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脑瘫中先天性异常的患病率及发病机制。

Prevalence and pathogenesis of congenital anomalies in cerebral palsy.

作者信息

Pharoah Peter O D

机构信息

FSID Unit of Perinatal and Paediatric Epidemiology, Department of Public Health, University of Liverpool, Liverpool L69 3GB, UK.

出版信息

Arch Dis Child Fetal Neonatal Ed. 2007 Nov;92(6):F489-93. doi: 10.1136/adc.2006.107375. Epub 2007 Apr 11.

Abstract

BACKGROUND

It has been hypothesised that cerebral palsy (CP) and other congenital anomalies are attributable to feto-fetal transfusion problems in a monochorionic multiple gestation. Thus more than one organ could be compromised leading to the coexistence of two or more anomalies in a fetus. Such anomalies in a singleton birth may be attributable to early demise of the co-conceptus as a vanishing twin.

AIM

To determine whether the coexistence of congenital anomalies and CP is greater than a chance finding by comparing the prevalence of congenital anomalies in children with CP with that in the general population of children.

METHODS

A population-based register of children with CP born in 1966-1991 in the counties of Merseyside and Cheshire, UK, comprised the index population. Coexisting congenital anomalies were recorded. For comparison the population prevalence of congenital anomalies was obtained from eight congenital malformation registers in the UK.

RESULTS

Children with CP were found to have highly significant increases in risk for microcephaly, isolated hydrocephaly, congenital anomalies of the eye, congenital cardiac anomalies, cleft lip and/or palate and congenital dislocation of the hips and talipes (p<0.001) and atresias of the oesophagus (p<0.001) and intestines (p<0.01). The relative risks ranged from 3.1 (95% CI 1.9 to 4.8; p<0.001) for congenital malformations of the cardiac septa to 116.09 (95% CI 84.0 to 162.3; p<0.001) for microcephaly.

CONCLUSIONS

Congenital anomalies in children with CP are found much more frequently than expected by chance. A common pathogenic mechanism may account for the coexistence of disparate congenital anomalies. A hypothesis is proposed for such a common pathogenic mechanism.

摘要

背景

有假说认为,脑性瘫痪(CP)及其他先天性异常可归因于单绒毛膜多胎妊娠中的胎儿-胎儿输血问题。因此,不止一个器官可能受到损害,导致胎儿出现两种或更多种异常并存的情况。单胎出生时的此类异常可能归因于作为消失双胎的共存胚胎的早期死亡。

目的

通过比较CP患儿与普通儿童群体中先天性异常的患病率,确定先天性异常与CP并存是否超出偶然发现的范畴。

方法

以英国默西塞德郡和柴郡1966 - 1991年出生的CP患儿的人群登记册作为索引人群。记录并存的先天性异常。为作比较,从英国八个先天性畸形登记册获取先天性异常的人群患病率。

结果

发现CP患儿患小头畸形、孤立性脑积水、眼部先天性异常、先天性心脏异常、唇裂和/或腭裂、先天性髋关节脱位和马蹄内翻足(p<0.001)以及食管闭锁(p<0.0

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