GANGGUAN PERTUMBUHAN PADA ANAK by 18kK3o4

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									    GANGGUAN
PERTUMBUHAN PADA
      ANAK
                   Gangguan pertumbuhan

                   G ro w th C h a rt                           Perawakan normal
             195

             190

             185
                                                       97       pertumbuhan
                                                                 terganggu
             180

             175                                        50
             170

             165                                        3
             160

             155

             150

             145

H e ig h t   140

(c m )       135

             130

             125

             120

             115

             110

             105

             100

              95

              90

              85

              80
                   2   4   6   8   10   12   14   16   18

                           A g e (y e a rs )
                   Gangguan pertumbuhan

                   G ro w th C h a rt                           Perawakan pendek
             195

             190

             185
                                                       97       pertumbuhan normal
             180

             175                                        50
             170

             165                                        3
             160

             155

             150

             145

H e ig h t   140

(c m )       135

             130

             125

             120

             115

             110

             105

             100

              95

              90

              85

              80
                   2   4   6   8   10   12   14   16   18

                           A g e (y e a rs )
        ETIOLOGI PERAWAKAN
              PENDEK
Perawakan pendek dapat disebabkan oleh kelainan
  endokrin ataupun non endokrin seperti
 Genetik atau familial
 Kelainan kromosom atau sindrom tertentu
 Penyakit kronis
 Gangguan gizi
 Deprivasi psikososial
 Skeletal disorder
 Intra Uterine Growth Retardation (IUGR)
 Constitutional Delay of Growth and Pubeerty (CDGP)
 Kelainan endokrin : defisiensi GH, Hipotiroidisme, dll.
            PERAWAKAN PENDEK


        KECEPATAN TUMBUH NORMAL?

   YA                              TIDA
                                     K

VARIAN NORMAL                   PATOLOGIS



                PROPORSI ?     DISMORFISM ?


                  BB/TB?
             PERAWAKAN PENDEK

                                       PATOLOGIS


  PROPORSIONAL
                                DISPROPORSIONAL

      BB/TB           BB/TB 

                                           KELAINAN
   ENDOKRIN
                    •MALNUTRISI            DISMORFIK
•DEFISIENSI GH
                    •INFEKSI KRONIS
•HIPOTROID
                    •PENYAKIT
•KORTISOL           KRONIS
•PSEUDOHIPOPARA     (ORGANIK)
TIROID              •PSIKOSOSIAL
                    •IUGR
    PERAWAKAN PENDEK


                          PATOLOGIS


 DISPROPORSIONAL                   KELAINAN DISMORFIK


•DISPLASIA TULANG             •KELAINAN KROMOSOM
  A/HIPO CHONDROPLASIA                TRISOMI 21
•KELAINAN METABOLIK                SINDROM TURNER
         RICKETS
                              •SINDROM-SINDROM ( IUGR)
•GANGGUAN SPINAL
                                FETAL ALCOHOL, RUSSELL-
   RADIASI KRANIOSPINAL           SILVER, PRADER-WILLI,
   SPONDYLODYSPLASIA           NOONAN, SECKEL, de LANGE,
                                  LARON, COCKAYNE dll
             PERAWAKAN PENDEK


VARIAN NORMAL



 FAMILIAL SS          CONSTITUTIONAL DELAY OF
                       GROWTH AND PUBERTY


USIA TULANG = USIA     USIA TULANG < USIA
KRONOLOGIS              KRONOLOGIS
TINGGI DEWASA <        TINGGI DEWASA >
PERSENTIL-3             PERSENTIL-3
SESUAI POTENSI         SESUAI POTENSI
GENETIK                 GENETIK
                        RIWAYAT KELUARGA (+)
Pola-pola pertumbuhan linier
Pola-pola pertumbuhan linier
    Sindrom Turner (ST)
 Tinggi dewasa 137-146.8 cm
 Etiologi perawakan pendek
   aksis longitudinal tubuh
   gangguan tulang panjang lebih berat dari
    vertebra
                                     (Lippe, 1993)

