成人发生的特发性低促性腺激素性性腺功能减退症临床特点分析  

Diagnosis and management of adult-onset idiopathic hypogonadotropic hypogonadism

作　　者：茅江峰[1] 王曦[1] 于冰青 高寅洁 聂敏[1] 伍学焱[1] Mao Jiangfeng;Wang Xi;Yu Bingqing;Gao Yinjie;Nie Min;Wu Xueyan(Department of Endocrinology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences ＆ Peking Union Medical College, Key Laboratory of Endocrinology, National Health and Family Planning Commission, Beijing 100730, China)

机构地区：[1]北京协和医学院中国医学科学院北京协和医院内分泌科国家卫生和计划生育委员会内分泌重点实验室,100730

出　　处：《中华医学杂志》2018年第20期1597-1600,共4页National Medical Journal of China

基　　金：国家重点研发项目（2016YFC0905102,2016YFA0101003）;国家自然科学基金（81771576）;中国医学科学院医学与健康科技创新工程（2016-12M-1-002,2017-I2M-3-007）

摘　　要：目的增加对成人发生的特发性低促性腺激素性性腺功能减退症（AIHH）诊断和治疗的认识。方法对2010年1月至2017年6月北京协和医院内分泌科门诊诊治的6例AIHH患者的临床特点和治疗效果进行回顾性分析。结果6例患者均为男性，年龄26（20—35）岁，以“性欲下降、勃起障碍和乳房发育”为主要临床表现。患者均经历完整的青春发育。查体示睾丸体积15（12～20）ml，第二性征完全发育。基础状态黄体生成素（LH）0．1（0．1～0．8）U／L，卵泡刺激素（FSH）0．4（0．1～0．9）U／L，睾酮0．62（0．10—0．90）nmol／L，垂体磁共振成像（MRI）和其他垂体前叶激素均正常。3例患者接受睾酮替代治疗，性欲和性生活恢复正常。2例患者在人绒毛膜促性腺激素／人绝经期促性腺激素（HCG／HMG）治疗4—6个月后产生大量精子。1例患者在随访6个月后，性腺轴功能恢复正常。结论AIHH病因不明。和先天性IHH相比，AIHH患者的生精治疗效果更好。极少患者性腺轴功能有发生逆转可能。Objective To investigate the clinical features and management of male patients with adult-onset idiopathic hypogonadotropic hypogonadism （AIHH）. Methods Clinical features and treatment of six patients with AIHH between January 2010 and June 2017 were retrospectively reviewed. Results The patients were all male, with an age of 26 （20 -35 ） years old and they experienced complete pubertal development. The main complaints were decreased libido, erectile dysfunction and gynecomastia. Physical examination found that the testicular size was 15 （12 -20） ml and they were fully virilized. The serum luteinizing hormone （LH）, follicle-stimulating hormone （FSH） and total testosterone was 0. 1 （0. 1 -0. 8 ） U/L, 0. 4 （0. 1 - 0. 9 ） U/L and 0. 62 （ 0. 10 - 0. 90 ） nmol/L, respectively. Pituitary MRI and other pituitary hormones were all normal. Testosterone was administrated to three patients and the libido and erectile function returned to normal. Sperm was successfully induced in two patients after combined gonadotropin therapy for 4 - 6 months. One patient had a reversed hypothalamus-pituitary-testis axis function. Conclusions The mechanism of male AIHH is unknown. Compared to those with congenital hypogonadotropic hypogonadism, patients with AIHH may achieve better spermatogenesis after gonadotropin therapy. Small portion of patients may have a reversal of hypothalarnus-pituitary-testis axis function.

关 键 词：性腺功能减退症 诊断 治疗 

分 类 号：R588[医药卫生—内分泌]