中华儿科杂志
中華兒科雜誌
중화인과잡지
Chinese Journal of Pediatrics
2015年
9期
670-675
,共6页
王大海%王芳%丁洁%肖慧捷%钟旭辉%刘晓宇
王大海%王芳%丁潔%肖慧捷%鐘旭輝%劉曉宇
왕대해%왕방%정길%초혜첩%종욱휘%류효우
肾小球肾炎,IgA%儿童%预后
腎小毬腎炎,IgA%兒童%預後
신소구신염,IgA%인동%예후
Glomerulonephritis,IgA%Child%Prognosis
目的 研究伴新月体形成和(或)表现为急进性肾炎(RPGN)的原发性IgA肾病患儿的临床病理表现、治疗方法和预后.方法 以2000-2014年北京大学第一医院儿科肾穿刺活检确诊的IgA肾病,肾活检时年龄≤18岁患儿作为纳人标准,并排除继发于系统性红斑狼疮、过敏性紫癜或肝脏疾病的IgA肾病患儿.根据临床表现分为RPGN组和非RPGN组,根据肾活检病理分为有新月体组和无新月体组以及新月体性IgA肾病组和非新月体性IgA肾病组.分别进行组间临床表现、病理特点、治疗和预后的比较.结果 共纳入265例患儿,RPGN组10例(3.8％),其余为非RPGN组;有新月体组151例,无新月体组114例;新月体性IgA肾病组19例,非新月体性IgA肾病组132例.RPGN组与非RPGN组相比,肾活检时肉眼血尿发生率(70.0％比29.4％)、血肌酐水平、校正肌酐清除率水平、病理为新月体性IgA肾病发生率(50.0％比5.5％)、随访肉眼血尿发生率(30.0％比4.3％)、随访血肌酐水平、随访校正肌酐清除率水平差异有统计学意义(P＜0.05),RPGN组采用甲泼尼龙冲击、血液净化治疗的例数比非RPGN组更多(P＜0.05);有新月体组和无新月体组相比,肾活检时肉眼血尿发生率(37.1％比22.8％)、血肌酐水平、校正肌酐清除率水平、24 h尿蛋白定量水平、随访肉眼血尿发生率(7.9％比1.8％),差异有统计学意义(P＜0.05),有新月体组应用甲泼尼龙冲击、口服激素、环磷酰胺冲击治疗的例数较无新月体组更多(P＜0.05);新月体性IgA肾病组和非新月体性IgA肾病组相比,肾活检时RPGN发生率(26.3％比1.5％)、血肌酐水平、24 h尿蛋白定量水平、随访血肌酐水平差异有统计学意义(P＜0.05),新月体性IgA肾病组应用甲泼尼龙冲击、血液净化治疗的例数较无新月体组更多(P＜0.05).随访时,临床表现为RPGN且病理为新月体性IgA肾病者有20.0％肾功能恢复正常,病理表现为新月体性IgA肾病但不伴RPGN者有71.4％肾功能恢复正常,临床表现为RPGN伴非新月体性IgA肾病者有80.0％肾功能恢复正常,病理有新月体形成但尚未达到新月体性IgA肾病者有87.5％肾功能恢复正常.结论 在伴有新月体形成和(或)表现为急进性肾炎的原发性IgA肾病患儿中,临床表现为RPGN且病理表现为新月体性IgA肾病者临床表现最重,预后最差;病理表现为新月体形成,但尚未达新月体性IgA肾病者临床表现最轻,预后最佳.
