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									 Metropolitan New York / New
Jersey Pediatric Board Review
            Course

    Pediatric Nephrology
         May, 2008
          Leonard G. Feld MD PhD
         Levine Children’s Hospital
               Charlotte, NC
           Howard Trachtman MD
        Schneider Children’s Hospital
            New Hyde Park, NY
                Materials
• Consider all other material to help you
  achieve a passing score
  – American Academy of Pediatrics – Pediatrics
    Review and Education Program (PREP),
    Pediatrics in Review.
               Renal / Urology
• General
  – Normal function
  – Proteinuria
  – Hematuria
     • Persistent microscopic hematuria
     • Causes of gross and microscopic hematuria
  – Dysuria / Incontinence
• Congenital
  – Renal dysplasia
  – Abnormalities of the collecting system, kidney,
    bladder
             Renal / Urology
• Acquired
  – Infection of the urinary tract
  – Acute glomerulonephritis
  – Nephrotic syndrome
  – Hemolytic uremic syndrome
  – Henoch-Schoenlein purpura
  – IgA nephropathy
• Other
  – Renal Failure,Trauma, renal stones, RTA
           Renal / Urology
• Hypertension
• Nephrogenic diabetes insipidus
• Cystinosis
         Outline – Part 1

• Hematuria
• Proteinuria
• Hypertension
• Urinary tract infections
• Glomerulonephritis
A 3-week-old male infant presents with a history of irritability,
low grade fever, emesis and diarrhea. Prenatal and family
history is non-contributory. On examination the infant is
irritable, temp of of 39.0°C, has mottled skin and a capillary
refill of 4 sec. The systolic blood pressure is normal and the
pulse is 185 beats/min. The anterior fontanelle is full.

Hemoglobin                14 g/dl
White cell count          30,000
Platelets                 110,000

What studies would you like to perform.
What is your initial therapy?
What is your initial diagnosis (es)?
                Answers
• Blood culture, urine, CXR, and LP
• Fluid resuscitation + broad spectrum
  antibiotics
• Late onset neonatal sepsis / meningitis
                                                   Suggested
                          Evaluation and Management of the Febrile Infant <60 Days of Age

                                                       GLOBAL ASSESSMENT




                                                       Appears Moderately Ill
                        Appears Toxic                                                      Appears Generally Well
                                                       or Your Are Not Sure



                      Treat Expectantly                 Evaluate for Possible
                                                                                          Skin, Soft Tissue, Bone or
                         Hospitalize                   Sepsis, Hospitalize and
                                                                                                Joint Infection?
                        See Table 1                             Treat

                                                                            NO                       YES

                                                                                            Evaluate as Indicated
                                                        WBC with Differential
                                                                                            Hospitalize and Treat
                                                            Urinalysis
                                                                                                See Table 1



                                  WBC = 5,000 - 15,000 /mm3, AND Band Count < 1,500 /mm3 AND
                             Urinalysis < 10 WBC/HPF in Unspun Sediment and Negative Gram Stain (OR
                                                 < 10 WBC/HPF in Spun Sediment)

                                                 YES                             NO

                                                                             LP = CSF Analysis; Cultures of Blood, CSF,
               History of Prematurity, Perinatal Problems,
                                                                            Urine (Suprapubic or catheter specimen only)
              Underlying Condition, Previous Antimicrobial      YES
                                                                            Hospitalize and Give Parenteral Antimicrobial
                                agents
                                                                                              Therapy

                        NO

              Culture Blood and Urine
              (Suprapubic or catheter
                                                      Physician Identified to Assume Full                           Hospitalize
                  specimen only)                                                                     NO
                                                   Responsibility for Outpatient Management
                  Observe without
               Antimicrobial Therapy
                                                                      YES
                                                                                                               NO
                                                   Caregiver with Good Observation Skills,
                      Option 1                    Telephone in Home, Can Meet Responsible              Option 2          Empiric
        No Antimicrobial                                Physicians within 30 Minutes
                                                                                                                       Antimicrobial
            Therapy
                                                                                                                         Therapy
           Manage as Outpatient, Instruct Parents to
                                                                                           LP - CSF Analysis; Culture Blood, CSF,
                Watch For SEE BELOW ….*                                                    Urine (Suprapubic or catheter specimen
         Phone Follow-up Within 12 Hours, Re-Examine                                                       only)
                       Within 24 Hours



                         * 1.) skin changes like a new rash or a change in a rash that is already present
                           2.)   discoloration like duskiness, cyanosis, mottling
                           3.)   the extremities feel cool
                           4.)   the infant feeds poorly, or vomits
                           5.)   the infant is difficult to comfort
                           6.)   the infant is difficult to arouse or is less interactive with the parent than usual
                           7.)   any evidence of a seizure, like eye rolling or quick jerky movements
                           8.)   bulging of the soft spot (anterior fontanelle)




From Consensus in Pediatrics Fever In Infants and Children, Feld LG, Hyams J eds. Mead Johnson Nutritionals, 2007.
PEARL – No question on the
 < 28 day old febrile infant
                 Hematuria
Case: Susan is an 8 year old noted on routine
 exam to have moderate hematuria on dipstick.
 She has an unremarkable past medical history.
 Family history is negative in the parents and
 siblings for any renal disease. History of
 hematuria is unknown. A repeat urine in one
 week is still positive and a urine culture showed
 no growth.
 What is the next step? What would be a major
 consideration for a referral to a pediatric
 nephrologist?
• Repeat a first AM void following restricted
  activity , perform a microscopic on a fresh urine
• Check the family members
• If there is still blood without protein, casts,
  crystals, normal BP with or without a strong
  family history, no further work-up is generally
  required.
• Caveat - Family anxiety because of the
  connotation of blood and cancer in adults.
       Classification of Hematuria

