单中心20年原发性肾病综合征不同激素效应患儿临床特点及预后分析
단중심20년원발성신병종합정불동격소효응환인림상특점급예후분석
Clinical characteristics and prognosis analysis of children with idiopathic nephrotic syndrome with different responses to steroid therapy from a single center in 20 years
  • 万方数据
    中华实用儿科临床杂志 중화실용인과림상잡지
    Journal of Applied Clinical Pediatrics
    2014年 17期 1291-1295 ,共5页

    管娜%丁洁%杨霁云%肖慧捷%姚勇%刘景城%朱赛楠%钟旭辉%王芳

    관나%정길%양제운%초혜첩%요용%류경성%주새남%종욱휘%왕방

    肾病综合征%激素效应%标准%儿童 腎病綜閤徵%激素效應%標準%兒童
    신병종합정%격소효응%표준%인동
    Nephrotic syndrome%Steroid response%Criteria%Child
    目的 了解原发性肾病综合征患儿激素效应状况及不同激素效应患儿的临床特点和预后.方法 回顾性分析北京大学第一医院1993年1月至2012年12月原发性肾病综合征(24h尿蛋白定量≥50 mg/kg,血浆清蛋白＜25 g/L)患儿的临床资料.纳入随访1年以上者,根据患儿对足量激素的效应分为4周内转阴组、4～8周转阴组和8周耐药组.比较各组性别、年龄、临床表型、并发症、激素不良反应和严重预后(慢性肾功能不全、肾移植或死亡)发生情况的差异.结果 共1 059例患儿符合原发性肾病综合征,激素效应明确者841例,其中4周以内转阴者603例(71.7％,603/841例),4～8周转阴者34例(4.0％,34/841例),8周未转阴者204例(24.3％,204/841例),初次治疗尿蛋白平均转阴时间(13.2±8.9)d,中位转阴时间10.0 d.对随访病程1年以上的369例行进一步分析,4周内转阴组276例(74.8％,276/369例),4～8周转阴组18例(4.9％,18/369例),8周耐药组75例(20.3％,75/369例).3组性别分布相似,起病年龄差异有统计学意义(P＜0.01),4周内转阴组、4～8周转阴组和8周耐药组起病年龄分别为(5.2±3.3)岁、(5.7±3.3)岁和(6.6±3.8)岁.4～8周转阴组(单纯型83.3％,15/18例)与4周内转阴组(单纯型95.3％,263/276例)均以单纯型为主,与8周耐药组(单纯型45.3％,34/75例)比较差异有统计学意义(P＜0.01).4～8周转阴组(11.1％,2/18例)与4周内转阴组(4.3％,12/276例)及8周耐药组(0)比较,急性肾上腺皮质功能不全发生率差异有统计学意义(P=0.04).3组间其余激素不良反应发生率比较差异无统计学意义.4周内转阴组(1.4％,4/276例)、4～8周转阴组(0)和8周耐药组(16.0％,12/75例)比较,严重预后发生率差异有统计学意义(P＜0.01).结论 71.7％的原发性肾病综合征患儿在足量激素治疗4周内尿蛋白转阴,4周内尿蛋白未转阴者14.3％的患儿继续足量激素治疗可能在8周内转阴,其余患儿即使足量激素治疗8周尿蛋白亦不能转阴.足量激素治疗8周内转阴患儿以单纯型为主,预后较好,8周耐药者以肾炎型为主,预后较差.对于足量激素治疗4～8周内转阴的患儿需警惕急性肾上腺皮质功能不全的风险.
    목적 료해원발성신병종합정환인격소효응상황급불동격소효응환인적림상특점화예후.방법 회고성분석북경대학제일의원1993년1월지2012년12월원발성신병종합정(24h뇨단백정량≥50 mg/kg,혈장청단백＜25 g/L)환인적림상자료.납입수방1년이상자,근거환인대족량격소적효응분위4주내전음조、4～8주전음조화8주내약조.비교각조성별、년령、림상표형、병발증、격소불량반응화엄중예후(만성신공능불전、신이식혹사망)발생정황적차이.결과 공1 059례환인부합원발성신병종합정,격소효응명학자841례,기중4주이내전음자603례(71.7％,603/841례),4～8주전음자34례(4.0％,34/841례),8주미전음자204례(24.3％,204/841례),초차치료뇨단백평균전음시간(13.2±8.9)d,중위전음시간10.0 d.대수방병정1년이상적369례행진일보분석,4주내전음조276례(74.8％,276/369례),4～8주전음조18례(4.9％,18/369례),8주내약조75례(20.3％,75/369례).3조성별분포상사,기병년령차이유통계학의의(P＜0.01),4주내전음조、4～8주전음조화8주내약조기병년령분별위(5.2±3.3)세、(5.7±3.3)세화(6.6±3.8)세.4～8주전음조(단순형83.3％,15/18례)여4주내전음조(단순형95.3％,263/276례)균이단순형위주,여8주내약조(단순형45.3％,34/75례)비교차이유통계학의의(P＜0.01).4～8주전음조(11.1％,2/18례)여4주내전음조(4.3％,12/276례)급8주내약조(0)비교,급성신상선피질공능불전발생솔차이유통계학의의(P=0.04).3조간기여격소불량반응발생솔비교차이무통계학의의.4주내전음조(1.4％,4/276례)、4～8주전음조(0)화8주내약조(16.0％,12/75례)비교,엄중예후발생솔차이유통계학의의(P＜0.01).결론 71.7％적원발성신병종합정환인재족량격소치료4주내뇨단백전음,4주내뇨단백미전음자14.3％적환인계속족량격소치료가능재8주내전음,기여환인즉사족량격소치료8주뇨단백역불능전음.족량격소치료8주내전음환인이단순형위주,예후교호,8주내약자이신염형위주,예후교차.대우족량격소치료4～8주내전음적환인수경척급성신상선피질공능불전적풍험.
    Objective To analyze the clinical characteristics and prognosis of children diagnosed with idiopathic nephrotic syndrome that showed different responses to steroid treatment.Methods In this study,idiopathic nephrotic syndrome was defined as a 24-hour urinary protein of more than 50 mg/kg body weight as well as a serum albumin concentration of less than 25 g/L.A retrospective study was undertaken to analyze the children who were patients at Peking University First Hospital