bartter综合征 Bartter综合征35例综合报道_王峥.doc

bartter综合征 Bartter综合征35例综合报道_王峥 导读：就爱阅读网友为您分享以下“Bartter综合征35例综合报道_王峥”资讯，希望对您有所帮助，感谢您对92的支持! 华西医学1999;14(4)　CN51-1356/R [文章编号]1002-0179(1999)04-0403-03403 Bartter综合征35例综合报道 SynthetiallyReportof35ChildrenwithBartter’sSyndrome 12王　峥　倪俊学　陈大鹏　陈　洪 WANGZheng,NIJun-xue,CHENDa-peng,etal. 华西医科大学附属第二医院儿科(成都　610041) DepartmentofPediatrics,TheSecondAffiliatedHospital,WCUMS,Chengdu,610041 　　摘要:Bartter综合征是一种极罕见的疾病。本文收集本院1例及全国其他34例,进行综合分析。发现儿童期发病与成人基本相等,儿童以男性为多(66.7%),成人则以女性为多(82.3%)。特点有:(1)水、电解质及酸碱失衡,包括低钾、钠血症、低氯性碱中毒及脱水等;(2)血肾素、血管紧张素Ⅱ及醛固酮等均明显升高,而血压正常;(3)生长发育迟滞在儿童最为突出(84.6%),其中4例有智力低下;(4)14例肾活检肾脏病变除肾小球旁器增生,还有肾小管间质及肾小球损害;(5)以吲哚美辛、螺内酯及补钾等综合治疗效果显著。结合病理、伴发疾病、症状及实验室检查等分析发现,成人以后天获得性为多,而儿童大多为特发性。 关键词:Batter综合征　低钾血症　低氯性碱中毒 [中图分类号]R722.11　　　[文献标识码]A 　　Abstract:Bartter’ssyndromeisararedisease.Thisstudyanalysed35casesofthediseaseforthefirsttimeinourcountry,inwhichonecaseswasreportedfromourhospitaland34casesreportedthroughoutthecountry.Wefoundtheincidencewasal-mostthesamebetweenchildrenandadults.Themajorityofthepatientsinchildhoodweremale(66.7%)andthatoftheadultwasfemale(82.3%).ThehallmarksofBartter’ssyndromewerementionedbelow:(1)Water-electrolytesandacid-basedis-turbancesincludinghypokalemia,hyponatremia,hypochloremicalkalosisanddehydration;(2)Obviouslyincreaseofrenin,an-giotensionⅡandaldosterone,butthebloodpressurealmostnormal;(3)Failuretothrivewasprominentfeatureofthepatientinchildhood,amongthem4caseshadmentalretardation;(4)Rnealhistologywascharacterizedbythedamagetorenaltubules,interstitialtissue,glomerulineighboringthejuxataglomerularcellhyperplasiaand(5)Comprehensivetherapywithindomethacin,spirolactoneandpotassiumhadremarkableeffect.Byanalysiscombinedwithclinicalmanifestations,laboratoryexaminationandrenalpathology,wefoundthatthepatientsinadultweremostlysecondaryandtheseinchildhoodweremainlyspecific. Keywords:Bartter’ssyndrome;Hypokalemia;Hypochloremicalkalosis (Receiveddate:1999-08-31) 　　Bartter综合征是一种低钾血症、低 氯性碱中毒、肾素、血管紧张素Ⅱ及醛 固酮增高,血压正常,而外源性血管加 压素反应低下等特点的疾病。Bartter于 1962年首先报告〔1〕,后经