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心内膜心肌纤维化:手术切除的心室心内膜的病理及分子学发现

Endomyocardial fibrosis: pathological and molecular findings of surgically resected ventricular endomyocardium.

作者信息

Iglezias Silvia D'Andretta, Benvenuti Luiz Alberto, Calabrese Fiorella, Salemi Vera Maria Cury, Silva Ana Maria Gonçalves, Carturan Elisa, de Oliveira Sergio Almeida, Thiene Gaetano, De Brito Thales

机构信息

Department of Pathology, University of São Paulo Medical School, São Paulo, Brazil.

出版信息

Virchows Arch. 2008 Sep;453(3):233-41. doi: 10.1007/s00428-008-0652-3. Epub 2008 Sep 2.

Abstract

Endomyocardial fibrosis (EMF) is a restrictive cardiomyopathy of unknown etiology prevalent in tropical regions affecting the inflow tract and apex of one or both ventricles, which show fibrous thickening of the endocardium and adjacent myocardium. Surgical treatment is recommended for patients in functional classes III or IV (New York Heart Association). The gross and histological features of the heart have been comprehensively studied in autopsies, but studies in surgical samples are still lacking. Histological and immunohistochemical features of EMF in surgical samples collected from 32 patients were described and correlated with clinical data. Polymerase chain reaction (PCR) and reverse transcription-PCR, performed on formalin fixed endomyocardial samples, were used retrospectively to detect genomes of certain cardiotropic viruses and Toxoplasma gondii. Ventricular endocardium was thickened by superficial acellular hyaline collagen fibers type I and III, with predominance of the former type. Besides fibrosis, a chronic inflammatory process and an anomalous lymphatic rich vascular pattern were observed in the deep endocardium, connected to the terminal coronary circulation of the myocardium, which might be an important pathological finding concerning EMF pathogenesis. Molecular analysis of the endomyocardium revealed high incidence of cardiotropic infective agents (6/12, 50%); however, their role in the disease pathogenesis is still controversial.

摘要

心内膜心肌纤维化(EMF)是一种病因不明的限制性心肌病,在热带地区较为常见,影响一个或两个心室的流入道和心尖,表现为心内膜和相邻心肌的纤维性增厚。对于纽约心脏协会心功能分级为III级或IV级的患者,建议进行手术治疗。心脏的大体和组织学特征已在尸检中得到全面研究,但手术样本的研究仍很缺乏。描述了从32例患者收集的手术样本中EMF的组织学和免疫组化特征,并将其与临床数据相关联。对福尔马林固定的心内膜样本进行聚合酶链反应(PCR)和逆转录PCR,以回顾性检测某些嗜心性病毒和弓形虫的基因组。心室心内膜被I型和III型浅表无细胞透明胶原纤维增厚,以前者为主。除纤维化外,在深心内膜中观察到慢性炎症过程和异常丰富的淋巴管血管模式,其与心肌的终末冠状动脉循环相连,这可能是关于EMF发病机制的一个重要病理发现。心内膜的分子分析显示嗜心性感染因子的高发生率(6/12,50%);然而,它们在疾病发病机制中的作用仍存在争议。

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