• Microscopic (vast majority of the cases)
  –   Transient
  –   Persistent
• Macroscopic (urologic / renal disorders)
  –   Transient
  –   Persistent (> 2 weeks)
• Persistent microscopic/ Transient
  macroscopic
  –   IgA or Berger’s; benign recurrent hematuria
Glomerular v. Non-glomerular bleeding
• Glomerular

  – oliguria, edema, hypertension, proteinuria, anemia

• Non-glomerular

  –   dysuria, frequency, polyuria, pain or colic, hx
      exercise

  –   crystals on microscopic

  –   mass on exam

  –   medication history - sulfas, aspirin, diuretics
   Who should be worked up?
• Presence of proteinuria and/or
  hypertension
• History consistent with infectious history,
  HSP, systemic symptoms, medication use
  or abuse, strong family history of stones or
  renal disease/failure.
• Persistent gross hematuria
• Family anxiety - limit evaluation
Initial evaluation of the patient with hematuria

• All patients: BUN, creatinine, CBC, kidney
  and bladder ultrasound
• Probable glomerular hematuria
  – C3, ASO titer
  – possible: hepatitis, HIV, SLE serology
  – renal biopsy
• Probable non-glomeurlar hematuria
  –   urine culture, urine Ca/creatinine ratio
  –   possible: hemoglobin electrophoresis,
  –   coagulation studies, isotope scans,
  –   Flat plate, CT, ??IVP, cystoscopy
         Pearls for Hematuria
• Hematuria is an important sign of renal or
  bladder disease
• Proteinuria (as we will discuss) is the more
  important diagnostic and prognostic finding.
• Hematuria almost never is a cause of anemia
• The vast majority of children with isolated
  microscopic hematuria do not have a treatable
  or serious cause for the hematuria, and do not
  require an extensive evaluation. So a VCUG,
  cysto and biopsy are not indicated.
                More Pearls
• Urethrorrhagia – boys with bloody spots in the
  underwear
  – Presentation – prepuberal ~ 10 yrs
  – It is painless
  – Almost 50% will resolve in 6 months and > 90% at 1
    year; it may persist for 2 yrs
  – Treatment – watchful waiting in most cases
• Painful gross hematuria – usually infection,
  calculi, or urological problems; glomerular
  causes of hematuria are painless.
 More Pearls – gross hematuria
• Gross hematuria is often a presentation of
  Wilms’ tumor
• All patients with gross hematuria require an
  imaging study.
• If a cause of gross hematuria is not evident by
  history, PE or preliminary studies, the differential
  is hypercalciuria, SS trait, or thin basement
  membrane disease.
• Cysto is rarely helpful
7 year old boy developed gross tea colored hematuria
after a sore throat and upper respiratory infection. No
urinary symptoms but urine output was decreased. He
complained of mild diffuse lower abdominal pain. There is
no fever, rash or joint complaints. Past med history was
unremarkable but had intermittent headaches for two
years.

On exam he was well with a BP of 95/65, no edema,
some suprapubic tenderness and red tympanic
membranes.
The mother thinks that a similar episode occur on
vacation a few months ago.


WHAT WOULD YOU LIKE TO DO?
                     Tests
•   Normal electrolytes
•   Creatinine 0.5 mg/dl
•   Urinalysis – large blood, no protein
•   Urine culture – no growth
          More to the story
• She calls with a recurrent episode of gross
  hematuria with a URI three months later
• So what do you do ?
             Other tests
• ANA, ANCA, ASO, Family screening
• Complement – C3NF
                Now what
• IGA nephropathy
  – Boys > girls
  – Mostly normotensive, with persistent
    microscopic hematuria
  – Chronic glomerulonephrits – up to 40% of
    primary glomerulonephritis
  – Complement studies are nl, some inc IgA
  – Prognosis – not so good if > 10 yrs of age,
    proteinuria, reduced GFR, hypertension and
    no macrohematuria
                                            Acute
                                       Glomerulonephritis

                                                                                              Normal
        Low Complement
                                                                                            Complement




     Systemic diseases                                                                Systemic diseases
               SLE                                                                       Polyarteritis nodsa
   Subacute Bact Endocarditis                                                         Hypersensitivity vasculitis
          Shunt nephritis                                                                    Wegener’s
 Essential mixed cryoglobulinemia                                                               HSP
         Visceral abscess                                                                 Goodpasture’s




       Renal diseases                                                                   Renal diseases
                                                                                            IGA
     Acute proliferative GN
                                                                                           RPGN -
    Membranoproliferative GN
                                                                                Anti-GBM, immune complex GN




                                       Serologic evidence of antecedent strep infection
                                               (ASO, anti-Dnase B, streptozyme


                                       Positive                            Negative




                                                                                             Lupus
                                                                                Essential mixed cryoglobulinemia
                       PSAGN                                                             Shunt nephritis
                  Strep endocarditis                                                    Visceral abscess
                                                                                             MPGN
                                                                                       Non-strep infection




                  Clinical evidence
                      to support
                     endocarditis




               YES
                                                  NO



 Blood cultures
Echocardiogram                                          Treat PSAGN
                                    Glomerular   Non-glomerular


