ABSTRACT
OBJECTIVE: Growth hormone through insulin-like growth factor 1 (IGF-1) plays an important role in both bone growth and mineralization. This cross-sectional study was carried out to evaluate the relationship between serum IGF-1 concentrations and dual energy X-ray (DXA) measured whole body less head bone area (BA), lean body mass (LBM), and bone mineral content (BMC). METHODS: One hundred and nineteen children (boys = 70, age = 7.3-15.6 years) were studied for their anthropometric parameters by standard methods and bone and body composition by DXA. Their fasting serum IGF-1 concentrations were assessed by enzyme-linked immunosorbent assay and Z-scores were calculated using available reference data. Bone and body composition parameter Z-scores were calculated using ethnic reference data. RESULTS: Mean age of the boys and girls was similar (11.5 ± 1.8 years). The mean serum IGF-1concentrations and IGF-1 Z-scores were similar (P > 0.1) between boys and girls and were of the order of (302.3 ± 140.0 and - 1.4 ± 1.1, respectively). The LBM for age and BA for age Z-score was greater in children with IGF-1 Z-score > median than children with IGF-1 Z-score < median. The mean BMC for age Z-scores were 0.4 ± 0.9 and - 0.2 ± 0.8 in children with above and below the median of IGF-1 Z-score (P > 0.1). CONCLUSION: Serum IGF-1 levels were more strongly associated with BA and LBM, suggesting that its effect on bone is greater with respect to periosteal bone acquisition and through its effect on muscle mass.
ABSTRACT
INTRODUCTION: Patients with growth hormone (GH) insensitivity (also known as Laron syndome) have been reported from the Mediterranean region and Southern Eucador, with few case reports from India. We present here the clinical and endocrine profile of 9 children with Laron syndrome from India. MATERIAL AND METHODS: Nine children diagnosed with Laron syndrome based on clinical features of GH deficiency and biochemical profile suggestive of GH resistance were studied over a period of 5 years from January 2008 to January 2013. RESULTS AND DISCUSSION: Age of presentation was between 2.5-11.5 years. All children were considerably short on contemporary Indian charts with mean (SD) height Z score -5.2 (1.6). However, they were within ± 2 SD on Laron charts. No child was overweight [mean (SD) BMI Z score 0.92 (1.1)]. All children had characteristic facies of GH deficiency with an added feature of prominent eyes. Three boys had micropenis and 1 had unilateral undescended testis. All children had low IGF-1 (<5 percentile) and IGFP-3 (<0.1 percentile) with high basal and stimulated GH [Basal GH mean (SD) = 13.78 (12.75) ng/ml, 1-h stimulated GH mean (SD) = 46.29 (25.68) ng/ml]. All children showed poor response to IGF generation test. CONCLUSION: Laron syndrome should be suspected in children with clinical features of GH deficiency, high GH levels and low IGF-1/IGFBP-3. These children are in a state of GH resistance and need IGF-1 therapy.
ABSTRACT
Disorders of sexual development (DSD) are a complex group of disorders to manage, not only because the diagnosis is difficult, but also because it involves many factors related to the psychosexual well being, the social stigma associated with the disorder, confusion and reluctance on the part of medical professionals in dealing with this issue, catastrophic experience of parents of these children and difficult interpersonal relationships between parents and children born with this disorder. This brief review attempts to throw light on the recent advances in understanding and management of these disorders to help improve immediate and long term care of these patients and their families.