 Rosenfeld   et al (1994)
   Pemberian dini terapi GH
   Kombinasi GH + oxandrolone
   Estrogen  induksi pubertas
          Penatalaksanaan
 Familial short stature : tidak diterapi
 Constitutional delay of growth and puberty
  (CDGP): tidak diterapi
 Tergantung kausal : nutrisi, infeksi, dll
 Hormonal : GH, tiroid, sex steroid
 Dysproporsional SS : achondroplasia,
  osteogenesis imperfecta, sindr. Down, dll;
  tidak diterapi
Anak umur 5 tahun ,)
Tall Stature
                  Introduction
 Definition
  Tall stature : height above 97th percentile
  for age, sex and race.
 GH excess, occurs during childhood when
  open epiphyseal growth plates allow for
  excessive linear growth
 Cause
     Intrinsic
     Acquired
           Growth cessation

   Puberty  sex steroid
    (estrogen)  
    epiphyseal fusion
   Bone age
       Girls 14 – 16 yrs
       Boys 18 – 20 yrs
   Sex steroid & growth
       Low dose: stimulate
       High dose: inhibit
    Short Stature History
Mother and fathers heights.
MPH = M(cm) + F (cm) 13 cm /2.
MPH range 8 cm.
FH short stature: males <165 cm
                females <152 cm
FH delayed puberty: menarche >14 yrs in
females and continued growth after high
school in males.
Look at other sibs child development
Child Development record

A valuable source of information.
Look at all available height and weight
measurements and growth trend.
 Remember that Plunket height
measurements are not precise and may be
misleading.
Check developmental milestones and
illnesses.
                       Soal
   Data anak lelaki
     usia 7tahun 4 bulan : 110 cm; bone age 5
      tahun
     usia 8 tahun : 114 cm; bone age; 6 tahun
     usia 9 tahun 6 bulan : 122 cm; bone age 7
      tahun
 Tinggi ayah 172 cm, tinggi ibu 166 cm
 Ibu menarche 15 tahun
  EVALUASI PERTUMBUHAN ANAK INI!
               Grow th Chart
         195       Familial Short Stature
         190

         185

         180

         175

         170

         165

         160

         155

         150

         145

Height   140

(cm)     135

         130

         125

         120

         115

         110

         105

         100

         95

         90

         85

         80
               2      4     6    8    10    12   14   16   18

                           Age (years)
                Grow th Chart
         195
               Constitutional Delay of Growth & Puberty
         190

         185

         180

         175

         170

         165

         160

         155

         150

         145

Height   140

(cm)     135

         130

         125

         120

         115

         110

         105

         100

         95

         90

         85

         80
                 2    4    6    8    10   12    14   16   18

                           Age (years)
       Normal Variant Short
            Stature
               FSS           CDGD
Bone Age       <1 yr from CA >1 yr from
                             CA
Puberty        On time       Delayed
Final Height   Short         Normal
 Pathological short stature

Proportionate: IUGR
               syndromes
               chronic illness
               drugs
               psychsocial deprivation
Disproportionate: Syndromes (partic Turner
S)
               hypothyroidism
             IUGR/SGA
Intrauterine growth retardation or small for
gestational age.
Very common.
Birth weight <10th PC for gestational age.
Catch-up growth above 3rd PC usually
occurs by 6 mos of age but may drag on to
2 yrs.
Short stature by 2 yrs usually associated
with short final height.
As a group these children do not reach
        Turner Syndrome

Consider in all girls with unexplained short
stature or Ht below MPH range.
Commonest feature is short for MPH
(100%).
50% will only have short stature as clinical
feature.
Present with short stature, poor HV or
delayed puberty.
                    Normal Growth
The ICP (infant/child/puberty) model of growth (Karlberg model)

Mathematically growth is characterised by 3 periods of growth
        Infant - Birth to 2 years. Rapid growth at birth declining
rapidly over the first 2 years of life – less growth hormone
dependent.
        Childhood - 2 years until puberty. Relatively constant
annual growth - growth hormone dependent.
        Puberty - growth primarily dependent on sex steroids and
increased growth hormone release. Sex steroids cause eventual
fusion of skeletal epiphyses and growth arrest.
       Intrinsic tall stature


 Familial genetic tall stature /
constitutional
   Cerebral gigantism (Soto’s syndrome)
   Marfan syndrome
   Homocystinuria
   Multiple endocrine neoplasia type 2b
    Intrinsic tall stature

