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Churg-Strauss syndrome in children: a case evolving in endomyocardiopathy

Authors:LU Shan, ZHOU Wen-Yan, ZHOU Wei, LI Zhao-Ping, CHANG Yan-Mei, WEI Ling, TONG Xiao-Mei.

Department of Pediatrics, Peking University Third Hospital, Beijing 100083, China. lucm@ccps.gov.cn

 

Abstract: A 13-year-old girl presented with a 1-month history of progressive exertional dyspnea (NYHA class ) and exophthalmos for 6 months. She had a history of long-standing asthma and the presence of allergy. Hypereosinophilia and increased serum IgE levels (2 472 IU/mL) were observed. Chest radiography and a high resolution CT scan documented a massive interstitial pulmonary infiltration. Echocardiography confirmed mild tricuspid regurgitation, apical obliteration of the right ventricle by fibrocalcific thickening of the endocardium and echogenic material suggestive of thrombosis. Churg-Strauss syndrome with cardiac involvement (endomyocardiopathy) was diagnosed. The patient received anticoagulation and corticosteroid therapy. In view of rapidly prog-ressive severe endomyocardiopathy and stable hematology, the patient was referred for cardiac surgery. Histopathological examination of resected specimens confirmed laminated thrombus but without any trace of eosinophils embedded.Chin J Contemp Pediatr, 2008, 10 (5):625-628

 

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