Urinalysis   Dysmorphic RBC                 +            -
             Cellular casts                 +            -
             Brown/tea color               ++            +
             Bright red                     +           ++
             Clots                          -            +
             Crystals                       -            +
             Protein                        +            -
History      Family Hx of ESRD             +             -
             Systemic disease              +             -
             Nephrolithiasis               -             +
             Trauma                        -             +
             Symptomatic vomiting          -             +
Physical     Hypertension                  ++            +
             Systemic signs                +             -
             Edema                         +             -
             Abdominal mass                 -            +
             Genital bruising               -            +
A four-year boy presents with a 5-day history of
  swollen eyes and ―larger ankles‖. On exam he
  has periorbital and pretibial edema. The most
  appropriate tests include all the following except.
• a. Urinalysis
• b. Blood tests for total protein and albumin
• c. Serum creatinine
• d. Sedimentation rate
• e. Serum complement (C3)
On routine physical examination, an 8-year-
  old boy is found to have microscopic
  hematuria. The first step in your evaluation
  should be.
• Examine the urine sediment
• Order an intravenous pyelogram
• Obtain a voiding cystourethrogram
• Perform a CBC in the office
• Order an ASO titer
An 8-year-old boy presents with tea colored
  urine. He has very mild edema. The work-
  up should include all the following except.
• Complement studies
• Serum creatinine
• Urinalysis for protein
• Monitor blood pressure and urine output
• Obtain an intravenous pyelogram and
  VCUG
              Proteinuria
John is an 12 year old noted on a basketball
  team physical to have 2+ protein on
  dipstick. He has an unremarkable past
  medical history. Family history is negative
  in the parents and siblings for any renal
  disease. A repeat urine in one week in his
  PMD’s office is still positive.

What is the next step? Should you refer?
• Repeat a first AM void following restricted
  activity, perform a microscopic on a fresh urine;
  also an alkaline pH may give a false positive
  result
• If there is still protein perform a more formal
  orthostatic test. If orthostatic, no further work-
  up is generally required, although no
  indemnification from subsequent renal disease.
• Caveat - Family anxiety because of the
  connotation of protein and friends told them
  about kidney failure.
              Definitions (Pearl)
• Urine protein to creatinine ratio
   –   Normal:             < 0.2 (< 0.15 adolescents)
   –   Mild to moderate:   0.2 to 1.0
   –   Heavy or severe:    > 1.0


• Persistent proteinuria: present both in the
  recumbent and the upright posture; even in this
  situation, proteinuira is less during recumbency
 What does Orthostatic Proteinuria
            mean?


 Protein
Excretion
            Threshold of Detection




            Normal             Orthostatic   Recumbant
                                             Erect
          Causes of Proteinuria
• Transient
  –   fever, emotional stress, exercise, extreme cold,
      abdominal surgery, CHF, infusion of epinephrine
• Orthostatic
  –   Transient or fixed / reproducible
• Persistent
  –   Glomerular disease: MCNS, FSGS, MPGN, MN
  –   Systemic:     SLE, HSP, SBE, Shunt infections
  –   Interstitial: reflux nephropathy, AIN, hypoplasia,
      hydronephrosis, PKD
Hypertension
               Hypertension
Case: David is a 10 year old boy first noted to
 have an elevated blood pressure of 140/85
 during a PE for headaches. Pt has a long history
 of learning and behavioral issues. Headache
 evaluation was normal (CT, sinus,etc.). Referred
 for evaluation. Initial evaluation noted a Ht / Wt >
 99%tile, BP of 128/86 mmHg, normal ultrasound
 and renal scan, although a plasma renin of 8
 ng/ml/min (nl < 2).
Do you perform an angiogram?
        Definition of Hypertension
   The 4th Report on High Blood Pressure in Children and
                       Adolescents
• Hypertension—average SBP and/or DBP that
  is greater than or equal to the 95th percentile for
  sex, age, and height on 3 or more occasions.
• Prehypertension—average SBP or DBP levels
  that are greater than or equal to the 90th
  percentile, but less than the 95th percentile.
   – Adolescents with BP levels greater than or equal to 120/80
     mmHg should be considered prehypertensive.
                     Evaluation of Hypertension
        Historical                                                         Physical
       Information                                                        Examination




                                                                        Vital signs
     Neonatal history
                                                                 (including extremities)
      Family history
                                                                      Height/Weight
      Dietary history
                                                                Specific attention to organ
 Risk Factors (smoking,
                                                                 systems - cardiac, eye,
  alcohol use, drug use)
                                                                abdominal or other bruits,
  Non-specific / specific
                                                                            etc.
     symtomatology
Review of Systems - sleep
                                                                Consider ambulatory blood
and exercise patterns, etc.
                                                                    pressure monitor




                                      Evaluation Phase 1

                       CBC, urinalysis, urine culture, electrolytes, BUN,
                            creatinine, plasma renin, lipid profile,
                        echocardiogram, renal ultrasound with duplex
                                            doppler




                                     Evaluation Phase 2
                         Selected studies based on magnitude of the
                        hypertension and/ or other clinical /laboratory
                                           findings

                                  Renal flow scan (MAG 3)
                                   CT Angiography (CTA)
                        MRA (may not provide adequate evaluation for
                             peripheral renal vascular lesions)
                        Renal arteriography with renal vein sampling
                        Plasma / urine catecholamines and/or steroid
                                       concentrations
  Therapeutic Lifestyle Changes

