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三个罕见病例的故事:复发性涎腺炎所致继发性淀粉样蛋白A(AA)淀粉样变性,并伴有肺动脉高压和肾上腺功能不全。

A Tale of Three Rarities: Secondary Amyloid A (AA) Amyloidosis Caused by Recurrent Sialadenitis and Complicated by Pulmonary Hypertension and Adrenal Insufficiency.

作者信息

Desai Parth, Mbachi Chimezie, Joshi Udit, Mba Benjamin

机构信息

Internal Medicine, John H. Stroger, Jr. Hospital of Cook County, Chicago, USA.

Cardiology, OSF Healthcare, Peoria, USA.

出版信息

Cureus. 2020 Apr 23;12(4):e7792. doi: 10.7759/cureus.7792.

Abstract

A 48-year-old lady presented with a parotid mass found to be secondary to recurrent sialadenitis. She was also found to have microcytic anemia, renal dysfunction, an elevated gamma gap, and an isolated alkaline phosphatase elevation. Later, she developed altered mental status and shock, and was found to have adrenal insufficiency, pulmonary hypertension, and pulmonary nodules. A liver biopsy was consistent with amyloid deposition. The constellation of findings was consistent with systemic amyloid A (AA) amyloidosis secondary to recurrent sialadenitis with hepatic, renal, pulmonary, and adrenal involvement. The patient later passed away due to acute hypoxic respiratory failure. This case demonstrates rare sequelae of systemic AA amyloidosis of pulmonary hypertension and adrenal insufficiency.

摘要

一位48岁女性因腮腺肿块就诊,发现该肿块继发于复发性涎腺炎。还发现她患有小细胞性贫血、肾功能不全、γ-间隙升高以及孤立性碱性磷酸酶升高。后来,她出现精神状态改变和休克,被发现患有肾上腺功能不全、肺动脉高压和肺结节。肝脏活检结果与淀粉样蛋白沉积一致。这些发现符合继发于复发性涎腺炎且累及肝脏、肾脏、肺和肾上腺的系统性淀粉样蛋白A(AA)淀粉样变性。该患者后来因急性低氧性呼吸衰竭去世。此病例展示了系统性AA淀粉样变性导致肺动脉高压和肾上腺功能不全的罕见后遗症。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/728c/7243608/301b7ecebdd9/cureus-0012-00000007792-i01.jpg

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