Isolated Gonadotropin Deficiency

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Abstract
Seven female patients with primary amenorrhea were shown to have isolated gonadotropin deficiency. Thyroid and adrenal function and growth hormone secretion were normal. Basal levels of follicle-stimulating hormone, luteinizing hormone and estradiol 17β were below the limit of sensitivity of our assays, and there was no change after intravenous estrogen or oral clomiphene citrate. With luteinizing-hormone-releasing hormone, levels of both follicle-stimulating and luteinizing hormones rose in five subjects, of luteinizing hormone only increased in one subject, and in the remaining patient there was no change in either hormone. In all three patients to whom urinary gonadotropins (menotropins) were administered serum estradiol 17β was elevated. Subsequent therapy with human chorionic gonadotropin led to ovulation, with an increase in serum progesterone, and two patients became pregnant.