目的 研究伴新月體形成和(或)錶現為急進性腎炎(RPGN)的原髮性IgA腎病患兒的臨床病理錶現、治療方法和預後.方法 以2000-2014年北京大學第一醫院兒科腎穿刺活檢確診的IgA腎病,腎活檢時年齡≤18歲患兒作為納人標準,併排除繼髮于繫統性紅斑狼瘡、過敏性紫癜或肝髒疾病的IgA腎病患兒.根據臨床錶現分為RPGN組和非RPGN組,根據腎活檢病理分為有新月體組和無新月體組以及新月體性IgA腎病組和非新月體性IgA腎病組.分彆進行組間臨床錶現、病理特點、治療和預後的比較.結果 共納入265例患兒,RPGN組10例(3.8％),其餘為非RPGN組;有新月體組151例,無新月體組114例;新月體性IgA腎病組19例,非新月體性IgA腎病組132例.RPGN組與非RPGN組相比,腎活檢時肉眼血尿髮生率(70.0％比29.4％)、血肌酐水平、校正肌酐清除率水平、病理為新月體性IgA腎病髮生率(50.0％比5.5％)、隨訪肉眼血尿髮生率(30.0％比4.3％)、隨訪血肌酐水平、隨訪校正肌酐清除率水平差異有統計學意義(P＜0.05),RPGN組採用甲潑尼龍遲擊、血液淨化治療的例數比非RPGN組更多(P＜0.05);有新月體組和無新月體組相比,腎活檢時肉眼血尿髮生率(37.1％比22.8％)、血肌酐水平、校正肌酐清除率水平、24 h尿蛋白定量水平、隨訪肉眼血尿髮生率(7.9％比1.8％),差異有統計學意義(P＜0.05),有新月體組應用甲潑尼龍遲擊、口服激素、環燐酰胺遲擊治療的例數較無新月體組更多(P＜0.05);新月體性IgA腎病組和非新月體性IgA腎病組相比,腎活檢時RPGN髮生率(26.3％比1.5％)、血肌酐水平、24 h尿蛋白定量水平、隨訪血肌酐水平差異有統計學意義(P＜0.05),新月體性IgA腎病組應用甲潑尼龍遲擊、血液淨化治療的例數較無新月體組更多(P＜0.05).隨訪時,臨床錶現為RPGN且病理為新月體性IgA腎病者有20.0％腎功能恢複正常,病理錶現為新月體性IgA腎病但不伴RPGN者有71.4％腎功能恢複正常,臨床錶現為RPGN伴非新月體性IgA腎病者有80.0％腎功能恢複正常,病理有新月體形成但尚未達到新月體性IgA腎病者有87.5％腎功能恢複正常.結論 在伴有新月體形成和(或)錶現為急進性腎炎的原髮性IgA腎病患兒中,臨床錶現為RPGN且病理錶現為新月體性IgA腎病者臨床錶現最重,預後最差;病理錶現為新月體形成,但尚未達新月體性IgA腎病者臨床錶現最輕,預後最佳.
목적 연구반신월체형성화(혹)표현위급진성신염(RPGN)적원발성IgA신병환인적림상병리표현、치료방법화예후.방법 이2000-2014년북경대학제일의원인과신천자활검학진적IgA신병,신활검시년령≤18세환인작위납인표준,병배제계발우계통성홍반랑창、과민성자전혹간장질병적IgA신병환인.근거림상표현분위RPGN조화비RPGN조,근거신활검병리분위유신월체조화무신월체조이급신월체성IgA신병조화비신월체성IgA신병조.분별진행조간림상표현、병리특점、치료화예후적비교.결과 공납입265례환인,RPGN조10례(3.8％),기여위비RPGN조;유신월체조151례,무신월체조114례;신월체성IgA신병조19례,비신월체성IgA신병조132례.RPGN조여비RPGN조상비,신활검시육안혈뇨발생솔(70.0％비29.4％)、혈기항수평、교정기항청제솔수평、병리위신월체성IgA신병발생솔(50.0％비5.5％)、수방육안혈뇨발생솔(30.0％비4.3％)、수방혈기항수평、수방교정기항청제솔수평차이유통계학의의(P＜0.05),RPGN조채용갑발니룡충격、혈액정화치료적례수비비RPGN조경다(P＜0.05);유신월체조화무신월체조상비,신활검시육안혈뇨발생솔(37.1％비22.8％)、혈기항수평、교정기항청제솔수평、24 h뇨단백정량수평、수방육안혈뇨발생솔(7.9％비1.8％),차이유통계학의의(P＜0.05),유신월체조응용갑발니룡충격、구복격소、배린선알충격치료적례수교무신월체조경다(P＜0.05);신월체성IgA신병조화비신월체성IgA신병조상비,신활검시RPGN발생솔(26.3％비1.5％)、혈기항수평、24 h뇨단백정량수평、수방혈기항수평차이유통계학의의(P＜0.05),신월체성IgA신병조응용갑발니룡충격、혈액정화치료적례수교무신월체조경다(P＜0.05).수방시,림상표현위RPGN차병리위신월체성IgA신병자유20.0％신공능회복정상,병리표현위신월체성IgA신병단불반RPGN자유71.4％신공능회복정상,림상표현위RPGN반비신월체성IgA신병자유80.0％신공능회복정상,병리유신월체형성단상미체도신월체성IgA신병자유87.5％신공능회복정상.결론 재반유신월체형성화(혹)표현위급진성신염적원발성IgA신병환인중,림상표현위RPGN차병리표현위신월체성IgA신병자림상표현최중,예후최차;병리표현위신월체형성,단상미체신월체성IgA신병자림상표현최경,예후최가.
