摘要
目的 了解我国儿童激素敏感、复发/依赖肾病综合征（NS）的诊疗现状,评价2009年《儿童激素敏感、复发/依赖肾病综合征诊治循证指南》（试行稿）（简称《指南》）的适用性和推广性.方法 由中华医学会儿科学分会肾脏病学组统一制定激素敏感、复发/依赖NS诊疗现状调研表和单位诊断信息表,分发至全国各协作单位进行回顾性病例登记,全国共19个省27个市2个自治区和4个直辖市的37所协作医院参与,对2008年和2011年首次于调研单位确诊为激素敏感、复发/依赖NS,年龄≤18岁的住院患儿进行回顾性调查分析.结果 原发性肾病综合征（PNS）患儿占同期泌尿系统疾病住院患儿总数的20.0％（7 444/37 134）.共3 725例激素敏感、复发/依赖NS患儿纳入研究,男女比例3.2：1,年龄2个月～18.0岁,中位数（四分位间距）年龄4.1岁（4.2）岁.临床分型以单纯型（3 186例,85.5％）为主,肾炎型为539例（14.5％）.初发2 825例（75.8％）,复发1 050例（28.2％）,频复发749例（20.1％）,激素依赖NS 221例（5.9％）.确诊时尿蛋白定性以3＋ ～4＋（3 260例,占87.5％）为主,24 h尿蛋白定量中位数（四分位间距）为112.0（109.3）mg/kg,平均血浆白蛋白水平为（17.9＆#177;6.4）g/L,血胆固醇水平为（10.0＆#177;3.1） mmol/L.初发治疗以口服泼尼松为主,为92.8％（2 622/2 825）,剂量为2 mg/（kg＆#183;d）.尿蛋白转阴中位时间为9（6）d,96.1％（2 714/2 825）的患儿于4周内转阴,4～8周转阴的仅占3.6％（101/2 825）.足量激素使用的中位时间2008和2011年分别为30（14）d和31（14）d,激素总疗程的中位数为10个月.复发、频复发/激素依赖NS的治疗均以再次使用足量激素为主,分别占38.3％ （402/1 050）和33.6％（252/749）.复发、频复发/激素依赖NS患儿经不同治疗方案治疗后尿蛋白转阴率分别为77.5％ （814/1 050）和75.4％（565/749）,尿蛋白转阴的中�
Objective Nephrotic syndrome （NS） was recognized as a clinical syndrome caused by massive proteinuria,leading to hypoalbuminemia and edema.Primary nephrotic syndrome,accounted for about 90% of NS,seems to be one of common urologic-kidney diseases in childhood.The diagnosis and treatment of NS had so far failed to be unified nationwide.The purpose of this research was to survey the diagnosis and treatment status of steroid-sensitive,relaping/steroid-dependent nephrotic syndrome （SSNS,RNS/SDNS） in Chinese children and to evaluate the applicability and promotion of the evidence-based guideline on diagnosis and treatment of SSNS,RNS/SDNS （for trial implementation） conducted by The Subspecialty Group of Nephrology,the Society of Pediatrics,Chinese Medical Association in 2009.Method Questionnaires concerning children with SSNS,RNS/SDNS were designed by The Subspecialty Group of Nephrology,Society of Pediatrics,Chinese Medical Association and distributed to the doctors of 37 centers of 27 cities,4 municipalities and 2 autonomous regions.A nationwide survey was conducted and data of hospitalized children （younger than 18 years of age） with SSNS,RNS/SDNS in 2008 and 2011 were analyzed retrospectively.Result Children diagnosed as primary nephrotic syndrome （PNS） accounted for 20.0% （7 444/37 134） of the hospitalized cases with urologic-kidney diseases in the corresponding time period; 3 725 cases were enrolled into this study.The male to female ratio was 3.2∶1 and the median age was 4.1 years（Q =4.2 years） ; 3 186 （85.5%） cases were diagnosed as simple NS and 539 （14.5%） as nephritic NS; 2 825（75.8%）PNS cases were the first episode and 1 050（28.2%） cases were the relapse; 221 （5.9%） cases were diagnosed as SDNS and 749（20.1%） as frequently relapse NS （FRNS）.At NS diagnosis,the proteinuria was 3 ＋-4 ＋ （3 260 cases,87.5%）,median quantitive proteinuria for 24 hours was 112.0 mg/kg（Q =109.3 mg/kg）,mean plasma albumin was（17.9 ＆#177; 6.4） g/L and mean c
出处
《中华儿科杂志》
CAS
CSCD
北大核心
2014年第3期194-200,共7页
Chinese Journal of Pediatrics
关键词
肾病
临床方案
多中心研究
儿童
Nephrosis
Clinical protocols
Multicenter study
Child
