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一名未确诊非经典型先天性肾上腺皮质增生症的女性患者双侧肾上腺髓质脂肪瘤

Bilateral Adrenal Myelolipomas in a Female Patient With Undiagnosed Non-classic Congenital Adrenal Hyperplasia.

作者信息

Vemula Bhavana R, Olajide Omolola B, Adepoju Yewande

机构信息

Endocrinology, Diabetes, and Metabolism, Marshall University Joan C. Edwards School of Medicine, Huntington, USA.

Endocrinology, Diabetes, and Metabolism, Oroville Hospital, Oroville, USA.

出版信息

Cureus. 2023 Feb 15;15(2):e35017. doi: 10.7759/cureus.35017. eCollection 2023 Feb.

Abstract

Non-classic congenital adrenal hyperplasia (CAH) usually presents later in life with signs of androgen excess but may also be diagnosed after the detection of an incidental adrenal myelolipoma. This is a patient with previously undiagnosed CAH who presented to the emergency department with chest discomfort and palpitations. A computed tomography (CT) scan of the chest done to rule out pulmonary embolism showed bilateral large adrenal myelolipomas. She also had evidence of marked hirsutism on examination, which prompted further workup, and her laboratory data was in keeping with CAH. Further management was unable to be pursued due to the patient's poor compliance, and she was subsequently lost to follow-up. Chronic exposure of the adrenal glands to high adrenocorticotropic hormone (ACTH) levels increases the risk of developing myelolipomas. CAH needs to be considered as a diagnosis in the evaluation of incidental adrenal myelolipomas.

摘要

非经典型先天性肾上腺皮质增生症(CAH)通常在生命后期出现雄激素过多的体征,但也可能在偶然发现肾上腺髓质脂肪瘤后被诊断出来。这是一名先前未被诊断出患有CAH的患者,因胸部不适和心悸到急诊科就诊。为排除肺栓塞而进行的胸部计算机断层扫描(CT)显示双侧肾上腺有大的髓质脂肪瘤。检查还发现她有明显多毛症的迹象,这促使进一步检查,其实验室数据与CAH相符。由于患者依从性差,无法进行进一步治疗,随后失访。肾上腺长期暴露于高促肾上腺皮质激素(ACTH)水平会增加发生髓质脂肪瘤的风险。在评估偶然发现的肾上腺髓质脂肪瘤时,需要考虑CAH这一诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/908b/10022842/26ae474751d5/cureus-0015-00000035017-i01.jpg

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