during Jan.1993 and Dec.2012 and conformed to the above criteria.According to their response to steroid therapy,the children were divided into 3 groups:group that demonstrated remission within 4 weeks after therapy,group that demonstrated remission within 4 to 8 weeks and group that demonstrated resistance to therapy after 8 weeks.Among the children who had more than 1 year of follow up,type of nephrotic syndrome(simple vs nephritic),complications,steroid side effects and serious prognosis (chronic renal failure,renal transplantation or death) were further analyzed.Results Out of total 1 059 children with idiopathic nephrotic syndrome,841 cases had a documented steroid response and were analyzed.Among them,71.7％ (603/841 cases) showed remission within 4 weeks following steroid treatment,while 4.0％ (34/841 cases) showed remission within 4 to 8 weeks and 24.3％ (204/841 cases) showed no remission even after 8 weeks.The average remission time after steroid therapy was(13.2 ± 8.9) d with a median of 10.0 d.Of these children,369 cases had at least 1 year of follow up.In this subset,74.8％ (276/369 cases) went into remission within 4 weeks of steroid therapy,4.9％ (18/369 cases) showed remission within 4 to 8 weeks,and 20.3％ (75/369 cases) were resistant to steroid therapy even after 8 weeks.Onset ages were significantly different(P ＜ 0.01) among the 3 groups [remission within 4 weeks:(5.2 ± 3.3) years old; remission within 4 to 8 weeks:(5.7±3.3) years old ; resistant to therapy after 8 weeks:(6.6 ± 3.8) years old].Simple type nephrotic syndrome was more common in children that responded to steroid therapy within 4 weeks (95.3 ％,263/276 cases) and within 4 to 8 weeks (83.3％,15/18 cases) than in children who were resistant to therapy after 8 weeks(45.3％,34/75 cases).The occurrence rates of acute adrenocortical insufficiency showed a significant difference(P =0.04) among the 3 groups(remission within 4 weeks:4.3％,12/276 cases ; remission within 4 to 8 weeks,2/18 cases:11.1％ ; resistant to therapy after 8 weeks:0).The occurrence rates of poor prognosis were also significantly different(P ＜0.0l) among the 3 groups(remission within 4 weeks:1.4％,4/276 cases ; remission within 4 to 8 weeks:0 ; resistant to therapy after 8 weeks:16.0％,12/75 cases).Conclusions Seventy-one point seven percent of children with idiopathic nephrotic syndrome responded to steroid therapy within 4 weeks.Among children that did not respond within 4 weeks following steroid therapy,14.3％ did respond within 8 weeks,while 85.7％ showed no remission 8 weeks after therapy.The children who responded to steroid therapy within 8 weeks mainly presented as a simple type of nephrotic syndrome and had better prognosis,whereas the children that did not respond within 8 weeks mainly presented with nephritic type nephrotic syndrome and had a poorer prognosis.It should be noted that acute adrenocortical insufficiency was more likely to occur in children that responded to steroid therapy within 4 to 8 weeks.
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