Studies of Endocrine Function in “Isolated” Gonadotropin Deficiency

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Abstract
Six chromatin-negative young men, ages 16–24 yr, with hypogonadotropic hypogonadism were studied in an attempt to clarify and define the endocrine abnormalities responsible for this disorder. Five of the six patients had associated congenital anomalies involving the skeletal system or midline facial structures. Pituitary function studies showed normal thyroid and adrenal function. Two of 6 patients showed subnormal growth hormone responses to arginine infusion and insulin hypoglycemia, and 4 tested showed subnormal sleep mediated growth hormone release. Evaluation of pituitary-gonadal function showed normal prepubertal concentrations of luteinizing hormone (LH), follicle-stimulating hormone (FSH) and testosterone. The plasma testosterone response to acute stimulation with human chorionic gonadotropin (hCG) (5000 units daily × 4 days) was subnormal in 3 of 6 patients; however, these 3 patients achieved normal plasma testosterone concentrations after more prolonged hCG administration (5000 units twice weekly for 4–8 weeks). These observations show that hypogonadotropic hypogonadism is associated with a high frequency of congenital abnormalities, subnormal sleep-mediated growth hormone release, plasma LH and FSH concentrations indistinguishable from normal prepubertal boys and normal plasma testosterone responses to long term hCG treatment.

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