• Normal     Encourage healthy diet, sleep, and physical
  activity.
• Prehypertension Recommend weight management
  counseling if overweight; introduce physical activity and
  diet management.
• Stage 1 hypertension Recommend weight
  management counseling if overweight; introduce
  physical activity and diet management.
• Stage 2 hypertension Recommend weight
  management counseling if overweight; introduce
  physical activity and diet management.
       Indications for Treatment
•   Symptomatic hypertension
•   Secondary hypertension
•   Hypertensive target-organ damage
•   Diabetes (types 1 and 2)
•   Persistent hypertension despite
    nonpharmacologic measures
         Pharmacologic Therapy
       for Childhood Hypertension
• The goal for antihypertensive treatment in
  children should be reduction of BP to
  <95th percentile, unless concurrent
  conditions are present. In that case, BP
  should be lowered to <90th percentile.
• Severe, symptomatic hypertension should
  be treated with intravenous
  antihypertensive drugs.
Urinary Tract
 Infections
                  Case History
  • A 12 mo old girl is diagnosed with the
    first febrile UTI. She is not eating well.
    UA shows pyuria and bacteria. Urine
    culture is obtained. Antibiotics are given
    (SMX-TMP).
  • How to proceed?
        – What are some of your concerns?
        – Radiographic follow-up
        – Long-term monitoring
Feld - 10/98                                     43
 Bacteriology /Pathogenesis UTI - 1

• Most Common - E. Coli, coliforms
• Virulence Factors
           • adherence to uroepithelium by P-fimbriae
           • endotoxin release
           • Pyelo vs cystitis - 80 to 20%




Feld - 10/98                                            44
 Bacteriology /Pathogenesis UTI 2

  • Perineal / urethral factors
     – uncircumcised - 10-20x risk
     – ? Urethral caliber (infant girls)
     – other myths such as bubble bath, wiping
       techniques
  • Low Urinary factors
           – dysfunctional voiding ; constipation
     • Other - indwelling catheters, congenital
          anomalies, Vesicoureteral reflux, sexual
          activity
Feld - 10/98                                         45
                Diagnosis
• Leukocyte test and nitrate test

• Urine culture > 40-50,000 CFU/mL

• Pyuria - not on recurrent UTIs




Feld - 10/98                         46
               Clinical Issues
 • Lower tract - frequency, urgency,
   enuresis, dysuria
 • Upper tract - fever - nearly all in boys
   under 1 year of age; females peak in
   first year but still significant through the
   first decade
 • Asymptomatic bacteriuria - low risk
Feld - 10/98                                      47
               Radiological Evaluation
• Renal ultrasound - anatomy, size, location,
  echogenicity
• DMSA (2nd choice glucoheptanate -
  SGH) - cortical integrity, photopenic regions,
    differential function, abscess
• CT scan - abscess
• VCUG - standard for first UTI; radionuclide
    for follow-up or siblings
• IVP - NO WAY
Feld - 10/98                                    48
               Grades of Reflux




Feld - 10/98                      49
               Reflux Recommendations
                    ―the simple way‖
• GRADES             I - III   Antibiotics



• GRADES             IV - V    Surgery




Feld - 10/98                                 50
                        Treatment
• Oral
     – SMX-TMP, Amoxicillin/Clavulanate
     – Cefuroxime, cefprozil, cefixime, cefprodoxime
• Parenteral
     – Neoates: Ampicillin / Gentamicin
     – Older Children:
           • Advanced level cephalosporin
           • Beta lactam + beta lactamase inhibitor
           • Aminoglycoside (+ ampicillin)
Feld - 10/98                                          51
                  Case History
  • A 12 mo old girl is diagnosed with the
    first febrile UTI. She is not eating well.
    UA shows pyuria and bacteria.. Urine
    culture is obtained. Antibiotics are given
    (SMX-TMP).
  • How to proceed?
        – What are some of your concerns?
        – Radiographic follow-up
        – Long-term monitoring
Feld - 10/98                                     52
               The Suggested Answers
 • What are your concerns?
       – Voiding history
 • Radiographic studies
       – ultrasound and VCUG
 • Follow-up (no reflux)
       – cultures every month for three months,
         then every other month for six months
         ( every 4 months)
 • Follow-up (reflux) - antibiotics
Feld - 10/98                                      53
Glomerulonephritis / Acute
      renal failure
                  Case History
  • A 3 year old boy was attending summer
    camp. Five days later he presents with
    diarrhea, abdominal pain and appear
    pale. His mother finds out that there
    was cook out at camp. On examination
    the child is pale and is unable to void -
    How to proceed?
        – What are some of your concerns?

Feld - 10/98                                    55
          Clinical prodrome
• Diarrhea prodrome 1-15 days
• Abdominal pain – may be confused with
  ulcerative colitis, appendicitis, rectal
  prolapse, intussusception
• Pallor
• Irritability, restlessnes
• Edema – after rehydration
• Oliguria/anuria
    HUS: Clinical manifestations
•   Thrombocytopenia
•   Hemolytic anemia
•   Renal failure
•   Neurologic (irritability, seizure, CVA)
•   Pancreatitis (IDDM) and colitis
•   Hypertension
        HUS: Pathogenesis
• Endothelial cell damage occurs secondary
  to toxin injury via binding to glycolipid
  receptor or lipopolysaccharide absorption.
   HUS: Differential diagnosis
• Other forms of acute Glomerulonephritis /
  renal failure
• Vasculitis
• Urosepsis
• Renal vein thrombosis
• Coagulopathy (DIC)
    Conservative management
• Fluid restriction to <insensible losses plus
  urine output
• Foley catheter – limit to 24-48 hrs
• Blood transfusion / platelets
• Routine use of antibiotics controversial
• Diuretics
• Nutrition
        Surgical Complications
•   Toxic megacolon
•   Rectal prolapse
•   Colonic gangrene
•   Intussusceptions
•   Perforation
•   Strictures
•   Mimic appendicitis, IBD
                   Case
• A four year old boy presents with a three day
  history of periorbital swelling and sox
  indentations around his ankles. He has been
  healthy without any intercurrent illnesses. The
  family and past medical history are
  unremarkable. On examination he has pretibial
  edema and has gained 2.5 kg since his
  examination 2 months ago for an otitis media.
• What are your thinking?
Nephrotic Syndrome
              Definition
• Nephrotic syndrome is a clinical state
  characterized by heavy proteinuria and
  hypoalbuminemia, often associated with
  edema, hypercholesterolemia, and
  generalized hyperlipidemia.
PRIMARY NEPHROTIC
    SYNDROME


