Response of Indian growth hormone deficient children to growth hormone therapy: association with pituitary size.

OBJECTIVE: To ascertain the impact of pituitary size as judged by Magnetic Resonance Imaging (MRI), on response to Growth Hormone (GH) therapy in GH deficient children. METHODS: Thirty nine children (9.1 ± 2.7 y, 22 boys) with non-acquired GH deficiency (21 Isolated GH deficiency and 18 Combined pituitary hormone deficiency) were consecutively recruited and followed up for one year. Clinical, radiological (bone age and MRI) and biochemical parameters were studied. RESULTS: Children with hypoplastic pituitary (pituitary height < 3 mm) had more severe height deficit (height for age Z-score -6.0 vs. -5.0) and retardation of skeletal maturation (bone age chronological age ratio of 0.59 vs. 0.48) at baseline as compared to children with normal pituitary heights (p < 0.05 for both). After one year of GH therapy, height for age Z scores and percentage change in height for age Z scores were significantly higher in children with hypoplastic pituitaries (13.8 ± 3.6 and 28.7 % vs. 11.2 ± 4.1 and 21.4 %). Significant co-relation was observed between pituitary height and height for age Z-scores at baseline (r = 0.39, p < 0.05). The predicted adult height using Tanner Whitehouse-2 equations improved from 140.8 to 152.3 cm in children with hypoplastic pituitary when compared to an increase from 145.8 to 153.5 cm observed in children with normal pituitary height (p < 0.05). CONCLUSIONS: Indian growth hormone deficient children with hypoplastic pituitary respond better to therapy with GH in short term.

Performance of WHO growth standards on Indian children with growth related disorders.

OBJECTIVE: To assess performance of WHO 2006 standards on anthropometric measurements of children referred for growth related disorders to a speciality pediatric clinic in Pune, India, from June 2006 through June 2010. METHODS: Data presented in this study were collected retrospectively from case records of all children from birth to 60 mo (n=1840, mean age 2.7±1.3 y) who presented with growth related disorders; healthy age and sex matched children were recruited as controls (n=824, mean age 2.8±1.2 y). Children were divided as per their clinical diagnosis into eight different groups: growth hormone deficiency, bone disorders, syndromic short stature, familial short stature, hypothyroidism, nutritional and systemic disorder, other endocrinopathies and overgrowth disorders. Anthropometric parameters for all study subjects were converted to standard deviation scores (SD scores) using the WHO Anthro 2005. RESULTS: Mean height SD scores of children with growth related disorders were significantly lower than that of the controls, while that of the tall children were significantly higher (p<0.05). All children who were clinically very short were below the 1st percentile, while none of the children with overgrowth or normal children were classified as stunted. Weight for height SD scores of children with nutritional and systemic disorders were the lowest, while those for the obese children were the highest. CONCLUSIONS: The present results suggest that the WHO 2006 growth standards classify children with growth disorders appropriately and the classification is in concordance with the clinical assessment. They provide health practitioners in a clinical setting with an effective tool to assess growth of children.

Evaluation of insulin-like growth factor-1 in Indian growth hormone-deficient children on growth hormone therapy.

OBJECTIVE: To evaluate the impact of recombinant human growth hormone therapy (GHT) on serum insulin-like growth factor 1 (IGF-1) concentrations in Indian children with growth hormone deficiency. METHODS: Data on anthropometry and serum IGF-1 concentrations were collected from 28 growth hormone-deficient prepubertal children (8.6 ± 2.9 years) on growth hormone therapy, 6 monthly over the period of 2 years. RESULTS: Height z-scores showed a steady increase from baseline to 24 months. However, IGF-1 z-scores showed a plateau after the first 6 months and then a small dip followed by a rise. The pattern of increase of IGF-1 z-scores was dissimilar to that of the height z-scores. CONCLUSION: The change in serum IGF-1 z-scores in response to GHT in Indian children may not be a good indicator for monitoring growth hormone responsiveness.

Macroorchidism in an Indian boy with McCune-Albright syndrome.

McCune-Albright syndrome (MAS) is defined by the clinical triad of fibrous dysplasia of bone (FD), café-au-lait spots and precocious puberty (PP). It is a rare disease with estimated prevalence between 1/100,000 and 1/1,000,000. The authors report a case of MAS in an Indian boy who had history of unilateral macroorchidism (but no other signs of MAS) since birth, then presented with PP, FD and café-au-lait spots at 6 years of age. On examination he had asymmetry of the face, café-au-lait spots and signs of sexual precocity with a right testicular volume of 25 ml (left 8 ml). Investigations revealed suppressed gonadotropins with elevated testosterone levels. Skeletal survey showed dysplastic changes involving multiple bones and advanced bone age. Reports of MAS in a boy with unilateral macroorchidism are scarce. Thus, unilateral macroorchidism at birth in a baby may be a feature of MAS and should be followed up with care.