原发性抗磷脂综合征并扩张型心肌病1 例报告并文献复习.pdfVIP

维普资讯 籼 豪京刍第21卷第9期2o06年5月JApplClinPedin．Vo1．21No．9．May2006 - 538 · · 论 著 - 原发性抗磷脂综合征并扩张型心肌病 1例报告并文献复 习 张清友 ，李新涛 ，杜军保 ，陈永红 ，肖慧捷 ，李万镇 (1．北京大学第一医院儿科，北京 100034；2．孝感市中心医院儿科，湖北 孝感 432000) CaseReportandLiteratureReview ofDilatedCardiomyopathyinPrimaryAntiphospholipidSyndrome ZHANG ng w “‘LIXin—tafJ，I)UJun ^Hf，0，()HEN Yoag hung。，XIAO Hui— ，‘LJV zhe~l‘ ， (1．DepartmentofPediatrics，theFirstHospitalof[ekingUniversity，Heijing100034，China：2．DepartmentofPdeiatrics，Xiaogan CityCentralHospital，Xiaogan432001)，China) Abstract：Objective Primaryantiph~xspholipidsyndrome(PAPS)isararesyndromeinchildren，anddilatedcardiomyopathyin PAPSisextremelyrare．Atpresent．only1childw rebX)rtedasantiphcxspholipidsyndmme(APS)withdilatedcardiomyopathy．Aenid sufferingfrom heartfailurewasdiagntxsed．Methods Accordingtothesyn~ptoms．signs，pasthistory，latx)ratoryexaminationandthepre— viouspublishedpapersofotherauthors，ehiktsd；seasewasdiagnose@andtreated。andrelateelliteraturewsreviewedResults A 12一 yearoIdLDvpre~ntde withheartfailure，fatigue，swollenlimbsandoliguriaHisechocardiogram andheartMRIshowde thattheleftex— tendedventriculardiameter．1ow systolicfunction，andmildmitral，pulmonaryvalveandaorticvalveregulation．HishaertMRIal showed D myocardialfibrosis．sothedilatde cardiomyopathywasdiagno~dl~alyoratorystudiesshowde thepreaeneeofmildthrombosytopenia，high tiltersofanticardiolipinnatibodies，anti一 glycoproteinlantitxxtiesandalupusnatiengulant．Adiagnosis0fPAPSwasmadebecna~we couldnotdocumentanyunderlyingcause8oftheAPS．He．wastreatedwithhighdn∞ intravenousimmuncglobuli