• 90% childhood cases
• unassociated with systemic
  disease
   CLINICAL PRESENTATION (1)

• EDEMA is the major symptom - first
  periorbital, then generalized. Happy
  parents: ―finally my child is gaining
  weight‖.
• Soft and pitting in nature.
• May cause anasarca with ascites, pleural
  effusions, labial and scrotal swelling.
    CLINICAL PRESENTATION (2)

• Poor appetite
• Diarrhea during massive edema
• Hepatomegaly
• Abdominal pain (need to r/o peritonitis or
  surgical abdomen)
• Respiratory difficulty
• Hypertension (15%-20% of MCNS)
     LABORATORY FEATURES
•   PROTEINURIA is the primary abnormality.
•   ―Selective‖- almost entirely albuminuria
•   > 40 mg/m2/hr or
•   U protein/creatinine ratio > 1 (mg/mg)
•   due to loss of charge selectivity of
    glomerular basement membrane
 COMPLICATIONS OF
NEPHROTIC SYNDROME
         INFECTION

• Impaired resistance to infection
  – -low immunoglobulin levels
  – -generalized protein deficiency
  – -defective opsonization
  – -splenic hypofunction
  – immunosuppressive therapy
• Peritonitis
• Pneumococcal infection
               TREATMENT
• Diuretics ?
• Albumin & Lasix infusion
• Prednisone 60 mg/m2/d x 4 weeks,
  then 40 mg/m2 every other day x 4
  wk
• Alternatives
  – Cyclophosphamide 2-3 mg/kg/d x 8
    weeks, not to exceed 200 mg/kg
  – Chlorambucil
  – Cyclosporine
                Requirements at a glance
                    Nephrolithiasis
Presentation
• Most patients present with abdominal, flank or pelvic
  pain depending upon the location of the calculus.
  Referred pain may be localized to the scrotum, penis or
  female genitalia.
• Patients may have associated nausea and vomiting,
  gross hematuria, or symptoms of a urinary tract infection
  (urinary frequency, dysuria, etc.)
• Not all patient with urinary calculi have gross or
  microscopic hematuria
• 20% of patients with microscopic hematuria and
  hypercalciuria will develop a urinary calculus within five
  years.
               Requirements at a glance
                   Nephrolithiasis
Medical Evaluation
• Family history is paramount as pediatric stones may be associated
  with inherited disorders such as cystinuria, primary hyperoxaluria or
  renal tubular acidosis.
• Patient’s past medical history including low fluid intake, dietary
  exess or deficiencies may predispose to calcium oxalate stone
  formation.
• Patients with a history of hyperthyroidism, myeloproliferative
  disorders, gastrointestinal disorders, chronic urinary tract infections
  or immobilization may be at increased risk for stone formation.
• Infants with a history of furosamide (Lasix) use are at an increased
  risk for stones and nephrocalcinosis.
• Pediatric patients with a history of stones are at an increased risk for
  recurrent stone formation
               Requirements at a glance
                   Nephrolithiasis
Radiographic evaluation
• CT scan without contrast is the most sensitive study for the
  detection of urinary calculi.
• KUB and renal ultrasound may be useful in specific situations.

Laboratory Evaluation
• Urinalysis of a first morning void including pH, specific gravity and
  present of bacteria are useful in the evaluation.
• Measurement of serum electrolytes including sodium, potassium,
  bicarbonate, chloride, uric acid, calcium, phosphorus and creatinine
  may provide useful information.
• A 24 hour urine analysis for volume, calcium, oxalate, citrate, uric
  acid, cystine, sodium, phosphate and creatinine. Measurements
  must be corrected to patient body mass.
        Outline – Part 2

• Dehydration
• Acute renal failure
• Chronic renal failure
• Fluids & Electrolytes
• Tubular disorders
• Cystic kidney disease
                          SCENARIO
A 10-day male infant presents with a history of irritability, low
grade fever, emesis and diarrhea. Prenatal and family history
is non-contributory. On examination the infant is irritable, temp
is 38°C, has mottled skin and a capillary refill of 4 sec. The
systolic blood pressure is barely palpable and the pulse is 195
beats/min. The anterior fontanelle is flat.
Hemoglobin               18 g/dl
White cell count         30,000
Platelets                280,000

What are key features in the history and examination?
What studies would you perform?
What is your initial therapy?
What is your initial diagnosis (es)?
        Dehydration: Clinical
• Importance of clinical history
  – Feeding history (BF, formula error)
  – Other conditions (CF, CV disease)
• Features to assess: change in weight,
  altered VS, orthostatic changes.
  turgor/refill, fontanelle, tears
• No specific diagnostic laboratory test –
  only supportive
• Key feature is reversibility
     Dehydration: Laboratory
• Urine
  – S.G.
  – UNa, FENA, Uosm
  – Microscopic examination
• Blood
  – BUN, creatinine
  – Bicarbonate
       Dehydration: Therapy
• Emergent therapy
  – 10-20 ml/kg boluses
  – Isotonic solution
  – Repeat until any evidence of improvement

