围产儿腹裂畸形的全国流行病学调查

EpidemiologyofGastroschisisinChina

目的：探讨我国围产儿腹裂畸形的临床表现和流行病学特征，为预防和治疗提供科学依据。方法：１９８６年１０月～１９８７年９月间，在我国原２９个省、市、自治区９４５所医院，对妊娠２８周至出生后７天的围产儿进行出生缺陷监测。结果：在监测的１２４３２８４例住院分娩围产儿中，共检出腹裂１９４例；全国平均发生率为１．６／万；各省发生率从０．４／万至３．４／万。山东省发生率最高（３．４／万），与全国平均水平比较，差异有显著性（Ｐ＜０．０１）。低体重儿中腹裂发生率（１．６／万）高于正常体重儿（０．６／万，Ｐ＜０．０１）；乡村发生率（２．５／万）高于城镇（１．１／万，Ｐ＜０．０１）。７０．６％的腹裂伴有其他畸形。患儿围产期死亡率９１．８％，且多发畸形的腹裂死亡率（９５．６％）显著高于单发腹裂畸形（８２．５％，Ｐ＜０．０１）。产前诊断的腹裂畸形仅占１５．０％。结论：我国围产儿腹裂畸形的状况与国外比较，产前诊断率低，畸形严重，且常伴有其他多发畸形，围产期死亡率高。

Objective:Tostudytheclinicalandepidemiologiccharacteristicsofperinateswithgastroschisis(GA)inChina.Methods:Thebirthdefectsmonitoringprogramwasundertakenin945hospitalsfrom29provinces,citiesandautonomousregionsofChina.Dataaboutperinatesincludingintrauterinedeathandstilbirthfrom28weeksofgestationtoaperiodof7daysafterbirthwascol-lectedthroughthemedicalrecordsbetweenOctober1986andSeptember1987.Results:Atotalof194caseswithGAwasfoundin1243284perinatesmonitored.TheincidenceofGAinChinavariedfrom0.4to3.4withameanof1.6per10000.ThehighestprevalencewasinShandongprovince(3.4/10000)whichwassignificantlyhigherthantheaverage(P<0.01).TheprevalenceofGAperinateswithlowbirthweightwashigherthanthatwithnormalbirthweight(1.6vs0.6per10000,P<0.01),aswelasthatintheruralareahigherthanintheurbanarea(2.5vs1.1per10000,P<0.01).70.6%ofthecaseswithGAwereacompaniedwithothermajordefects.Theover-alperinatalmortalityrate(PMR)ofGAwas91.8%.PMRofGAwithotherbirthdefects(95.6%)wassignificantlyhigherthanthatofGAonly(82.5%,P<0.01).Only15%ofGAwerediagnosedbeforebirth.Conclusions:GAwasaseriousmalformation.CaseswithGAhadhighPMRandlowdetectionratesinprenataldiagnosis,andmostoftenacompaniedwithmultipledefects.