摘要
为探讨儿童狼疮脑病的临床特点及预后，通过长期随访，对１３０例系统性红斑狼疮（ＳＬＥ）患儿中的３９例狼疮脑病的临床资料进行了分析和总结。结果，狼疮脑病的发生率为３０％。其中８５％（３３／３９）的患儿在确诊ＳＬＥ第１年内出现中枢神经系统（ＣＮＳ）受损，１８％（７／３９）以ＣＮＳ损害作为ＳＬＥ首发症状。最常见ＣＮＳ异常是癫痫发作、头痛、昏迷、急性器质性脑病综合征样表现。随访的２５例中，坚持按医嘱服泼尼松者１２例，病情持续稳定；间断服药、自行加减泼尼松剂量１１例，病情波动，其中６例间断发热、皮疹、关节肿痛，５例遗留ＣＮＳ后遗症；自行停止治疗的２例均死亡。提示，ＣＮＳ受累是儿童ＳＬＥ常见的严重并发症，临床表现具多样性，故应注意鉴别诊断。早期诊断和治疗，长期随访和坚持按医嘱服药，可望获得良好预后。
Tostudythecharacteristicsandprognosisofcentralnervoussystem(CNS)involve-mentinchildhoodsystemiclupuserythematosis(SLE).Wereviewed39childrenwithCNSinvolve-mentamong130patientswithSLEfrom1984to1994.Thirty-theseofthe39children(85%)hadCNSmanifestationsoneyearaftertheonsetofSLE;7of39(18%)hadCNSsymptomsattheonsetofthedisease.Themostcommonmanifestationsobservedwereseizures,headacheandcoma.Twen-ty-fivecaseswereunderfolow-up.Twelevepatientsconditionwasstablewithregularadministrationofprednisone.Elevenpatients′conditionwasunstablebecauseoftakingprednisoneiregularly.Sixofthepatientshadfever,arthritis,rashofandonandfivehadresidualCNSabnormalities.Twocasesdiedfromdisecontinuedtreatment.WeconcludethatCNSinvolvementisacommonandseri-ouscomplicationofchildhoodslewithbroaddiversityinclinicalmanifestations.Long-termfolow-upisneded.CNSlupusinchildrhoodmayhaveafavourableprognosisiftreatedtimelyandregularly.
出处
《中华儿科杂志》
CAS
CSCD
北大核心
1996年第4期240-242,共3页
Chinese Journal of Pediatrics
