between the DEXA Z-score and vitamin D prescription         tamin B12 1000 microgm hydroxycobalamine once intramuscularly. After
or PTH levels.                                                                  therapy the mean Hcy decreased significantly in those who received folic
Although these children are 1 SD shorter than their peers, their growth ve-     acid and vitamin B12 (7.80±3.77 micromol/L) (p<0.001) to a level com-
locity remains normal despite considerable renal impairment. Maintenance        parable to the basal levels in conservative and control groups. A non signifi-
of normal PTH levels in children in CRF allows normal growth velocity           cant decrease was found in those who received folic acid only (13.3±11.47
and bone density. Prescription of CaCO3 affects bone density.                   micromol/L) (p>0.05).
                                                                                In conclusion, Hcy is high in children with ESRD on regular HD and
                                                                                combined therapy of active form of folic acid and vitamin B12 is of value
 M871 ANTIOXIDANT VITAMINS IN PEDIATRIC PATIENTS WITH                           in decreasing Hcy to values comparable to that in controls.
          CHRONIC RENAL FAILURE
Mohamed Salah Faheem1 , Ihab Zaki El-Hakim1 , Sanaa Eissa2 ,
                                                                                 M873 LEFT VENTRICULAR HYPERTROPHY (LVH) IN CHILDREN
Mayssa Khalil1 . 1 Pediatrics, Ain Shams University, Cairo, Egypt;
2                                                                                         WITH MILD-TO-MODERATE CHRONIC RENAL FAILURE
  Biochemistry, Ain Shams University, Cairo, Egypt
                                                                                          (CRF)
Objectives: We attempted to assess the pattern of anioxidant vitamins




                                                                                                                                                                Downloaded from http://ndt.oxfordjournals.org/ by guest on May 1, 2013
                                                                                Stefano Picca1 , Maria Chiara Matteucci1 , Gabriele Rinelli2 ,
in pediatric patients with chronic renal failure (CRF) and the effect of
                                                                                Franz Schaefer3 , Elke Wuhl3 , Gianfranco Rizzoni1 , Otto Mehls3 . Division
hemodialysis (HD) on them.
                                                                                of Nephrology and Dialysis, Bambino Gesù Children’s Hospital and
Methods: The present study included 15 patients with CRF on mainte-
                                                                                Research Institute, Rome, Italy; Division of Cardiology, Bambino Gesù
nance HD (group A), 15 patients with CRF on conservative treatment
                                                                                Children’s Hospital and Research Institute, Rome, Italy; Division of
(group B) and 15 healthy age and sex matched subjects for control (group
                                                                                Pediatric Nephrology, University’s Children’s Hospital, Heidelberg,
C). All subjects discontinued vitamins before sampling by enough time.
                                                                                Germany; for the European Study Group on Chronic Renal Failure in
Retinol and alpha-tocopherol by HPLC and L-ascorbic by colorimetric
                                                                                Children
method.
Results: Plasma vitamin C levels were significantly low in group A in            LVH is a common feature in adults with predialysis renal insufficiency. In
comparison with group B and C. Plasma vitamin A levels were significantly        children, LVH is described both before ESRD (Johnstone, 1996) and at the
low in group B in comparison with group C. Plasma vitamin E levels were         beginning of chronic dialysis (Mitsnefes, 2000). No definite data on preva-
significantly low in group A in comparison with group B and C as well as in      lence of LVH in children with CRF on conservative treatment are available.
group B in comparison with group C. The dialysis session had significant         As a part of a prospective study on the renoprotective effect of blood pres-
lowering effect only on vitamin E.                                              sure (BP) control on the progression of CRF in children, we evaluated
Conclusion: Patients with CRF have some deficiency of antioxidant vita-          left ventricular mass (LVM) in 109 patients (61 males, median age 11 yrs,
mins that might be exacerbated by HD. Thus supplementation with such            median GFR 42 ml/min/1.73m2 ) from ten European Centers, using two-
vitamins may be helpful. On the other hand, vitamin A should not be sup-        dimensional M-mode echocardiography according to the American Society
plemented to CRF patietns to prevent hypervitaminosis A.                        of Echocardiography criteria. LVM was calculated with the Devereux for-
                                                                                mula and indexed to height2.7 (LVMi). LVMi was compared with a normal
                                                                                pediatric population and expressed as SD (deSimone 1998). Simultaneous
 M872 STUDY OF THE HOMOCYSTEINE STATUS IN CHILDREN                              24-hrs-ABPM was performed. Echocardiograms were recorded on VHS
          WITH CHRONIC RENAL FAILURE                                            cassettes and collected for central reading. The possible influence of ACE
                                                                                polymorphism was also evaluated in 80/109 children.
Farida A. Farid1 , Mohamed S. Faheem1 , Nahla M. Heshmat1 , Karim
                                                                                30/109 (27.5%) patients showed LVH. No correlation was found between
Y. Shaheen2 , Sherine S. Saad1 . 1 Pediatrics, Ain Shams University, Cairo,
                                                                                LVMi and BP or Hb levels (univariate+multivariate analysis). Children
Egypt; 2 Clinical Pathology, Ain Shams University, Cairo, Egypt
                                                                                with GFR<30 ml/min/1.73m2 and with ID+DD genotype, as compared
The role of hyperhomocysteinemia in the development of accelerated              with those with II genotype, showed higher, although not significant, LVH
atherosclerosis and/or thrombosis in end stage renal disease (ESRD) pa-         prevalence (32 vs 30% and 27 vs 19%, respectively). Conclusions: as in
tients has been suggested possibly due to homocysteine (Hcy) induced            adult patients, LVH largely predates RRT phase. Renal impairment seems
endothelial cell injury. This in turn stimulates the local coagulation system   to predispose to LVH, independently of factors typical of adult age and
and consequently the anticoagulation activity especially protein S, protein     of the influence of ACE genotype. The entity of the problem seems more
C and activated protein C- resistance (APC-R). Homocysteine is mainly           severe than previously reported.
catbolized by remethylation reaction that needs folic acid as methyl donor
and vitamin B12 as a cofactor.
In our study, we aimed at evaluating the Hcy status in children with chronic     M874 PAEDIATRIC RENAL REPLACEMENT THERAPY IN
renal failure (CRF) especially in those suffering from ESRD and the pos-                  GERMANY: FACTS AND FIGURES OF THE
sible role of folic acid and vitamin B12 therapy in the correction of hyper-              QUASI-NIERE-REGISTRY
homocysteinemia if present.
                                                                                Hans-Juergen Schober-Halstenberg, Kirsten Haas, Guenther Edenharter,
This work was carried out in the Pediatric Nephrology and Dialysis Unit
                                                                                Gisela Offner, Ulrich Frei. Quality Assurance, QuaSi-Niere gGmbH,
of Ain Shams University from January 2000 to January 2001. It included
                                                                                Berlin, Germany
40 patients with CRF, 30 on regular hemodialysis (HD) treatment (group I)
and 10 on conservative (medical) treatment (group II) in comparison to 10       Paediatric RRT in Germany started in the mid Sixties in a few centres.
healthy age and sex matched controls (group III). The basal serum levels        From 1967-1991 less than 2000 children on RRT had been reported from
of Hcy, folic acid and vitamin B12 as well as plasma level of APC-R were        20 German paediatric centres to the EDTA Registry suggesting a high rate
measured in patients and controls. The mean serum Hcy was significantly          of underreporting. Since the beginning of the German Renal Data Registry
higher in those on regular HD (17.9±10.07 micromol/L) in comparison to          QuaSi-Niere in 1994 all (100%) paediatric patients have been registered
those on conservative treatment (8.05±2.99 micromol/L) (p<0.001) and            and monitored with follow-up data.
controls (7.03±2.27 micromol/L) (p<0.001), while there was no signifi-           The German Renal Data Registry QuaSi-Niere observes all 926 prevalent
cant variation between the later two groups. The mean values of APC-R,          and 128 incident paediatric patients in RRT of the year 2002. It has ex-
280         E4. Paediatric transplantation                                                                                              Monday, June 9