• Correction therapy
  – Isotonic over 24 hours
  – Ongoing losses: diarrhea, vomiting
• Maintenance therapy
        Dehydration: Pearls
• Dehydration is reversible
• Dehydration is a misnomer and reflects
  loss of sodium not water
• Isotonic solutions are fluid of choice
• FENA is best test to assess severity of
  dehydration
                           SCENARIO
A 6 year boy is diagnosed as having ALL. He is started on
chemotherapy and his white blood cell count drops
precipitously. The child is discharged and the family is
encouraged. However, after two days at home he spikes a
temperature to 39 C. The parents contact the heme/ onc
fellow who tells them to come to the hospital immediately.

On arrival to the ER, the child is a bit lethargic. His BP is
60/40.

What is the most important first step in the management of
this child?
What are the most useful diagnostic tests?
What are the possible causes of his condition?
How should his condition be treated?
   Acute Renal Failure (ARF) vs
       Pre-renal Azotemia
• Key maneuver is restore RBF to
  distinguish reversible pre-renal state from
  short-term irreversible
• Options
  – Bolus infusion of crystalloid solutions
  – Infusion of albumin
  – Administration of pressors
  – Administration of antagonists of clinical
    condition as in anaphylaxis
              ARF: Diagnosis
       Pre-renal   AGN      ATN       Obstruction

UA     Marginal    Key       RTEC     Marginal value
       value       RBC casts
SG     >1.020      >1.020   1.008-    1.008-1.012
                            1.012

UNa    <20         <20      >40       >40


FENA   <1%         <1%      >1%       >1%


Uosm   >400        >400     200-400   200-400
         ARF: Diagnosis
• AGN
 – PSAGN
 – HSP
 – SLE
 – MPGN
 – Wegener’s
          ARF: Diagnosis
• ATN
 – Unreversed pre-renal azotemia
 – Nephrotoxic meds
 – Contrast agents
 – High calcium, uric acid, phosphate
 – Rhabdomyolysis (myoglobin)
 – Intravascular hemolysis (hemoglobin)
           ARF: Diagnosis
• Obstructive uropathy
  – PUV
  – Prune belly
  – Vesicoureteric reflux
  – Neurogenic bladder (myelomeningocele)
  – Megacystis/megaureter
  – Secondary: stones, fibrosis
• Effect of age and gender
               ARF: Testing
•   Key labs: BUN, creatinine, K
•   EKG
•   CXRay
•   Renal ultrasound
•   Specific blood tests based on underlying
      condition
         ARF: Management
• Urgent issues
  – Potassium
    • Calcium
    • Glucose/insulin
    • NOT bicarbonate
  – Blood pressure: parenteral therapy
    • Labetalol
    • Nitroprusside
  – ECF volume
          ARF: Conservative
            Management
• Potassium
  – Diet restriction
  – Kayexalate
• Blood pressure
  – IV/PO meds
• ECF volume
  – Na restriction
  – Diuretic use – need for furosemide
    ARF: Indications for Dialysis
•   Refractory hyperkalemia
•   Refractory hypertension
•   Symptomatic ECF volume overload
•   Symptomatic azotemia
    – Infection
    – Bleeding
    – CNS changes
             ARF: Pearls
• Pre-renal azotemia and AGN are similar
• ATN and post-renal failure are similar
• Potassium kills first in ARF
                           SCENARIO
A 6 year boy is seen at a routine physical examination.
Although he has no specific complaints, his mother says he
has been very listless and his appetite is very poor. He has
not been playing well with his friends in play group. Although
he is toilet trained he seems to be having more accidents
during the night.

On examination, he looks a bit pale and tired. His height has
fallen from the 50% at his last visit 18 months ago to 10%. His
BP is 106/62 mm Hg.

What is the most important first step in the diagnosing this
child’s problems?
What are the likely causes his condition?
How should his condition be treated?
           CKD: Diagnosis
• Stages
  – CKD I: renal injury GFR >90
  – CKD II: GFR 60-90
  – CKD III: GFR 30-60
  – CKD IV:GFR 15-30
  – CKD V: ESRD
       CKD: Common features
•   Impact on growth
•   Impact on bone: osteodystrophy
•   Impact on puberty
•   Impact on development – social and
    cognitive
             CKD: Causes
• Non-glomerular
  – Hypoplasia/dysplasia
  – Reflux nephropathy
  – Obstructive uropathy
    • PUV
    • Prune Belly
    • Neurogenic bladder
  CKD: Clinical manifestations
• Growth failure
  – Dependent on age of onset
  – Dependent on level of GFR
• UTIs
  – Pyelonephritis
• Electrolyte abnormalities
  – Pseudohypoaldosteronism
  – Nephrogenic DI
• Neurocognitive disability
           CKD: Diagnosis
• Structural assessment
• Imaging studies
  – US
  – VCUG: dye vs radioisotope
  – DMSA scan
  – Retrograde studies, etc
           CKD: Diagnosis
            ARF
Younger child, abd mass, UTI


           UA
WBC, impaired concentration



     US, VCUG, DMSA


                                  Retrograde studies
                               Cystoscopy, urodynamics
                           SCENARIO
A 15 year old girl comes to the clinic because she has not had
her period for the last 8 months. She feels tired all the time at
home school and is having a hard time concentrating in
school.

She is not taking any medications except for occasional
NSAIDs for headaches and some vitamins. Her parents are in
good health.

