OMPHALOCELE AND GASTROSCHISIS IN EUROPE - A SURVEY OF 3-MILLION BIRTHS 1980-1990
OMPHALOCELE AND GASTROSCHISIS IN EUROPE - A SURVEY OF 3-MILLION BIRTHS 1980-1990
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DOI：
10.1002/ajmg.1320580218
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发表时间：
1995-08-28
期刊：
影响因子：
--
通讯作者：
STONE, DH
中科院分区：
文献类型：
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作者：
CALZOLARI, E;BIANCHI, F;STONE, DH
在1980-1990年期间，在欧洲21个地区的登记处（EUROCAT登记处）共登记了732例脐膨出和274例胃裂。脐膨出的总患病率为2.52 / 10000，胃裂的总患病率为0.94 / 10000。脐膨出在不同地区的总患病率存在显著的异质性。在不列颠群岛的五个中心，脐膨出的总患病率始终高于平均水平。这在很大程度上与脐膨出和神经管缺陷之间的联系有关。在与神经管缺陷相关的脐膨出病例中，与其他脐膨出病例中微不足道的男性过剩相比，观察到明显的女性过剩。腹裂总患病率的地理差异部分是由调查人群中产妇年龄分布的差异所解释的。脐膨出为孤立性畸形，占46%；79%的病例分离出胃裂。单发畸形和多发畸形脐膨出和胃裂出的平均出生体重和胎龄均较低，尤其是多发畸形，且单发胃裂出比单发脐膨出的出生体重和胎龄更低。产前诊断导致终止妊娠的比例为33.2%的脐膨出和26.5%的胃裂，这表明目前产前筛查项目的影响相当大。根据临床表现、流行病学特征以及其他畸形的存在和类型，脐膨出和胃裂可被认为是异质性疾病。(C) 1995 Wiley-Liss, Inc。
A total of 732 cases of omphalocele and 274 cases of gastroschisis was registered in 21 regional registers in Europe (EUROCAT registers) during the period 1980-1990. The total prevalence rates were 2.52 pes 10,000 for omphalocele and 0.94 per 10,000 for gastroschisis. There was significant heterogeneity in total prevalence rates among regions for omphalocele. Consistently higher than average total prevalence rates of omphalocele were found in the five centers of the British Isles. This was in large part linked to the association between omphalocele and neural tube defects. A significant female excess among the cases of omphalocele associated with neural tube defects, in comparison with an insignificant male excess for other cases of omphalocele, was observed. Geographical differences in the total prevalence of gastroschisis are partly explained by differences in maternal age distributions in the populations surveyed. Omphalocele was an isolated malformation in 46% of cases; gastroschisis was isolated in 79% of cases. The average birthweight and gestational age of both isolated and multiply malformed cases of both omphalocele and gastroschisis were low, especially for multiply malformed cases, and to a greater extent for isolated gastroschisis than for isolated omphalocele. Prenatal diagnosis leading to termination of pregnancy was reported in 33.2% of omphalocele and in 26.5% of gastroschisis cases, demonstrating the considerable impact of current prenatal screening programs. On the basis of clinical manifestations, epidemiologic characteristics, and the presence and type of additional malformations, omphalocele and gastroschisis can be considered heterogeneous conditions. (C) 1995 Wiley-Liss, Inc.