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[一例双侧睾丸肿瘤合并先天性肾上腺皮质增生症]

[A case of bilateral testicular tumors with congenital adrenal hyperplasia].

作者信息

Tsurukawa H, Iuchi H, Tokumitsu M, Yamaguchi S, Hashimoto H, Yachiku S

机构信息

Department of Urology, Wakkanai City General Hospital.

出版信息

Hinyokika Kiyo. 2000 May;46(5):311-3.

Abstract

We report a case of congenital adrenal hyperplasia (CAH) occurring in a 21-year-old man. He was found to have 21-hydroxylase deficiency shortly after birth in search for the cause of vomiting and adrenal insufficiency, and placed on steroid therapy. He had an uneventful childhood with normal onset of puberty. At the age of 21 years he was hospitalized with bilateral testicular masses. They were non-tender, firm and nodular on palpation. The levels of adrenocorticotrophic hormone (ACTH), 17 alpha-hydroxyprogesterone (17 alpha-OHP) were found to be elevated. Testicular biopsy revealed that the nodule comprised mainly eosinophilic sheets and nests and polygonal cells with abundant, granular cytoplasm, but no crystals of Reinke were seen. Testicular tumor with congenital adrenal hyperplasia is typically bilateral and develops in untreated or inadequately treated males with CAH.

摘要

我们报告一例发生在一名21岁男性身上的先天性肾上腺皮质增生症(CAH)。他在出生后不久因呕吐和肾上腺功能不全的病因检查被发现患有21-羟化酶缺乏症,并接受了类固醇治疗。他童年时期平安无事,青春期正常开始。21岁时,他因双侧睾丸肿块住院。触诊时肿块无压痛、质地硬且呈结节状。发现促肾上腺皮质激素(ACTH)、17α-羟孕酮(17α-OHP)水平升高。睾丸活检显示,结节主要由嗜酸性片状和巢状结构以及具有丰富颗粒状细胞质的多边形细胞组成,但未见莱因克晶体。先天性肾上腺皮质增生症伴发的睾丸肿瘤通常为双侧性,发生于未经治疗或治疗不充分的CAH男性患者。

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