Adult-onset idiopathic hypogonadotropic hypogonadism:possible aetiology, clinical manifestations and management

• 来源期刊: 亚洲男性学杂志（英文版）
• 年，卷（期）: 2010,（4）
• 页码范围: 611-614
• 页数: 4页
• 语种: 英文
• DOI: 10.1038/aja.2010.34

摘要

Dear Editor, We 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.