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表现为淀粉样变性所致肾病综合征的高安氏病。

Takayasu's disease presenting as a nephrotic syndrome due to amyloidosis.

作者信息

Sousa A E, Lucas M, Tavora I, Victorino R M

机构信息

University Hospital of Santa Maria, Faculty of Medicine of Lisbon, Department of Medicine 2, Portugal.

出版信息

Postgrad Med J. 1993 Jun;69(812):488-9. doi: 10.1136/pgmj.69.812.488.

Abstract

We report an 18 year old black woman who presented with nephrotic syndrome in whom the investigations led to the diagnosis of diffuse Takayasu's disease, renal amyloidosis of AA type and interstitial lung disease. Proteinuria in Takayasu's disease is usually ascribed to hypertension or more rarely to glomerulonephritis. This case suggests that amyloidosis should be considered also in the investigation of proteinuria in these patients in view of the serious prognostic implications. This case represents further evidence that Takayasu's disease can be the cause of systemic reactive amyloidosis which may also be the presenting feature.

摘要

我们报告了一名18岁的黑人女性,她因肾病综合征就诊,检查后诊断为弥漫性大动脉炎、AA型肾淀粉样变性和间质性肺病。大动脉炎中的蛋白尿通常归因于高血压,或更罕见地归因于肾小球肾炎。鉴于其严重的预后影响,该病例提示在这些患者蛋白尿的调查中也应考虑淀粉样变性。该病例进一步证明大动脉炎可能是系统性反应性淀粉样变性的病因,而系统性反应性淀粉样变性也可能是首发特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/39f2/2399860/634d94d89a3f/postmedj00054-0074-a.jpg

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