K - 7 Short Stature & Tall Stature

8/10/2019 K - 7 Short Stature & Tall Stature

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Short Stature and Tall Stature

Dr.HAKIMI,SpAK

Dr.MELDA DELIANA,SpAK

Dr.Siska Mayasari Lubis,SpA

Dept. of child health

Medical School, University of North Sumatra


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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

GH Sex 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

CanalizationCatch-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, RWT

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

105

110

115

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135

140

145

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155

160

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185

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195

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

160

165

170

175

180

185

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195

2 4 6 8 10 12 14 16 18

Age (years)

Height

(cm)

97

50

3

HACA

Tinggi Potensi Genetik


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Bone age

Greulich & Pyle Comparison of left wrist

Prediction of FH after 6 years Table Bayley & Pinneau

Tanner Whitehouse II

Maturation of ossificationcenter 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 of growth more important

rather than absolute position on

growth curve


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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

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 ShortStature

Short Stature

Abnormal

Normal

Constitutional DelayNormal Variant

Proportional Dysproportional

ExtrauterineIntrauterine


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Endocrinological causes

Normal variant : CDGP

GH Deficiency

Hypothyroid

Precocious Puberty

Cushings syndrome

Pseudohypoparathyroidism:genetic disorder in which

the body fails to respond to parathyroid hormone. If you have pseudohypoparathyroidism,

your body produces the right amount of PTH, but is "resistant" to its

effect. This causes low blood calciumlevels and high blood phosphatelevels.
http://www.nlm.nih.gov/medlineplus/ency/article/003690.htmhttp://www.nlm.nih.gov/medlineplus/ency/article/003690.htm


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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 : ITTinsulin tolerance test& Arginine,

Low IGF-1


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Causes of GH Deficiency

Congenital

Idiopathic

Genetic Assosiated anatomic defect : SOD Septo-optic

dysplasia, 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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Cushings 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

Albrights 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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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


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Documents

K - 7 Short Stature & Tall Stature