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坎特雷尔五联症:病例报告及文献综述

Pentalogy of Cantrell: case report and review of the literature.

作者信息

Kheir Abdelmoneim E M, Bakhiet Elghazali A, Elhag Salma M M, Karrar Mohamed Z

机构信息

Department of Paediatrics and Child Health , Faculty of Medicine, University of Khartoum , Sudan.

Department of Obstetrics and Gynaecology , Faculty of Medicine, University of Khartoum , Sudan .

出版信息

Sudan J Paediatr. 2014;14(1):85-8.

Abstract

Pentalogy of Cantrell is a syndrome that consists of five anomalies: a midline, upper abdominal wall abnormality; lower sternal defect; anterior diaphragmatic defect; diaphragmatic pericardial defect, and congenital abnormalities of the heart. The pathogenesis of this condition is not fully known yet, associations are common with this condition and treatment is extremely challenging. Prognosis and outcome depends on the type of the pentalogy of Cantrell and associated cardiac anomalies. It can be diagnosed reliably by antenatal ultrasound during the first trimester. We describe a Sudanese baby of undetermined sex who had the typical features of pentalogy of Cantrell and died shortly after birth.

摘要

坎特雷尔五联症是一种由五种异常组成的综合征

中线、上腹壁异常;胸骨下段缺损;前膈缺损;膈心包缺损以及心脏先天性异常。这种病症的发病机制尚未完全明确,常伴有其他相关病症,治疗极具挑战性。预后和结果取决于坎特雷尔五联症的类型以及相关心脏异常情况。在孕早期通过产前超声检查可可靠地诊断出来。我们描述了一名性别未明的苏丹婴儿,其具有坎特雷尔五联症的典型特征,出生后不久便死亡。

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