我国儿童激素敏感、复发/依赖肾病综合征诊疗现状的多中心研究
아국인동격소민감、복발/의뢰신병종합정진료현상적다중심연구
Multicenter survey of diagnostic and therapeutic status in Chinese childhood patients with steroid-sensitive, relaping/steroid-dependent nephrotic syndrome
  • 万方数据
    中华儿科杂志 중화인과잡지
    Chinese Journal of Pediatrics
    2014年 3期 194-200 ,共7页

    全国儿童常见肾脏病诊治现状调研工作组

    전국인동상견신장병진치현상조연공작조

    肾病%临床方案%多中心研究%儿童 腎病%臨床方案%多中心研究%兒童
    신병%림상방안%다중심연구%인동
    Nephrosis%Clinical protocols%Multicenter study%Child
    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±6.4)g/L,血胆固醇水平为(10.0±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·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),尿蛋白转阴的中位时间为8(7)d和8(8)d.频复发/激素依赖NS患儿中有53.7％(402/749)加用免疫抑制剂,首选环磷酰胺211例(52.5％),其后依次为环孢素108例(26.9％),霉酚酸酯70例(17.4％)和他克莫司40例(10.0％).24.0％(894例)的患儿加用血管紧张素转换酶抑制剂类药物,0.8％(28例)加用血管紧张素受体拮抗剂(ARB)类药物以及2.8％(106例)加用左旋咪唑.结论 近年来我国儿童PNS的住院患儿数占泌尿系统疾病住院患儿总数的比例较为稳定,发病高峰在学龄前期,以男孩和初发病例多见.《指南》(试行稿)推荐的诊断标准更适合我国PNS患儿使用,尚待进一步推广.《指南》推荐的治疗方案有较好的近期疗效,长期疗效有待进一步研究.
    목적 료해아국인동격소민감、복발/의뢰신병종합정(NS)적진료현상,평개2009년《인동격소민감、복발/의뢰신병종합정진치순증지남》(시행고)(간칭《지남》)적괄용성화추엄성.방법 유중화의학회인과학분회신장병학조통일제정격소민감、복발/의뢰NS진료현상조연표화단위진단신식표,분발지전국각협작단위진행회고성병례등기,전국공19개성27개시2개자치구화4개직할시적37소협작의원삼여,대2008년화2011년수차우조연단위학진위격소민감、복발/의뢰NS,년령≤18세적주원환인진행회고성조사분석.결과 원발성신병종합정(PNS)환인점동기비뇨계통질병주원환인총수적20.0％(7 444/37 134).공3 725례격소민감、복발/의뢰NS환인납입연구,남녀비례3.2:1,년령2개월～18.0세,중위수(사분위간거)년령4.1세(4.2)세.림상분형이단순형(3 186례,85.5％)위주,신염형위539례(14.5％).초발2 825례(75.8％),복발1 050례(28.2％),빈복발749례(20.1％),격소의뢰NS 221례(5.9％).학진시뇨단백정성이3+ ～4+(3 260례,점87.5％)위주,24 h뇨단백정량중위수(사분위간거)위112.0(109.3)mg/kg,평균혈장백단백수평위(17.9±6.4)g/L,혈담고순수평위(10.0±3.1) mmol/L.초발치료이구복발니송위주,위92.8％(2 622/2 825),제량위2 mg/(kg·d).뇨단백전음중위시간위9(6)d,96.1％(2 714/2 825)적환인우4주내전음,4～8주전음적부점3.6％(101/2 825).족량격소사용적중위시간2008화2011년분별위30(14)d화31(14)d,격소총료정적중위수위10개월.복발、빈복발/격소의뢰NS적치료균이재차사용족량격소위주,분별점38.3％ (402/1 050)화33.6％(252/749).복발、빈복발/격소의뢰NS환인경불동치료방안치료후뇨단백전음솔분별위77.5％ (814/1 050)화75.4％(565/749),뇨단백전음적중위시간위8(7)d화8(8)d.빈복발/격소의뢰NS환인중유53.7％(402/749)가용면역억제제,수선배린선알211례(52.5％),기후의차위배포소108례(26.9％),매분산지70례(17.4％)화타극막사40례(10.0％).24.0％(894례)적환인가용혈관긴장소전환매억제제류약물,0.8％(28례)가용혈관긴장소수체길항제(ARB)류약물이급2.8％(106례)가용좌선미서.결론 근년래아국인동PNS적주원환인수점비뇨계통질병주원환인총수적비례교위은정,발병고봉재학령전기,이남해화초발병례다견.《지남》(시행고)추천적진단표준경괄합아국PNS환인사용,상대진일보추엄.《지남》추천적치료방안유교호적근기료효,장기료효유대진일보연구.
    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 ± 6.4) g/L and mean cholesterol was(10.0 ±3.1) mmol/L.Oral prednisone 2 mg/(kg · d) was the main treatment of the first episode cases (92.8％,2 622/2 825).The median time of proteinuria remission was 9 (6) days; 96.1％ (2 714/2 825) cases reached complete remission in 4 weeks and 3.6％ (101/2 825) in 4-8 weeks.The median time for using full dose corticosteroid (GC) was 31 days and total time for using GC was 10 months.Of 1 050 cases,402 (38.3％) RNS and 33.6％ (252/749) FR/SD cases were treated with full dose GC again.After different treatment regimens,77.5％ (814/1 050) RNS and 75.4％ (565/749) FR/SDNS cases reached remission.The median time of proteinuria remission was 8 days.53.7％ (402/749) FR/SDNS were treated with immunosuppressant and the first choice was CTX 211 cases (52.5％),then CsA 108 cases (26.9％),MMF 70 cases (17.4％) and FK506 40 cases (10.0％).The cases treated with ACEI accounted for 24.0％ (894 cases),ARB and levamisole accounted for 0.8％ (28 cases) and 2.8％ (106 cases),respectively.Conclusion In recent years,the proportion of children with PNS in the hospitalized childhood patients with urologic-kidney diseases was stable.The most common cases were the first episode,mainly in pre-school boys.The evidence-based guideline on diagnosis of SSNS,RNS/ SDNS (for trial implementation) conducted by The Subspecialty Group of Nephrology,Society of Pediatrics,Chinese Medical Association in 2009 was suitable for Chinese children with PNS,and remained to be further popularized.The guideline recommendation on treatment had a satisfactory recent curative outcome,and the long-term outcome needs further research.
    원문보기 원문다운로드 사이트로이동
저자의 최근 논문