Abstract
Five males and 2 females with the Prader-Willi Syndrome, aged 10-26 years, had low levels of circulating LH with a subnormal response to 100 and 500 mcg of gonadotrophin releasing hormone (Gn-RH) IV. Serum FSH response to Gn-RH was absent in 3, but normal in only one. Gonadotrophin response to Gn-RH increased in 4 patients during oral clomiphene (200 mg daily) given one to 6 weeks. This suggests that the pituitary gonadotrophes can function normally and that the deficiency is hypothalamic. Five males received 2000 u HCG twice weekly IM for up to 6 weeks. In 2 there was no response; in the remainder the rise in plasma testosterone was subnormal. Six of 7 patients had exaggerated TSH responses to 200 mcg thyrotrophin releasing hormone IV. All had normal thyroid function and serum prolactin and no evidence of thyroiditis. While gonadotrophin deficiency is present, primary gonadal failure may also be a feature of the hypogonadism.
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Jeffcoate, W., Laurance, B., Besser, G. et al. Endocrine Function in the Prader-Willi Syndrome. Pediatr Res 12, 158 (1978). https://doi.org/10.1203/00006450-197802000-00075
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DOI: https://doi.org/10.1203/00006450-197802000-00075