tended the actual database with harmonized and adjusted historical data         treated with Rituximab, but in whom prolonged B-lymphopenia developed
from the EDTA data base. QuaSi-Niere actually explores more than 5000           as a consequence.
paediatric data sets. This allows to observe more than 30 years of RRT. Age     A 5-year old boy was successfully transplanted with a cadaveric donor
specific analysis shows the evolution of dialysis and transplantation in dif-    kidney. Initial immunosuppression consisted of steroids, cyclosporine A
ferent age groups in comparison to other international registries (USRDS,       (CyA) and mycophenolate mofetil (MMF). Six months after engraft-
ANZDATA). The long term observation shows no relevant shift of primary          ment, transplant function deteriorated. Ultrasound examination of the graft
diagnoses; further investigation from national registries have to prove these   demonstrated a large tumor which caused obstruction of the pyelum.
epidemiological assumptions.                                                    Histopathologic examination of a biopsy specimen from the tumor was
                                                                                consistent with a B-cell lymphoma, which was positive for EBV infection
                                                                                markers. MMF was discontinued, CyA dosage was diminished, aiming
                                                                                for trough levels around 50 µg/L and prednisone was continued. Ritux-
                                                                                imab (375 mg/m2 ) was given 4 times at a 2 week interval. Tumor size was
                                                                                followed by ultrasound. Quantitative polymerase chain reaction on whole
                                                                                blood was used to follow EBV load. Serum immunoglobulin concentra-
                                                                                tions were determined every 3 weeks and lymphocyte subpopulations were
                                                                                determined by flow-cytometry.
                                                                                Reduction of immunosuppression and treatment with Rituximab resulted in
                                                                                rapid resolution of EBV infection. Tumor size started to decrease 4 weeks
                                                                                after initiation of treatment. After 3 months, histopathologic examination
                                                                                of a repeat biopsy showed no viable lymphoma cells. Serum immunoglobu-
                                                                                lin concentrations were decreased within two weeks after start of treatment
                                                                                (IgA 0.4 g/L, IgG 2.1 g/L, IgM 0.2 g/L) and intravenous immunoglobulin




