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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.

DOI:10.1007/s00120-008-1857-4
PMID:18762902
Abstract

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.

摘要

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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引用本文的文献

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[Testicular adrenal rest tumors in adolescents].青少年睾丸肾上腺残余肿瘤
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Testicular adrenal rest tissue in a patient with classical congenital adrenal hyperplasia: color Doppler findings.经典型先天性肾上腺皮质增生症患者的睾丸肾上腺残余组织:彩色多普勒表现
J Ultrasound. 2013 May 7;16(2):81-4. doi: 10.1007/s40477-013-0015-z. eCollection 2013.
3
[Congenital adrenal hyperplasia and growth hormone deficiency. Special care in transition to adulthood].[先天性肾上腺皮质增生症与生长激素缺乏症。成年期过渡的特殊护理]

本文引用的文献

1
Classical forms of congenital adrenal hyperplasia due to 21-hydroxylase deficiency in adults.成人因21-羟化酶缺乏所致先天性肾上腺皮质增生症的经典类型。
Horm Res. 2008;69(4):203-11. doi: 10.1159/000113020. Epub 2008 Jan 21.
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Adult consequences of congenital adrenal hyperplasia.先天性肾上腺皮质增生症的成人后果。
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Congenital adrenal hyperplasia: adolescence and transition.先天性肾上腺增生症:青春期与过渡阶段
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Long-term followup after elective testis sparing surgery for Leydig cell tumors: a single center experience.对睾丸间质细胞瘤行择期保留睾丸手术后的长期随访:单中心经验
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Testicular tumors in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency show functional features of adrenocortical tissue.因21-羟化酶缺乏所致先天性肾上腺皮质增生症患者的睾丸肿瘤表现出肾上腺皮质组织的功能特征。
J Clin Endocrinol Metab. 2007 Sep;92(9):3674-80. doi: 10.1210/jc.2007-0337. Epub 2007 Jun 26.
6
Repeated successful induction of fertility after replacing hydrocortisone with dexamethasone in a patient with congenital adrenal hyperplasia and testicular adrenal rest tumors.在一名患有先天性肾上腺皮质增生症和睾丸肾上腺残余肿瘤的患者中,用 dexamethasone 替代 hydrocortisone 后反复成功诱导生育。
Fertil Steril. 2007 Sep;88(3):705.e5-8. doi: 10.1016/j.fertnstert.2006.11.148. Epub 2007 May 22.
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Long-term outcome of patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.21-羟化酶缺乏所致先天性肾上腺皮质增生症患者的长期预后。
Horm Res. 2007;67(6):268-76. doi: 10.1159/000098017. Epub 2006 Dec 14.
8
Testicular adrenal rests in a patient with congenital adrenal hyperplasia: US and MRI features.先天性肾上腺皮质增生症患者的睾丸肾上腺残余组织:超声和磁共振成像特征
Comput Med Imaging Graph. 2006 Dec;30(8):465-8. doi: 10.1016/j.compmedimag.2006.09.012. Epub 2006 Oct 16.
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Extensive clinical experience: nonclassical 21-hydroxylase deficiency.丰富的临床经验:非经典型21-羟化酶缺乏症
J Clin Endocrinol Metab. 2006 Nov;91(11):4205-14. doi: 10.1210/jc.2006-1645. Epub 2006 Aug 15.
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Microsurgical "testis-sparing" surgery for nonpalpable hypoechoic testicular lesions.针对不可触及的低回声睾丸病变的显微外科“保留睾丸”手术。
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