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扩张型心肌病(DCM)是儿童时期心肌疾病中最常见的一种类型,近年来诊断的病例逐渐增多,对小儿健康造成严重危害,是儿童心力衰竭的常见病因。本病病理改变主要为心肌广泛纤维变性。本病起病隐匿,而病情进展快。临床表现以心力衰竭和心脏扩大为主,大多数患儿临床诊断时已有心功能减低。本病预后差,死亡率高,误诊率达34%,故提高对本病的认识,早期诊断及治疗对改善预后至关重要。目前DCM的病因及发病机制仍不完全明确,主要认为与遗传因素、病毒感染以及病毒感染后免疫反应参与等有关。研究发现,在30%~48%DCM患儿中有亲子遗传现象,主要为常染色体显性遗传、常染色
Dilated cardiomyopathy (DCM) is the most common type of childhood heart disease. The number of cases diagnosed in recent years has been gradually increased, causing serious harm to children’s health. It is a common cause of heart failure in children. Pathological changes of the disease mainly for myocardial extensive fibrosis. The disease occult onset, and the rapid progress of the disease. The clinical manifestations of heart failure and enlarged heart-based, most children have clinical diagnosis of cardiac dysfunction. The poor prognosis of the disease, high mortality, misdiagnosis rate of 34%, so to improve awareness of the disease, early diagnosis and treatment of the improvement of prognosis is essential. At present, the etiology and pathogenesis of DCM is still not completely clear, mainly related to genetic factors, viral infection and immune response after virus infection. The study found that in 30% to 48% of DCM children with parental genetic phenomenon, mainly autosomal dominant inheritance, often stained