On examination, her height and weight are normal. Her BP is
162/98 mm Hg. She is pale and has a mild amount of edema
in both legs. She has no rash or arthritis.

What is the most important first step in diagnosing this
adolescent’s problem?
What are the most likely causes?
How should her condition be treated?
               CKD: Causes
• Glomerular
  – FSGS
  – HUS
  – SLE
  – Membranoproliferative MPGN)
  – Alport
  – IgA Nephropathy
  – Membranous nephropathy
  – NOT diabetic or hypertensive nephropathy
   CKD: Clinical manifestations
• Growth failure
  – Dependent on age of onset
• Hypertension
  – Role of ECF volume and PRA
• Electrolyte abnormalities
  – Acute
  – Hyperkalemia
• Edema
• Signs of underlying disease
           CKD: Diagnosis
• Low value of radiology tests
• Blood tests
  – C3, C4, CH50
  – ASLO
  – ANA, dsDNA, Ro, La, Sm
  – ANCA
  – Anti-GBM
  – Renal biopsy
         CRF: Management
• Nutritional supplementations
  – CHO deficiency
• Protein restriction
  – Impact on growth
  – Effect in more advanced CKD
• BP control
  – Disease progression
  – ACEI/ARB
         CRF: Management
• Interference with renin-angiotensin
  aldosterone axis
  – Safety of ACEI even with advanced CKD
  – Role of combined ACEI/ARB
  – Effect of aldosterone antagonists
• Safety issues
  – Hyperkalemia
  – Reduction in GFR
           CRF: Management
• Endocrine treatments
  – rhGH
     • Doubles growth velocity
     • Minimal risk of progression
  – Erythropoietin
     • Nearly always effective
     • Antibody induced pure red cell aplasia
  – Calcitriol
     • IV route
     • More selective agents
              CRF: Pearls
• Chronic glomerular diseases have oliguria
  vs chronic tubular diseases which can
  have polyuria and sodium loss
  – Nocturia and enuresis may indicate CRF
• Severity of growth failure and
  neurocognitive deficits are inversely
  related to age of onset of CRF
         CRF: More pearls
• Most important feature of nutritional
  support is to correct low caloric intake
• Medication doses need to be adjusted as
  GFR declines
• Almost no form of CRF is a
  contraindication to transplant
                          SCENARIO
A 10-day male infant presents with a history of irritability,
low grade fever, emesis and diarrhea. Prenatal and family
history is non-contributory. On examination the infant is
irritable, temp is 38°C, has mottled skin and a capillary
refill of 4 sec. The systolic blood pressure is barely
palpable and the pulse is 195 beats/min. The anterior
fontanelle is flat.
Hemoglobin                  18 g/dl
White cell count            30,000
Platelets                   280,000

What are key features in the history and examination?
What studies would you perform?
What is your initial therapy?
What is your initial diagnosis (es)?
 Electrolyte Disorders: Sodium
• KEY function of Na+
  – ECF cation
  – Maintenance of intravascular compartment
• Disturbances in ECF volume are
  secondary to disturbances in Na+ balance
• ECF volume assessment is clinical
  – Reduced – see dehydration above
  – Increased – pulmonary and/or peripheral
    edema
Electrolyte disorders: Sodium

                  Assess ECF




    Measure serum Na




High ECF          Normal ECF   Low ECF
    Electrolyte Disorders: Sodium
•   History
•   Source of Na loss
•   Change in body weight
•   Renal response to low ECGF volume
    – Oliguria
    – Reduced urine Na+
    – Reduced FENA
  Electrolyte disorders: Sodium
120

100

80
                               ICF
60
                               ECF
40

20

 0
      Normal   Hypo    Hyper
  Electrolyte disorders: Sodium
• Hypernatremia
  – Risk factors
     •   Breast feeding
     •   Feeding errors
     •   Impaired thirst
     •   Impaired access to water
  – Presentation
     • Irritability, seizures
  – Treatment
     • SLOW
     • HYPOTONIC FLUIDS – 1/5 NS
  Electrolyte disorders: Sodium
• Hyponatremia
  – Risk factors
     •   Feeding errors (Keating)
     •   Salmonella diarrhea
     •   Increased extra-renal salt loss
     •   Pain, anesthesia, post-operative picture
     •   Female gender
  – Presentation
     • Lethargy, seizures
  – Treatment
     • ?SLOW
     • Correction 25 mmol/L OR 130 mmol/L over initial 48 hr
   Electrolyte disorders: Sodium
• Bad outcomes
• Brain
  – Hemorrhage and cerebral edema in
     hypernatremia
  – Osmotic demyelinating syndrome and acute CNS
    deterioration in Hyponatremia
• DKA
  – ?Hyponatremia (100 glucose mg/dl  1.6 Na meq/l)
  – Comparison to hypernatremia
                        SCENARIO
A 4-week old infant presents with a history of irritability, low
grade fever and poor feeding. Prenatal and family history is
non-contributory. On examination the infant is irritable,
temp is 37°C, has dark skin and a capillary refill of 4 sec.
The systolic blood pressure is barely palpable and the
pulse is 195 beats/min. The anterior fontanelle is sunken.
Hemoglobin              18 g/dl
White cell count        30,000
Platelets               280,000

