中华肾脏病杂志
中華腎髒病雜誌
중화신장병잡지
2011年
11期
797-801
,共5页
肾小球肾炎%补体C3%补体
腎小毬腎炎%補體C3%補體
신소구신염%보체C3%보체
Glomerulonephritis%Complement 3%Complements
目的 研究新命名的C3肾小球肾炎的临床和病理特点,明确血浆补体活化状况及其与临床的相关性.方法 回顾性分析我科1999年1月至2010年6月符合最新C3肾小球肾炎定义的12例患者的临床病理资料,区分为膜增生型(MPGN)和非MPGN型.测定肾活检当天血浆部分补体成分水平以明确补体活化途径,分析补体活化产物与临床的相关性.结果 12例患者中10例为非MPGN型,2例为MPGN型.所有患者均有蛋白尿.10例非MPGN型中2例为肾病水平蛋白尿,6例伴镜下血尿,1例伴肉眼血尿,2例伴肾功能减退.2例MPGN型中1例为肾病水平蛋白尿,伴镜下血尿、高血压、肾功能减退；另1例为单纯蛋白尿,伴高血压,而肾功能正常.所有患者血浆C3均在正常范围,但血浆B因子水平显著低于健康对照组(n=10),Ba、C3a、C4a和C5a水平均显著高于健康对照组.血浆Ba水平与尿蛋白量呈正相关；而C3a、C4a、C5a与尿蛋白量、Scr无相关.非MPGN型C3肾小球肾炎多表现为轻度系膜增生,4例患者出现不同程度新月体.MPGN型则与Ⅰ型MPGN类似,其中1例47.1％肾小球出现新月体.结论 本组C3肾小球肾炎中以非MPGN型为主；临床上多表现为肾炎综合征；病理多表现为轻度系膜增生性肾小球肾炎.补体旁路活化在C3肾小球肾炎中可能发挥重要作用.
目的 研究新命名的C3腎小毬腎炎的臨床和病理特點,明確血漿補體活化狀況及其與臨床的相關性.方法 迴顧性分析我科1999年1月至2010年6月符閤最新C3腎小毬腎炎定義的12例患者的臨床病理資料,區分為膜增生型(MPGN)和非MPGN型.測定腎活檢噹天血漿部分補體成分水平以明確補體活化途徑,分析補體活化產物與臨床的相關性.結果 12例患者中10例為非MPGN型,2例為MPGN型.所有患者均有蛋白尿.10例非MPGN型中2例為腎病水平蛋白尿,6例伴鏡下血尿,1例伴肉眼血尿,2例伴腎功能減退.2例MPGN型中1例為腎病水平蛋白尿,伴鏡下血尿、高血壓、腎功能減退；另1例為單純蛋白尿,伴高血壓,而腎功能正常.所有患者血漿C3均在正常範圍,但血漿B因子水平顯著低于健康對照組(n=10),Ba、C3a、C4a和C5a水平均顯著高于健康對照組.血漿Ba水平與尿蛋白量呈正相關；而C3a、C4a、C5a與尿蛋白量、Scr無相關.非MPGN型C3腎小毬腎炎多錶現為輕度繫膜增生,4例患者齣現不同程度新月體.MPGN型則與Ⅰ型MPGN類似,其中1例47.1％腎小毬齣現新月體.結論 本組C3腎小毬腎炎中以非MPGN型為主；臨床上多錶現為腎炎綜閤徵；病理多錶現為輕度繫膜增生性腎小毬腎炎.補體徬路活化在C3腎小毬腎炎中可能髮揮重要作用.
목적 연구신명명적C3신소구신염적림상화병리특점,명학혈장보체활화상황급기여림상적상관성.방법 회고성분석아과1999년1월지2010년6월부합최신C3신소구신염정의적12례환자적림상병리자료,구분위막증생형(MPGN)화비MPGN형.측정신활검당천혈장부분보체성분수평이명학보체활화도경,분석보체활화산물여림상적상관성.결과 12례환자중10례위비MPGN형,2례위MPGN형.소유환자균유단백뇨.10례비MPGN형중2례위신병수평단백뇨,6례반경하혈뇨,1례반육안혈뇨,2례반신공능감퇴.2례MPGN형중1례위신병수평단백뇨,반경하혈뇨、고혈압、신공능감퇴；령1례위단순단백뇨,반고혈압,이신공능정상.소유환자혈장C3균재정상범위,단혈장B인자수평현저저우건강대조조(n=10),Ba、C3a、C4a화C5a수평균현저고우건강대조조.혈장Ba수평여뇨단백량정정상관；이C3a、C4a、C5a여뇨단백량、Scr무상관.비MPGN형C3신소구신염다표현위경도계막증생,4례환자출현불동정도신월체.MPGN형칙여Ⅰ형MPGN유사,기중1례47.1％신소구출현신월체.결론 본조C3신소구신염중이비MPGN형위주；림상상다표현위신염종합정；병리다표현위경도계막증생성신소구신염.보체방로활화재C3신소구신염중가능발휘중요작용.
Objective To study the clinicopathological features of C3 glomerulonephritis and the associations between plasma complement fragments level and clinical manifestations.Methods The clinical and pathological data of the 12 patients with C3 glomerulonephritis in our division from January 1999 to June 2010 were analyzed retrospectively.Concentrations of plasma factor B,Ba,C3,C3a,C4a and C5a were detected by commercial available sandwich ELISA kits on the day of renal biopsy.Results Ten of the 12 patients were C3 glomerulonephritis without MPGN,and the rest 2 were C3 glomerulonephritis with MPGN.All the patients presented proteinuria.Two of the 10 C3 glomerulonephritis patients without MPGN presented nephrotic range proteinuria,6 with microhematuria,1 with gross hematuria,and 2 with renal insufficiency.One of the 2 C3 glomerulonephritis patients with MPGN presented nephrotic range proteinuria,accompanied by microhematuria,hypertension and renal insufficiency.The other patient showed moderate proteinuria with normal renal function.Most of C3 glomerulonephritis patients without MPGN showed mild mesangial proliferative glomerulonephritis,and 4/10 patients had various degree cresentic formation.One C3 glomerulonephritis patient with MPGN had 47.1％ cresentic formation.The concentration of plasma C3 in C3 glomerulonephritis patients was normal,while the plasma factor B was significantly decreased,and the concentrations of plasma Ba,C3a,C4a and C5a were significantly elevated.The concentration of plasma Ba was positively correlated with the proteinuria level,while the concentrations of plasma C3a,C4a and C5a were not correlated with the levels of proteinuria or plasma creatinine.Conclusions Majority of these 12 patients were C3 glomerulonephritis without MPGN,manifests as nephritic syndrome clinically and mild mesangial proliferative glomerulonephritis histopathologically.Complement activation via alternative pathway may play an important role in the pathogenesis of C3 glomerulonephritis.
