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.
Zhongguo Dang Dai Er Ke Za Zhi. 2008 Oct;10(5):625-8.
A 13-year-old girl presented with a 1-month history of progressive exertional dyspnea (NYHA class IV) and exophthalmos for 6 months. She had a history of long-standing asthma and the presence of allergy. Hypereosinophilia and increased serum IgE levels (2472 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 progressive 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.
一名13岁女孩，有1个月进行性劳力性呼吸困难病史（纽约心脏协会IV级），突眼6个月。她有长期哮喘病史且存在过敏。观察到嗜酸性粒细胞增多和血清IgE水平升高（2472 IU/mL）。胸部X线摄影和高分辨率CT扫描显示大量肺间质浸润。超声心动图证实有轻度三尖瓣反流，右心室心尖因心内膜纤维钙化增厚和提示血栓形成的回声物质而闭塞。诊断为伴有心脏受累（心肌内膜炎）的变应性肉芽肿性血管炎。患者接受了抗凝和皮质类固醇治疗。鉴于严重的心肌内膜炎迅速进展且血液学指标稳定，患者被转诊进行心脏手术。切除标本的组织病理学检查证实有层状血栓，但未发现任何嵌入的嗜酸性粒细胞痕迹。
