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[婴儿心内膜弹力纤维增生症的诊断与治疗]

[Diagnosis and treatment of infantile endocardial fibroelastosis].

作者信息

Wang H

机构信息

Beijing Heart Lung and Blood Vessel Medical Center, Beijing Anzhen Hospital.

出版信息

Zhonghua Yi Xue Za Zhi. 1993 Jul;73(7):396-9, 446.

PMID:8293339
Abstract

The diagnosis, treatment and etiology of primary infantile endocardial fibroelastosis were studied in 33 patients from 1989 to 1992. Two dimensional echocardiography with Doppler examination showed that the thickness of the endocardium obviously increased and systolic and diastolic functions of the left ventricles were abnormal. Endomyocardial biopsy proved that the endocardium and myocardium under went pathological changes. The virus probe examination of biopsy tissue showed negative results and the effectiveness of immunosuppressive therapy (total effective rate 96%) indicated that this disease is probably of autoimmune etiology. The facts led us to nominate this disease as infantile endomyocardial disease. Besides, the classification, diagnostic criteria, principles of treatment and prognosis of this disease were discussed.

摘要

1989年至1992年对33例原发性婴儿心内膜弹力纤维增生症患者的诊断、治疗及病因进行了研究。二维超声心动图加多普勒检查显示心内膜厚度明显增加,左心室收缩和舒张功能异常。心内膜心肌活检证明心内膜和心肌发生了病理变化。活检组织的病毒探针检查结果为阴性,免疫抑制治疗的有效性(总有效率96%)表明本病可能为自身免疫性病因。这些事实使我们将本病命名为婴儿心内膜心肌疾病。此外,还讨论了本病的分类、诊断标准、治疗原则及预后。

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