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一名男婴因组织细胞样心肌病猝死:尸检病例及文献复习

Sudden Death in a Male Infant Due to Histiocytoid Cardiomyopathy: An Autopsy Case and Review of the Literature.

作者信息

Xie Hanbing, Chen Xueqin, Chen Ni, Zhou Qiao

机构信息

From the Department of Pathology and State Key Laboratory of Biotherapy, West China Hospital, Sichuan University, Chengdu, China.

出版信息

Am J Forensic Med Pathol. 2017 Mar;38(1):32-34. doi: 10.1097/PAF.0000000000000289.

Abstract

Histiocytoid cardiomyopathy (HC) is a very rare cardiac disorder that mainly affects female infants younger than 2 years. It may manifest as ventricular tachycardia or dilated cardiomyopathy and frequently causes sudden death. The most common grossly change is multifocal uneven thickening of the endocardium, presenting a yellowish color, with some area forming nodular appearance, and histologically featured by scattered clusters of histiocytoid myocytes under the endocardium. Here, we present a 19-month-old male infant who died of heart failure, and an autopsy was performed and confirmed the diagnosis of HC. We also reviewed the literature and focused on the research progression of the origin and genetic/molecular features of HC in recent years.

摘要

组织细胞样心肌病(HC)是一种非常罕见的心脏疾病,主要影响2岁以下的女婴。它可能表现为室性心动过速或扩张型心肌病,并常导致猝死。最常见的大体改变是心内膜多灶性不均匀增厚,呈淡黄色,有些区域形成结节状外观,组织学特征为心内膜下散在的组织细胞样心肌细胞簇。在此,我们报告一名19个月大死于心力衰竭的男婴,经尸检确诊为HC。我们还回顾了文献,并重点关注了近年来HC的起源及遗传/分子特征的研究进展。

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