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先天性肾上腺皮质增生症成年男性患者的睾丸肾上腺残余瘤:患病率及其对睾丸功能的影响

Testicular adrenal rest tumours in young adult males with congenital adrenal hyperplasia: prevalence and impact on testicular function.

作者信息

Chihaoui M, Kanoun F, Chaker F, Yazidi M, Bouchrit K, Mizouni H, Feki M, Kharrat M, Slimane H

机构信息

Department of Endocrinology, University Hospital La Rabta, Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia.

Department of Radiology, University Hospital La Rabta, Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia.

出版信息

Andrologia. 2016 Feb;48(1):45-50. doi: 10.1111/and.12416. Epub 2015 Apr 16.

DOI:10.1111/and.12416
PMID:25880899
Abstract

Testicular adrenal rest tumours (TARTs) have been described in patients with congenital adrenal hyperplasia (CAH). The aim of the study was to determine the prevalence of TARTs in patients with CAH, the associated factors and their impact on gonadal function. It is a prospective study concerning six young adult men with CAH, four cases with 21-hydroxylase deficiency and two cases with 11-hydroxylase deficiency. All patients were under glucocorticoid therapy. The mean age was 25 years (range: 20-31). All patients underwent a physical examination with testicular palpation, scrotal ultrasonography, a blood sample for serum testosterone, FSH, LH, inhibin B, ∆4-androstenedione and 17-OH-progesterone measurements and a semen analysis. Ultrasound revealed TARTs in four patients; three were bilateral. The mean tumour size was 6.3 ml (range: 0.02-14.1). The tumours were palpable in two cases. 17-OH-progesterone was <10 ng/ml in all cases. Decreased testosterone level was found in one case. The semen analysis revealed azoospermia in one case and poor semen quality in four patients. TARTs were common and associated with impaired spermatogenesis.

摘要

先天性肾上腺皮质增生症(CAH)患者中已发现睾丸肾上腺残余肿瘤(TARTs)。本研究旨在确定CAH患者中TARTs的患病率、相关因素及其对性腺功能的影响。这是一项针对6名患有CAH的年轻成年男性的前瞻性研究,其中4例为21-羟化酶缺乏症,2例为11-羟化酶缺乏症。所有患者均接受糖皮质激素治疗。平均年龄为25岁(范围:20 - 31岁)。所有患者均接受了体格检查,包括睾丸触诊、阴囊超声检查、采集血样检测血清睾酮、卵泡刺激素(FSH)、黄体生成素(LH)、抑制素B、Δ4-雄烯二酮和17-羟孕酮,并进行了精液分析。超声检查发现4例患者存在TARTs;3例为双侧。肿瘤平均大小为6.3毫升(范围:0.02 - 14.1)。2例可触及肿瘤。所有病例中17-羟孕酮均<10纳克/毫升。1例患者睾酮水平降低。精液分析显示1例无精子症,4例患者精液质量差。TARTs很常见且与精子发生受损有关。

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