                                                                                                                                                               Downloaded from http://ndt.oxfordjournals.org/ by guest on May 1, 2013
                                                                                substitution was started. Flow cytometry demonstrated less than 1% CD20
                                                                                positive cells 1 month after start of treatment. After 12 months, the per-
Figure 1.                                                                       centage of CD20 and CD19 positive cells (B-lymphocytes) is still less than
                                                                                1%. Immunoglobulin substitution is continued. No opportunistic infections
                                                                                have occurred over the last 12 months. Graft function is still satisfactory.
                                                                                Treatment of PTLD with reduction of immunosuppression and Rituximab
                                                                                results in rapid remission of the tumor. Prolonged B-lymphopenia can de-
                                                                                velop as a consequence of this treatment regimen. We suggest close mon-
                                                                                itoring of B-lymphocyte population and immunoglobulin concentrations
                                                                                in patients treated with Rituximab. Immunoglobulin substitution should be
                                                                                considered in cases of acquired hypogammaglobulinemia. It will increase
                                                                                the costs of the treatment substantially.


                                                                                 M876 HIGH PRETRANSPLANT SERUM sCD30 CONTENT AS A
                                                                                          POSSIBLE EXPLANATION FOR THE HIGH REJECTION
                                                                                          RATE IN YOUNG KIDNEY GRAFT RECIPIENTS

Figure 2.                                                                       Caner Suesal, Steffen Pelzl, Gerhard Opelz, for the Collaborative
                                                                                Transplant Study. Department of Transplantation Immunology, Institute of
                                                                                Immunology, University of Heidelberg, Heidelberg, Germany
Follow-up data is available from 926 patients equivalent to 60 pmcp (per
million child population). In the 15 EU countries 5.183 patients on RRT         Young kidney graft recipients demonstrate a higher rate of graft rejection
could be expected if German prevalence rates in paediatric nephrology           than adults. We showed recently that the pretransplant (pre-Tx) serum
would be used as reference. The cooperation between QuaSi-Niere and             sCD30 content is an excellent predictor of kidney graft outcome and that
the European renal registries will give further information and will try        sCD30 measured in the early post-Tx period is predictive of acute rejection.
to explain the differences within Europe. -Survival analysis is currently       In the present study we investigated whether high levels of sCD30 could
performed and will be provided by the time of presentation-.                    be responsible for the high rejection rate in <20 year old recipients.
This abstract is published on the behalf of all 20 centres for paediatric       Pre-Tx sera of 5653 kidney recipients from 38 different centers were tested
nephrology in Germany                                                           for sCD30 content in a specific ELISA.
                                                                                1550 recipients with a high pre-Tx serum sCD30 content of ≥100 U/ml had
                                                                                a 3-year graft survival rate of 71±1%, compared with a 82±1% rate in 4103
                                                                                recipients with a low sCD30 of <100 U/ml (p<0.0001). sCD30 levels were
E4 Paediatric transplantation                                                   significantly higher in presensitized patients and regraft recipients than in
                                                                                non-sensitized patients and first graft recipients, respectively (p<0.0001),
                                                                                which underlines the association of high sCD30 with increased alloreac-
 M875 PROLONGED B-LYMPHOPENIA AFTER RITUXIMAB                                   tivity. 309 <20 year old kidney graft recipients had a strikingly higher
             TREATMENT                                                          sCD30 level than 5344 ≥20 year old recipients (203±7 versus 80±1 U/ml,
                                                                                p<0.0001). An even higher sCD30 was found in the subgroup of 80 <10
Marc Lilien1 , Tom Revesz2 , Ger Rijkers3 . 1 Pediatric Nephrology,
                                                                                year old recipients (267±13 U/ml). Among <20 year old recipients, the
University Medical Center, Utrecht, Netherlands; 2 Pediatric Hematology
                                                                                incidence of patients with a high sCD30 was 79%, strikingly higher than
and Oncology, University Medical Center, Utrecht, Netherlands;
3                                                                               the 24% incidence rate among ≥20 year old recipients (p<0.0001). The
  Laboratory for Pediatric Immunology, University Medical Center,
                                                                                sCD30 effect was evident in adult as well as young recipients (11% and
Utrecht, Netherlands
                                                                                10% difference in 3-year graft survival between high and low sCD30, re-
Posttransplant lymphoproliferative disease (PTLD) is a serious complica-        spectively).
tion after solid organ transplantation, associated with primary Epstein-Barr    Our data indicate that, together with the previously shown absence of pro-
virus (EBV) infection. Treatment consists of reduction of immunosup-            tective IgA-anti-Fab autoantibodies, the higher incidence of a high pre-
pression, which carries the risk of inducing acute rejection. Rituximab, a      transplant sCD30 content is a possible explanation for the high graft rejec-
humanized anti-CD20 monoclonal antibody, has been proposed as an al-            tion rate in young kidney graft recipients.
ternative treatment. We report a patient in whom PTLD was successfully

				
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