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两个扩张型心肌病家族中心肌炎的临床与病理表现

Clinical and pathologic findings of myocarditis in two families with dilated cardiomyopathy.

作者信息

O'Connell J B, Fowles R E, Robinson J A, Subramanian R, Henkin R E, Gunnar R M

出版信息

Am Heart J. 1984 Jan;107(1):127-35. doi: 10.1016/0002-8703(84)90146-7.

Abstract

The use of endomyocardial biopsy and gallium-67 scans in patients with dilated cardiomyopathy (DCM) has demonstrated the presence of myocardial inflammation in a subset of patients. A family with DCM was studied with endomyocardial biopsy and gallium-67 scanning; both identified the presence of myocarditis in the proband. Evaluation of histologic sections from decreased family members revealed myocarditis as the principal pathologic finding. This patient identified during life demonstrated a defect in suppressor lymphocytic function and improved with immunosuppressive therapy. A second family with DCM was discovered when postmortem examination of the proband and his father's heart showed myocarditis. A living sibling was identified with asymptomatic myocardial dysfunction. Longitudinal follow-up of surviving members of both families are in progress. This study indicates that thorough diagnostic evaluation of all patients with familial DCM should be pursued to identify subgroups with potentially treatable inflammation.

摘要

在扩张型心肌病(DCM)患者中使用心内膜心肌活检和镓-67扫描已证实在一部分患者中存在心肌炎症。对一个患有DCM的家族进行了心内膜心肌活检和镓-67扫描研究;两者均在先证者中发现了心肌炎。对家族中患病成员的组织学切片评估显示,心肌炎是主要的病理发现。该生前确诊的患者表现出抑制性淋巴细胞功能缺陷,并通过免疫抑制治疗得到改善。当对先证者及其父亲的心脏进行尸检时发现了第二个患有DCM的家族。一名在世的同胞被确定存在无症状心肌功能障碍。两个家族存活成员的纵向随访正在进行中。这项研究表明,应对所有家族性DCM患者进行全面的诊断评估,以识别出具有潜在可治疗炎症的亚组。

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