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LINEAR GROWTH-RESPONSE TO EXOGENOUS GROWTH-HORMONE IN PRADER-WILLI SYNDROME AMERICAN JOURNAL OF MEDICAL GENETICS Lee, P. D., Wilson, D. M., ROUNTREE, L., Hintz, R. L., Rosenfeld, R. G. 1987; 28 (4): 865-871

Abstract

Linear growth retardation and adult short stature are usual characteristics of Prader-Willi syndrome. Several lines of evidence suggest that a deficiency in growth hormone (GH) secretion may contribute to this abnormal growth pattern. We have recently reported observations in 4 children with Prader-Willi syndrome treated with GH. This report extends our observations in 2 of these cases. Both cases had abnormally low growth rate, normal stimulated GH levels, and low somatomedin-C levels prior to therapy. GH treatment led to significant increases in linear growth rate and somatomedin-C levels. An additive effect of oxandrolone therapy on linear growth rate was demonstrated in one case. Our results support the possibility of a neuro-secretory GH deficiency in Prader-Willi syndrome and suggest a need for further investigations.

View details for Web of Science ID A1987K914200010

View details for PubMedID 3688024