获得性低促性腺激素性性腺功能减退症临床分析(附6例报告)

ACQUIRED HYPOGONADOTROPIC HYPOGONADISM:A CLINICAL ANALYSIS OF SIX CASES

目的探讨获得性低促性腺激素性性腺功能减退症(AHH)的病因、临床特点、检查方法及治疗措施等,提高对AHH的认识。方法回顾2010年1月—2019年7月我科确诊的6例AHH病人的临床资料并进行文献复习。结果6例病人病因:血色病1例,颅咽管瘤术后3例,生长激素瘤术后1例,无功能垂体大腺瘤术后1例。单纯性低促性腺激素性性腺功能减退症1例,5例合并继发性甲状腺功能减退症和继发性肾上腺皮质功能减退症,3例合并中枢性尿崩症。病程1~30年,平均10.2年。1例进行了肝穿刺活检和基因检测,5例于垂体和鞍区肿瘤术后取组织行病理学检查。6例均行颅脑MRI+增强检查及相关实验室检查。根据病史、临床表现、检验结果诊断为AHH。给予对因治疗,同时予以相应靶腺激素替代治疗,病情部分改善。结论AHH早期明确病因、早期治疗,可提高病人生活质量。

Objective To investigate the etiology,clinical features,examination methods,and treatment measures of acquired hypogonadotropic hypogonadism(AHH),and to improve the awareness of AHH.Methods A retrospective analysis was performed for the clinical data of six patients with AHH who were diagnosed in our department from January 2010 to July 2019,and a literature review was performed.Results Among the six patients,one had the etiology of hemochromatosis,three underwent the surgery for craniopharyngioma,one underwent the surgery for somatotropinoma,and one underwent the surgery for non-functional pituitary adenoma.Of all patients,one had simple hypogonadotropic hypogonadism,five had secondary hypothyroidism and secondary adrenocortical insufficiency,and three had central diabetes insipidus.The course of the disease ranged from 1 year to 30 years(mean 10.2 years).One patient underwent liver biopsy and gene detection,and five patients received pathological biopsy of the tissue collected from pituitary and sellar tumors.All six patients underwent craniocerebral MRI+contrast-enhanced examination and related laboratory tests,and the patients were diagnosed with AHH based on medical history,clinical manifestations,and examination results.All patients were given etiological treatment and hormone replacement therapy for corresponding target glands and then achieved a certain degree of improvement in disease conditions.Conclusion Early identification of etiology and early treatment of AHH can improve patients’quality of life.