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一名患有女性假两性畸形的成年患者的大结节性先天性肾上腺增生。

Macronodular congenital adrenal hyperplasia in an adult with female pseudohermaphroditism.

作者信息

Norris A M, O'Driscoll J B, Mamtora H

机构信息

Department of Diagnostic Radiology, Hope Hospital, Salford Royal NHS Trust, United Kingdom.

出版信息

Eur Radiol. 1996;6(4):470-2. doi: 10.1007/BF00182473.

Abstract

We report a case of previously undiagnosed congenital adrenal hyperplasia presenting with virilisation in a 59-year-old woman. Biochemical analysis revealed C-21 hydroxylase deficiency. CT demonstrated adrenal hyperplasia and a 3.8-cm adrenal nodule, raising the possibility of the development of an autonomous adrenal adenoma or carcinoma. The adrenal nodule regressed significantly with oral replacement steroid therapy over the next 30 months, indicating it to be an ACTH-dependent hyperplastic nodule and thus avoiding the need for biopsy or surgical excision. Macronodular adrenal hyperplasia should be considered in the differential diagnosis of a patient presenting with virilisation and an adrenal mass.

摘要

我们报告一例59岁女性因男性化表现而被诊断出患有此前未被诊断出的先天性肾上腺增生症。生化分析显示为21-羟化酶缺乏症。CT显示肾上腺增生以及一个3.8厘米的肾上腺结节,增加了自主性肾上腺腺瘤或癌发展的可能性。在接下来的30个月里,肾上腺结节通过口服替代类固醇疗法显著消退,表明其为促肾上腺皮质激素依赖性增生性结节,从而避免了活检或手术切除的必要性。在鉴别诊断出现男性化表现和肾上腺肿块的患者时,应考虑大结节性肾上腺增生。

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