成年男性经典型先天性肾上腺皮质增生症（CAH）中的睾丸肾上腺残余肿瘤（TART）
[Testicular adrenal rest tumors (TART) in adult men with classic congenital adrenal hyperplasia (CAH)].
作者信息
Knape P, Reisch N, Dörr H-G, Reincke M, Lenk S, Quinkler M
机构信息
Klinische Endokrinologie, Charité-Universitätsmedizin Berlin, Campus Mitte, Charitéplatz 1, 10117 Berlin.
出版信息
Urologe A. 2008 Dec;47(12):1596-7, 1599-602. doi: 10.1007/s00120-008-1857-4.
BACKGROUND
Information about treatment of adult male patients with congenital adrenal hyperplasia (CAH) and testicular adrenal rest tumors (TART) is scarce. Diagnostic and therapeutic guidelines do not exist. The aim of this review is to evaluate the current state of therapeutic options in adult male patients with CAH.
METHODS
We performed an extensive search of the literature of the last 10 years by using PubMed/MEDLINE.
RESULTS
The aims of treatment in adult male patients with CAH are prevention of adrenal crisis and TART, improvement of general well-being, good quality of life and sexual well-being, fertility, and prevention of side effects of gluco- and mineralocorticoid therapy. However, fertility is impaired in these patients and correlates with TART. The current therapeutic concepts are discussed.
CONCLUSIONS
A future system of regular follow-up visits and standards in therapeutic concepts is needed to guarantee an improved fertility and lifelong good quality of life in adult male patients with CAH.
背景
关于成年男性先天性肾上腺皮质增生症（CAH）和睾丸肾上腺残余肿瘤（TART）治疗的信息匮乏。目前尚无诊断和治疗指南。本综述的目的是评估成年男性CAH患者治疗选择的现状。
方法
我们使用PubMed/MEDLINE对过去10年的文献进行了广泛检索。
结果
成年男性CAH患者的治疗目标包括预防肾上腺危象和TART、改善总体健康状况、提高生活质量和性健康、生育能力以及预防糖皮质激素和盐皮质激素治疗的副作用。然而，这些患者的生育能力受损，且与TART相关。本文讨论了当前的治疗理念。
结论
需要建立一个定期随访的未来体系和治疗理念标准，以确保成年男性CAH患者的生育能力得到改善，并享有终身良好的生活质量。
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