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儿童变应性肉芽肿性血管炎:临床与病理综述

Churg-Strauss syndrome in children: a clinical and pathologic review.

作者信息

Boyer Debra, Vargas Sara O, Slattery Dubhfeasa, Rivera-Sanchez Yadira M, Colin Andrew A

机构信息

Department of Medicine, Division of Respiratory Diseases, Children's Hospital Boston, Harvard Medical School, Boston, Massachusetts 02155, USA.

出版信息

Pediatrics. 2006 Sep;118(3):e914-20. doi: 10.1542/peds.2006-0113. Epub 2006 Aug 7.

Abstract

Churg-Strauss syndrome is a vasculitis accompanied by asthma and eosinophilia. It is generally considered a disease of adults; occurrence in children has been reported infrequently. Here we report 2 pediatric patients with Churg-Strauss syndrome manifesting with prominent pulmonary involvement. One, a 16-year-old with a previous history of asthma, presented with pleuritic chest pain and a peripheral pulmonary nodule complicated by an eosinophilic pleural effusion. The other patient presented at age 6 with cough, weight loss, and radiographic infiltrates. Lung biopsies revealed elements characteristic of Churg-Strauss syndrome, including eosinophilic microabscesses and vasculitis. Three- and 5-year follow-up showed continued symptoms in both patients despite medical therapy. Both patients illustrate many of the typical features of Churg-Strauss syndrome. We report these cases to expand the scant knowledge about Churg-Strauss syndrome in pediatric patients and to heighten awareness that this serious disease may affect the pediatric population. The relevant literature on Churg-Strauss syndrome, with specific reference to childhood cases, is reviewed.

摘要

变应性肉芽肿性血管炎是一种伴有哮喘和嗜酸性粒细胞增多的血管炎。它通常被认为是一种成人疾病;儿童发病的报道很少。在此,我们报告2例以显著肺部受累为表现的儿童变应性肉芽肿性血管炎患者。其中1例为16岁,既往有哮喘病史,表现为胸膜炎性胸痛和外周肺结节,并伴有嗜酸性胸腔积液。另一例患者6岁时出现咳嗽、体重减轻和影像学浸润。肺活检显示有变应性肉芽肿性血管炎的特征性表现,包括嗜酸性微脓肿和血管炎。3年和5年的随访显示,尽管进行了药物治疗,两名患者仍有持续症状。两名患者均表现出变应性肉芽肿性血管炎的许多典型特征。我们报告这些病例是为了扩展关于儿童变应性肉芽肿性血管炎的稀少知识,并提高对这种严重疾病可能影响儿童人群的认识。本文回顾了变应性肉芽肿性血管炎的相关文献,特别提及了儿童病例。

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