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一名21岁女孩患有需要血液透析的新月体性肾小球肾炎、MPO-ANCA水平升高及变应性皮肤血管炎。

Crescentic glomerulonephritis requiring hemodialysis and elevated MPO-ANCA level and vasculitis allergica cutis in a 21-year-old girl.

作者信息

Kara Ismail H, Yilmaz M Emin, Sari Yasin, Gür Ali, Büyükbayram Hüseyin

机构信息

Department of Family Practice, Dicle University Medical School, Diyarbakir, Turkey.

出版信息

MedGenMed. 2002 May 2;4(2):2.

Abstract

Microscopic polyangiitis (mPA) is a systemic necrotizing nongranulomatous vasculitis that affects small blood vessels. Clinical features include constitutional symptoms such as fever, anorexia, fatigue, and weight loss. Skin lesions include purpura and splinter hemorrhages, which occur in 50% of patients. Another characteristic feature is rapidly progressive glomerulonephritis, which often affects the kidneys in the early stages of the condition. Diagnosis is based on typical clinical features, biopsy, and presence of antineutrophil cytoplasmic antibodies (ANCA). This disorder tends to involve middle-aged and older persons, with a predilection for males; it is very rare in children. A 21-year-old female patient with mPA who did not respond well to treatment, required hemodialysis, developed vasculitis allergica cutis (VAC) later, and had a relatively short-term survival is reported herein.

摘要

显微镜下多血管炎(mPA)是一种影响小血管的系统性坏死性非肉芽肿性血管炎。临床特征包括发热、厌食、疲劳和体重减轻等全身症状。皮肤病变包括紫癜和甲下出血,见于50%的患者。另一个特征性表现是快速进行性肾小球肾炎,常在疾病早期累及肾脏。诊断基于典型的临床特征、活检以及抗中性粒细胞胞浆抗体(ANCA)的存在。这种疾病倾向于累及中老年人,男性更易受累;在儿童中非常罕见。本文报告了一名21岁患有显微镜下多血管炎的女性患者,对治疗反应不佳,需要血液透析,后来发生了变应性皮肤血管炎(VAC),且生存时间相对较短。

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