Objective IgA nephropathy is the most common type of glomerulonephritis in the world.Its clinical and pathological manifestations vary.A few of the patients with IgA nephropathy present with rapidly progressive glomerulonephritis (RPGN) and/or crescent formation.Their conditions are serious and acute,but there are few reports on their characteristics,treatment and outcome.This study aimed to analyze the clinicalopathological features,treatment and prognosis of primary IgA nephropathy in children,to provide a reference for clinical diagnosis and treatment.Method A retrospective study was conducted in children with primary IgA nephropathy with crescent formation and/or rapidly progressive glomerulonephritis admitted to our department from 2000 to 2014.The patients meeting the inclusion and exclusion criteria were included.Patients were divided into RPGN group and non-RPGN group according to the clinical manifestations,crescent formation group and non-crescent group,crescentic IgA nephropathy group and noncrescentic IgA nephropathy group according to renal biopsy.Their clinical manifestations and pathological features,treatment and prognosis were compared.Result A total of 265 patients were recruited,10 patients (3.8％) had RPGN,151 patients (57.0％) had crescent formation,19 cases (7.2％) showed crescentic IgA nephropathy.Compared with non-RPGN group,RPGN group showed more gross hematuria,higher serum creatinine,lower creatinine clearance correction at biopsy and follow-up,and more crescentic IgA nephropathy (P ＜ 0.05).The percent of patients who received methylprednisolone pulse and blood purification therapy in RPGN group is higher than that of non-RPGN group (P ＜ 0.05).Compared with noncrescent group,crescent formation group showed more gross hematuria at biopsy and follow-up,higher serum creatinine at biopsy,lower creatinine clearance correction,more 24-hour urinary protein at biopsy and higher serum creatinine at follow-up (P ＜ 0.05).The percentage of patients received more methylprednisolone pulse,oral steroids,cyclophosphamide pulse in crescent formation group was higher than that of noncrescent group (P ＜ 0.05).Compared with non-crescentic IgA nephropathy group,crescentic IgA nephropathy group showed more RPGN percent,higher serum creatinine,more 24-hour urinary protein at biopsy (P ＜0.05).The percentage of patients who received more methylprednisolone pulse and blood purification therapy in crescentic IgA nephropathy group was more than non-crescentic IgA nephropathy group (P ＜ 0.05).At follow-up,20.0％ of the patients with RPGN and crescent nephritis returned to normal renal function and the percent of crescent glomerulonephritis but not RPGN was 71.4％,RPGN but not crescent glomerulonephritis was 80.0％,crescent formation but not crescent nephritis was 87.5％.Conclusion In primary IgA nephropathy with crescent formation and/or rapidly progressive glomerulonephritis,the patients with both RPGN and crescentic IgA nephropathy showed the worst clinical manifestations,its prognosis was worst while the patients with crescent formation showed the mildest clinical manifestations and best prognosis.
