Approach To Short Stature Pediatric Oncall

4th Pediatric Infectious Diseases Conference

ISSN 0973 - 0958

CHRISTMAS Pedicon 2005

APPROACH TO SHORT STATURE

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APPROACH TO SHORT STATURE

CHRISTMAS PEDICON 2005, RAJKOT

Dr. Mathew John, MD, DM (Endo), DNB (Endo)
Consultant Endocrinologist, Kerala Institute of Medical Sciences, Trivandrum.
Dr. Nalini S. Shah, MD, DM (Endo)
Prof. & Head, Dept of Endocrinology, Seth G.S. Medical College & KEM Hospital

Table 1: Classification of Growth Retardation

Primary Growth Abnormalities
1. Osteochondrodysplasias
2. Chromosomal abnormalities
3. Intrauterine growth retardation
Secondary Growth Disorders
1. Malnutrition
2. Chronic disease
  1. Malabsorption and Gastrointestinal Diseases
  2. Anemia
  3. Chronic Liver Disease
  4. Cardiovascular Disease
  5. Renal Disease
  6. Diabetes Mellitus
  7. Pulmonary Disease
3. Endocrine disorders
  1. Hypothyroidism
  2. Cushing's syndrome
  3. Pseudohypoparathyroidism
  4. Rickets a. Vitamin D-resistant rickets
  5. IGF deficiency
    - GH deficiency and insufficiency
      - Congenital
      - Acquired
    - GH insensitivity
      - Primary
      - Secondary
Idiopathic Short Stature
- Genetic short stature
- Constitutional delay of growth and maturation
- Heterozygous defects of the GH receptor.

Table 2 : Assessment of a Short Child

History:
- Exact age, ethnicity
- Birth history including birth weight, presentation at birth, signs and symptoms of GHD at birth (Micropenis, hypoglycemia, jaundice)
- Milestones
- Onset of short stature
- Any history suggestive of chronic diseases: cardiac symptoms, polyuria, malabsorption, tetany and seizures, rachitic deformities, hypothyroidism
- Childhood CNS infections and treatment including TB meningitis
- Signs of raised intracranial tension and visual abnormalities
- Intracranial surgery, childhood malignancies, chemotherapy and craniospinal radiation
- Weight gain, feeding abnormalities, mental subnormality, abnormal behavior
- Pubertal status
- Drug history, including chronic steroid intake, testosterone injection etc.
- Psychosocial history
- Family history including consanguinity, short stature and deformities, delayed puberty
Examination:
- Height (centile), Weight (centile), Segments (US: LS ratio), Arm span, Midparental height
- Pulse rate, blood pressure including lower limb BP and radiofemoral delay
- Signs of systemic disease: pallor, edema, goiter, clubbing, cyanosis, vitamin deficiency, signs of malnutrition, rickets.
- Dentition and dental age
- Stigmata of skeletal dysplasia
- Stigmata of Turner's syndrome and other syndromic short stature (Noonan, Prader Willi, Russel Silver syndrome, Seckel syndrome, Albrights osteodystrophy phenotype)
- Stigmata of GH deficiency (frontal bossing, midfacial hypoplasia, abdominal fat pads)
- Midline defects (single central incisor, cleft lip and palate)
- Systemic examination: CVS, chest and abdomen
- Signs of CPHD (Micropenis, hypothyroidism)
- Central nervous system including fundus (esp. optic disc) and visual fields.

Table 3: Investigations in a short child

Table 4 : Classification of GH deficiency syndromes (4, 49, 84)

A) CONGENITAL CAUSES

Hormone gene defects

Gene defect	Inheritance	Hormonal deficits	Associated features
GHRH gene			Not described
GHRH receptor	AR	GH	Dwarfism of Sindh
GH-1 gene-type 1A	AR	GH	No GH produced
GH-1 gene-type 1B	AR	GH	Abnormal GH
GH-1 gene-type II	AD	GH
GH-1 gene-type III	X linked	GH	Hypogammaglobulinemia
Bioinactive GH	AD	GH

Abnormal pituitary development

PROP1	AR	GH, TSH, Prl, LH, FSH+ ACTH	Pituitary hyperplasia
Pit-1 (POU1F1)	AR	GH, TSH, Prl	Anterior pituitary hypoplasia
HESX1	AR / AD	GH, TSH, Prl, LH, FSH+ ACTH	Septo-optic dysplasia
Lhx3	AR	GH, TSH, Prl, LH, FSH GH, TSH, Prl, LH, FSH	Rigid cervical spine
Lhx4	AD	GH, TSH, ACTH	Cerebellar tonsil herniation
Pitx2 (REIG)	AD	? GH / Prl	Reiger's syndrome
SOX3	X linked	GH	Mental retardation

Associated with structural defects of the brain
1. Agenesis of corpus callosum
2. Septo-optic dysplasia
3. Holoprosencephaly
4. Encephalocoele
5. Hydrocephalus
Associated with midline facial defects
1. Cleft lip and palate
2. Single central maxillary incisor
Miscellaneous
1. Prader Willi syndrome

B. ACQUIRED CAUSES

Trauma: perinatal trauma, postnatal trauma
Infections: meningitis, encephalitis
Langerhans cell histiocytosis
CNS tumors: craniopharyngioma, pituitary germinoma, pituitary adenoma, optic glioma
Post cranial radiation
Post chemotherapy
Pituitary infarction
Psychosocial deprivation
Neurosecretory dysfunction
Hypothyroidism
Thalassemia

Table 5: Classification of Growth Hormone Insensitivity

(i)	Primary GH insensitivity Growth hormone receptor (GHR) defects (quantitative and qualitative) Abnormalities of GH signal transduction (postreceptor defects) Primary defects of insulin like growth factor-1 (IGF-I) synthesis or secretion Bioinactive GH molecule
(ii)	Secondary GH insensitivity Circulating antibodies of GH that inhibit GH action Antibodies to the GHR GHI caused by malnutrition GHI caused by liver disease

Table 6: Tests to Provoke Growth Hormone Secretion

Test	Procedure	Sampling	Comments
Exercise	Step climbing; exercise cycle for 10 min	0, 10, 20	Observe child closely when on the steps
Levodopa	<15 kg: 125 mg 10-30 kg: 250 mg > 30 kg: 500 mg	0, 60, 90	Nausea, rarely emesis
Clonidine hypotension	0.15 mg/m2	0, 30, 60, 90	Tiredness, postural
Arginine HCI (IV)	0.5 g/kg (max 30 g) 10% arginine HCI in 0.9% NaCI over 30 min.	0, 15, 30, 45, 60
Insulin (IV)	0, 15, 30, 60, 75, 90, 120	0, 15, 30, 60, 75, 90, 120	Hypoglycemia, requires close supervision
Glucagon (IM)	0.03 mg/kg (max 1 mg)	0, 30, 60, 90, 120, 150, 180	Nausea, occasional emesis
GHRH (IV)	1 µg/kg	0, 15, 30, 45, 60, 90, 120	Flushing, metallic taste
GHD: Growth hormone deficiency; GHRH: Growth hormone-releasing hormone; IM: Intramuscular; IV: Intravenous

Table 7: Investigation of suspected disorder of GH-IGF-1 Axis

Last Updated on 15-08-2006

How to cite this url

Christmas Pedicon 2005 - Conference Abstracts. Pediatric Oncall [serial online] 2006 [cited 15 August 2006(Supplement 8)];3. Available from:
http://www.pediatriconcall.com/fordoctor/Conference_abstracts/
CHRISTMAS_PEDIACON/approach_short_stature.asp

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