Chromosome
       47, XYY; Klinefelter syndrome (46, XXY);
       Fragile X syndrome
Beckwith-Wiedemann     syndrome (IGF2)
Weaver syndrome

Simpson-Golabi-Behmel    syndrome (GPC3)
Bannayan-Riley-Ruvalcaba    syndrome (PTEN)
Deficiency of aromatase/loss of function
mutations of estrogen receptor (a) in male
     Acquired tall stature
Infant   of diabetic mother (hyperinsulinism)
Obesity   (tall child normal adult height)
Sexual   precocity (tall child but short adult)
Primary    hypogonadism (eunuchoid)
Hyperthyroidism

Growth hormone excess (Gigantism,
acromegaly)
    Evaluation – History & PE
 Family history (constitutional, Marfan,
  familial precox etc)
 Developmental history
 Birth weight and length
 Stigmata of syndrome
 Pubertal status
               Evaluation – Lab
   Growth pattern
       Parallel or not
       Potential genetic height
   Bone age (prediction of final height)
   As indicated
       Chromosome
       Mutation analysis
       Others (hormonal, imaging, cardiovascular, eye etc)
Marfan-arachnodactyly
             Soto's syndrome
   rare genetic disorder with excessive physical
    growth during the first 2 to 3 years of life.
   mild mental retardation, delayed motor,
    cognitive, and social development, hypotonia
    (low muscle tone), and speech impairments.
   large at birth, large heads (macrocrania)
   disportionately large and long head with a
    slightly protrusive forehead, large hands and
    feet, hypertelorism (an abnormally increased
    distance between the eyes), and downslanting
    eyes.
       Klinefelter syndrome
 Tall stature
 Chromosom : 47, XXY
 Tend to gynecomastia, Ca. mammae
 Micropenis, infertility
                  Therapy
 Causal
 Constitutional Tall stature
     reassurance
     Boys: testosterone 500mg/m2/month
     Girls: estradiol 0,1 mg/day
 PENDEKATAN DIAGNOSIS GANGGUAN
         PERTUMBUHAN
Kreteria awal untuk pemeriksaan lebih lanjut :
1.  Tinggi badan lebih dari 2,5 dibawah tinggi badan rata-
    rata untuk umur kronologisnya
2.  Kecepatan tumbuh dibawah persentil ke 25 kurva
    kecepatan tumbuh atau kurang dari 4 cm /tahun pada
    anak berumur 4-10 bulan.
3.  Prakiraan tinggi dewasa dibawah potensi tinggi
    genetiknya.
4.  Kecepatan tumbuh melambat setelah umur 3 tahun
    dan turun menyilang garis persentilnya pada kurva
    panjang/tinggi badan .
 PENDEKATAN DIAGNOSIS GANGGUAN
         PERTUMBUHAN
ANAMNESIS
Riwayat perinatal, panjang / berat lahir, trauma
  lahir
Riwayar tumbuh kembang
Asupan nutrisi
Riwayat penyakit
Lingkungan psikosoaial
 PENDEKATAN DIAGNOSIS GANGGUAN
         PERTUMBUHAN
PEMERIKSAAN FISIK
Stigmata sindrom dismorfik / kromosom
Tingkat kecerdasan
Tanda / gejala penyakit sistemik
Tanda KEP
Tingkat maturasi kelamin
Antropometri
        PENDEKATAN DIAGNOSIS
       GANGGUAN PERTUMBUHAN
    PEMERIKSAAN PENUNJANG

 FT4, TSH
 LED, darah rutin (CBC)
 Elektrolit, BUN, creatinin, urinalisis,
 tinja
 Skrining TBC
 Umur tulang (bone age)
 Rujuk untuk pemeriksaan GH / IGF-1
ACHONDROPLASIA
             HIPOTIROID
              DIDAPAT




HIPOTIROID
KONGENITAL
           KESIMPULAN
 Pertumbuhan menggambarkan keadaan
  kesehatan seorang anak
 Pemantauan pertumbuhan memerlukan
  pengukuran teratur dengan alat yang
  tepat,
  penting interpretasi hasil pemantauan
 Perlu diingat bahwa gangguan
  pertumbuhan
   lebih sering disebabkan oleh kelainan
  non-endokrin

								
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