FAMILIAL HYPOGONADOTROPIC EUNUCHOIDISM

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Abstract
An isolated deficiency of pituitary gonadotropin is rare. Familial occurrence of this form of hypogonadism is recorded infrequently, and only in male siblings. Since the majority of reported cases of familial eunuchoidism were studied before the clinical use of gonadotropin assays, it is not known whether the defect lay in the pituitary or the gonads of the patients. This report is concerned with 5 instances of hypogonadotropic eunuchoidism in 2 families: 3 male siblings in 1 family and a brother and sister in another. The affected siblings in both families had normal brothers and sisters. Growth, thyroid function and adrenal cortical function were normal in all 5 cases. All 5 patients were sexually underdeveloped, had eunuchoid proportions and lacked urinary gonadotropins. Satisfactory genital development was maintained in these patients by "end-organ therapy," i.e., substitution of gonadal rather than gonadotropic hormones. Orally or bucally given sex hormones are therapeutically effective, easy to give and economical.
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