Isolated Gonadotrope Failure in the Polyglandular Autoimmune Syndrome

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Abstract
Hypogonadism is a component of the polyglandular autoimmune syndromes and usually results from primary gonadal failure. Isolated gonadotropin deficiency is a disorder of prepubertal onset that usually results from a failure of hypothalamic secretion of gonadotropin-releasing hormone (GnRH). We describe here two men with polyglandular autoimmune syndrome and isolated gonadotropin deficiency acquired after puberty. Plasma levels of luteinizing hormone and follicle-stimulating hormone in response to bolus doses of GnRH and to pulsatile GnRH injections (25 ng per kilogram of body weight intravenously every two hours) over a four-day period were subnormal. Pituitary secretion of thyroid-stimulating hormone, prolactin, growth hormone, and ACTH was not impaired.