Background Men with isolated gonadotropin-releasing hormone(GnRH) deficiency typically present with an absence of pubertaldevelopment. We describe an adult-onset form of idiopathic hypogonadotropichypogonadism that develops after puberty.
Methods We studied 10 men (age, 27 to 57 years) with normalsexual maturation, idiopathic infertility, sexual dysfunction,low serum testosterone concentrations, and apulsatile secretionof luteinizing hormone on frequent blood sampling. All the menhad otherwise normal anterior pituitary hormone secretion andsellar anatomy. We compared the results of semen analyses andmeasurements of testicular volume, serum testosterone, inhibinB, and gonadotropins in these men with the results in 24 menwith classic GnRH deficiency before and during GnRH-replacementtherapy and in 29 normal men of similar age.
Results Serum gonadotropin concentrations in the men with adult-onsetGnRH deficiency were similar before and during pulsatile GnRHadministration to those in the men with classic GnRH deficiency.However, as compared with men with classic GnRH deficiency,men with adult-onset hypogonadotropic hypogonadism had largermean (±SD) testicular volumes (18±5 vs. 3±2ml, P<0.001), serum testosterone concentrations (78±34vs. 49±20 ng per deciliter [2.7±1.2 vs. 1.7±0.7nmol per liter], P = 0.004), and serum inhibin B concentrations(119±52 vs. 60±21 pg per milliliter, P<0.001).Treatment with GnRH reversed the hypogonadism and restored fertilityin each of the five men who received long-term therapy.
Conclusions The recognition of adult-onset hypogonadotropichypogonadism in men as a distinct disorder expands the spectrumof GnRH deficiency and identifies a treatable form of male infertility.
Source Information
From the Reproductive Endocrine Unit and National Center for Infertility Research, Massachusetts General Hospital, Boston (L.B.N., P.A.B., W.F.C.), and the Division of Endocrinology, Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland (F.P.P.).
Address reprint requests to Dr. Crowley at the Reproductive Endocrine Unit, BHX-5, Massachusetts General Hospital, Fruit St., Boston, MA 02114.
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