What are key features in the history and examination?
What studies would you perform?
What is your initial therapy?
What is your initial diagnosis (es)?
Electrolyte Disorders: Potassium
• KEY function of K+
  – ICF cation
  – Transmembrane potential, secretion,
    neuromechanical coupling
• Disturbances in K+ reflect sudden
  changes in serum concentration and
  transmembrane ratio
• Assessment is linked to cardiac impact of
  abnormal K+ concentration
Electrolyte disorders: Potassium
• Regulatory organs
  – Kidney secretion
       • Na+
       • Urine flow rate
  – Adrenal
       • Aldosterone
  – GI tract
• Transmembrane
  –   pH
  –   Osmolality
  –   Beta adrenergics
  –   Insulin
• Diet
                Potassium
• Key tests
  – BUN, Cr, Na, K, bicarbonate
  – Urine K useless
  – Urine Na/K ratio
  – Hormones
    • PRA
    • Aldosterone
Electrolyte disorders: Potassium
• Hyperkalemia
  – EKG
    • Peaked T waves
  – Treatment
    •   Calcium infusion
    •   Glucose/insulin
    •   NOT Bicarbonate
    •   Kayexalate
    •   DIALYSIS
Hyperkalemia: differential diagnosis
• No real disease
  – Increase cells: WBC, polycythemia, thrombocytosis,
    crush injury
  – Transmembrane
• Renal
  – ARF
  – CRF
  – Liddle’s
• Adrenal
  – Adrenal failure
  – Congenital adrenal hyperplasia – ambiguous genitalia
  – Isolated renin abnormalities
        Hyperkalemia: Work-up
•   BUN, creatinine, Na, K, Bicarbonate
•   PRA
•   Aldosterone
•   Urinary Na/K ratio
Electrolyte disorders: Potassium
• Hypokalemia
  – EKG
    • U waves
  – Treatment
    • Restore ECF volume to 2hyperaldosteronism
    • PO potassium
       – Limitations: tolerance
    • IV potassium
       – Limitation: 0.3 meq/kg/hr
       – Central vs peripheral IV
Hyperkalemia: differential diagnosis
• Systemic
  – Malnutrition

• Adrenal
  – Adrenal overactivity
  – Congenital adrenal hyperplasia
  – Primary renin abnormalities
• Renal
  – DKA
  – Osmotic diuresis
                       SCENARIO
A 15 month child presents with a history of poor feeding
and impaired growth. Prenatal and family history is non-
contributory. On examination the infant’s height and
weight are below the 5th percentile. The systolic blood
pressure is 102 and the pulse is 110. The rest of the
examination is normal.
Na               138
Cl               114
Bicarbonate      16

What are key features in the history and examination?
What studies would you perform?
What is your initial therapy?
What is your initial diagnosis (es)?
Electrolyte disorders: acid-base
              Acid load




          Chronic-Kidney             Chronic-Kidney
  Acute
             Proximal                    Distal



          Reclaim filtered             Regenerate
  Lung
           bicarbonate            Titrated bicarbonate



                        Large frequent
                                                 1-3 mmol/kg/day
                            doses
        Electrolyte disorders
• Anion gap

• [Na] – {[Cl] + [HCO3]}

• Normal value: 4-12

• Impact of serum albumin
  Electrolyte disturbances: RTA
• Metabolic acidosis
  – Normal anion gap -- hyperchloremic
     • Diarrhea
     • RTA
  – High anion gap -- normochloremic
     • MUDPIES or KUSSMAUL
     • Key entities:
        –   DKA
        –   Lactic acidosis
        –   Uremia
        –   Metabolic disease
        –   Toxins
 Electrolyte disturbances: RTA
• Proximal
  – Low K
  – Primary
  – Secondary
    •   Glycogen storage
    •   Wilson’s, fructose intolerance, tyrosinemia
    •   PTH, Vitamin D
    •   Cystinosis
  Electrolyte disturbances: RTA
• Distal
  – Primary
  – Secondary
     • Transplant rejection
     • Drugs: amphotericin, cisplatinum
     • Collagen vascular disease
   Electrolyte disorders: RTA
• Assessment
  – SMAC: Cl-
  – VBG: Bicarbonate
  – Urine: calcium, citrate
  – Urine anion gap: unmeasured cation (NH4+)
  – Xrays
 Electrolyte disturbances: RTA
• Treatment
• Proximal
  – Higher doses of bicarbonate
  – More frequent dosing
  – Exacerbation of hypokalemia with Rx
• Distal
  – 1-3 mmol/kg varying with age and diet
  – 3 doses
  – Stabilization of K with Rx
Electrolyte disorders: Fanconi’s
                Fanconi’s
                Syndrome




              Complete proximal
              tubule dysfunction




                         Phosphaturia   Amino
 RTA    Glycosuria
                            TRP        Aciduria
   Electrolyte disorders: metabolic
               alkalosis
• Extrarenal/GI loss of K
  – CF
• Vomiting
  – NG suction
  – Pyloric stenosis
• Distal GI loss of bicarbonate
  – Chloride diarrhea
• Renal
  – Bartter’s
  – Gitelman’s
  – Apparent mineralocorticoid excess (AME)/licorice
     Electrolyte disorders: DI
• Central
• Nephrogenic
• Risk of CNS disease
  – 1/12 (1/3 X ¼) of loss from ECF
  – Limited access to water
  – Altered thirst
     Electrolyte disorders: DI
• Central
  – AVP replacement
• Nephrogenic
  – Adequate water intake
  – Low solute diet
  – Hydrochlorothiazide
        Electrolytes: Pearls
There are three pure renal causes of FTT –
 azotemia, DI, and RTA
RTA causes hyperchloremic acidosis
Bartter’s and Gitelman’s differ in calcium
 excretion – high in former low in latter
Thank you

GOOD LUCK

								
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