7.19 KULIAH Short Stature&Tall Stature
Transcript of 7.19 KULIAH Short Stature&Tall Stature
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Short stature and tall stature
Dr. ELLI KUSMAYATI, SpA
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Growth Normal growth
Infant
Childhood
Puberty
Growth : change in size & maturation
Stature : size
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Infant Childhood Puberty
Cm/tahun
GHSex steroid
Growth Velocity Curve
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Growth rate by age
1-6 mo : 18 - 22 cm/yr
6-12 mo : 14 – 18 cm/yr
1 yr : 11 cm/yr
2 yr : 8 cm/yr3 yr : 7 cm/yr
4 – puberty : 5 – 6 cm/yr
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Potensi TinggiGenetik
Canalization Catch-down
INFANT PERIOD
Growth Chart
0 1 2 3
cm
tahun
Catch-up
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Evaluation of growth Anthropometry
Standing & sitting : proportional
Arm span
Bone age :
GP, TW, RUT
Normal, delayed, advanced
prediction of final height
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Growth chart Growth curve
NCHS, Jumadias, Husaini
Plotting growth
Normal, short, tall
Interpretation of plot
Potential genetic height
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Growth Chart
80
85
90
95
100
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115
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135
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145
150
155
160
165
170
175
180
185
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2 4 6 8 10 12 14 16 18
Age (years)
Height
(cm)
97
50
3
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Growth Chart
80
85
90
95
100
105
110
115
120
125
130
135
140
145
150
155
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165
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180
185
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195
2 4 6 8 10 12 14 16 18
Age (years)
Height
(cm)
97
50
3
HA CA
Tinggi Potensi Genetik
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Bone age Greulich & Pyle
Comparison of left wrist(pergelangan
tangan) Prediction of FH after 6 years
Table Bayley & Pinneau
Tanner Whitehouse II Maturation of ossification center
More reliable : scoring system
complicated
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Usia tulang Tangan kiri cara
Atlas Greulich - Pyle
Tanner-WhitehouseII
RWT
Pusat-pusat osifikasi
Prediksi tinggi akhir
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Short stature Height < 2SD
Sex, age and race
Pattern(pola) of growth more importantrather than absolute position on growthcurve
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Short stature
Variants of normal
Prenatal onset
Post natal onset
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Variants of normal (normal growth
velocity) Familial short stature
Parents height genetically short
normal bone age short adult
Constitutional delay of growth &
puberty Delayed puberty in the family
Normal growth velocity
normal adult height
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Prenatal onset Primary growth deficiency
Malformation
syndromes
Secondary growth deficiency
Low maternal socioeconomic
Undernutrition
Maternal disorder or disease
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Post natal onset Nutritional
Deprivation syndrome
Cardiac defect
Respiratory insufficiency
Renal disfunction Hormonal
Chronic infection, chronic disease
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Etiology Primary disturbances of growth
Skeletal dysplasias
Chromosomal abnormalities
Metabolic causes
IUGR (retardasi intrauterine)
Syndromes Genetic
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Etiology Secondary growth disturbances
Undernutrition
Disorder of the bowel, kidney, heart, lung
Psychosocial deprivation
Chronic infection, drugs
Endocrine disorder Idiopathic growth delay
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Diagnostic Approach to Short
Stature
Short Stature
Abnormal
Normal
Constitutional Delay Normal Variant
Proportional Dysproportional
Extrauterine Intrauterine
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Endocrinological causes
Normal variant : CDGP
GH Deficiency
Hypothyroid
Precocious (prekok) Puberty Cushing’s syndrome
Pseudohypoparathyroidism
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Treatment Depend on the etiology
Nutrition
Organic disease CHD, IDDM
Hormonal
GH, Thyroid, Sex hormone Mechanical
Bone lengthening : achondroplasia
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GH deficiency Short stature
Chubby
Abdominal adiposity
Micropenis
Single central incisor Frontal bossing
Proportional
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GH Deficiency Low growth velocity : < 25%
Retarded bone age
GH level < 10 ng/dl
Screening tests : sleep & excercise
Stimulation test
2 tests : ITT & Arginine,
Low IGF-1
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Causes of GH Deficiency Congenital
Idiopathic
Genetic Assosiated anatomic defect : SOD,midline defect
Acquired
Trauma
Neoplasma
Cranial irradiation
etc
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Treatment Varies: GH 14 - 21 IU/m2/day
6 times/week
Monitoring: Height, velocity
Side effect
Terminate : No response < 2 cm
Epiphyseal plate closure
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CDGP Family history
Growth velocity normal
Bone age retarded
Puberty : late
Adult height : normal
Treatment : No Need
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Congenital hypothyroidism Large posterior fontanelle
Respiratory difficulty
Hypothermia Feeding difficulty
Lethargy
Delay in passing meconium
Abdominal distention
Vomitting
Prolonged jaundiced
Oedema
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Cushing’s Syndrome Corticosteroid excess
Growth retardation due to
Interferring protein anabolism Impairing somatomedin production
Hypertension
Obesity predominant : trunk and neck, moon
face Disturbances of glucose metabolism
Purple striae, hirsutism, osteoporosis,hypogonadism
Muscular weakness
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Pseudohypoparathyroidism Short stature
Mental retardation
Albright’s osteodystrophy
Round facies
Ectopic calcification
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Pola pertumbuhan linier
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Pola pertumbuhan
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Conclusion Short stature
Etiology : varies; endocrinology
Treatable, depend on the etiology
Psychosocial problem
Growth monitoring : important
Anthropometric measurement
Clue to diagnosis
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Etiology Primary disturbances of growth
Skeletal dysplasias
Chromosomal abnormalities
Metabolic causes
IUGR
Syndromes Genetic
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Tall Stature Height > 2 SD
Age, sex and race
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Etiology Genetic
Familial tall stature
Familial rapid maturation
Hormonal GH excess
Hyperthyroid
Androgen/estrogen excess Syndromes
Weaver, Sotos, Marfan, Kleinefelter
Metabolic disorders
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Diagnostic approaches Height, velocity
Dysmorphism
Family history
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Treatment To treat or not to treat
Family or patient request
Contra indication Psychosocial problem
Female > male
Ethynil estradiol 100 ug/day Testosterone 200 - 250 mg every 2 – 3
week
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Conclusion Short stature & tall stature
Treatable, depend on the etiology
Psychosocial problem
Growth monitoring : important
Anthropometric measurement
Clue to diagnosis