【摘 要】
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Dear Editor,rnWe are doctors from Peking Union Medical College Hospital, Beijing, China. Male idiopathic hypogonadotropic hypogonadism (IHH) is a congenital disease that manifests as small testes, a short penis and no pubertal development. Some patients e
【机 构】
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Department of Endocrinology, Peking Union Medical College Hospital, Beijing 100730, China
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Dear Editor,rnWe are doctors from Peking Union Medical College Hospital, Beijing, China. Male idiopathic hypogonadotropic hypogonadism (IHH) is a congenital disease that manifests as small testes, a short penis and no pubertal development. Some patients exhibit cryptorchism and ambiguous genitalia [1]. IHH has been reported being caused by gene mutations in KAL-1, FGFR1, GnRHR,GPR54, and several other ligands and receptors [2].For these patients, gonadotropins or testosterone is the primary choice of therapy. However, adult-onset IHH,defined as idiopathic isolated hypogonadotropic hypogonadism, is very rare, occurring in sexually mature men who have experienced full pubertal development,normal sexual activity and spermatogenesis [3]. The possible aetiology, clinical manifestations, management and prognosis in this disease are quite different from those of congenital IHH.
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