单中心110例青少年期起病的儿童原发性肾病综合征临床与病理特征  

Clinical and pathological characteristics of adolescent-onset primary nephrotic syndrome in 110 children in a single center

作　　者：赵三龙 吴红梅 赵非 韩媛 朱春华[1] 成学琴 陈秋霞 黄松明[1] Zhao Sanlong;Wu Hongmei;Zhao Fei;Han Yuan;Zhu Chunhua;Cheng Xueqin;Chen Qiuxia;Huang Songming(Department of Nephrology,Children's Hospital of Nanjing Medical University,Nanjing 210008,China)

机构地区：[1]南京医科大学附属儿童医院肾脏科,南京210008

出　　处：《中华肾脏病杂志》2023年第10期738-744,共7页Chinese Journal of Nephrology

摘　　要：目的分析青少年期(10岁≤年龄≤18岁)起病的儿童原发性肾病综合征(primary nephrotic syndrome,PNS)的临床与病理特征,探讨青少年期起病儿童PNS的肾活检指征。方法该研究为单中心回顾性观察性研究,回顾性分析2004年12月至2022年6月在南京医科大学附属儿童医院肾脏科住院行肾活检,且起病年龄≥10岁的PNS儿童的临床和病理资料。结果共110例患儿被纳入该研究,其中男性76例(69.1%),女性34例(30.9%),起病年龄10岁至14岁9个月。49例(44.5%)伴有血尿,其中肉眼血尿14例(12.7%),镜下血尿35例(31.8%)。起病时,25例(22.7%)合并高血压,19例(17.3%)伴有肾功能不全,4例(3.6%)伴有低补体C3血症。激素敏感型肾病综合征52例(47.3%),激素耐药型肾病综合征58例(52.7%)。病理类型依次为微小病变(minimal change disease,MCD)[52例(47.3%)]、局灶节段性肾小球硬化(focal segmental glomerular sclerosis,FSGS)[22例(20.0%)]、IgA肾病(IgA nephropathy,IgAN)[17例(15.5%)]、膜性肾病(membranous nephropathy,MN)[7例(6.4%)]、系膜增生性肾小球肾炎[5例(4.5%)]、IgM肾病[4例(3.6%)]、膜增生性肾小球肾炎[2例(1.8%)]和C1q肾病[1例(0.9%)]。临床表现为单纯型肾病综合征的44例(40.0%)患儿,病理类型大多为MCD(77.3%);临床表现为肾炎型肾病综合征的66例(60.0%)患儿,病理类型多为非MCD(72.7%),如IgAN、FSGS、MN等。如具有持续性血尿、高血压、肾功能不全及低补体C3血症4种临床表现中的2种及以上时,非MCD的比例进一步增加至92.0%(23/25)。伴肉眼血尿或低补体C3血症者,病理类型均为非MCD。非MCD组血尿(69.0%比17.3%,χ^(2)=29.619,P<0.001)、高血压(31.0%比13.5%,χ^(2)=4.821,P=0.028)及肾功能不全(24.1%比9.6%,χ^(2)=4.047,P=0.044)的发生率均明显高于MCD组。结论10岁以上的青少年原发性肾病综合征如临床表现为单纯型肾病综合征,病理类型大多为MCD,且多数激素敏感,建议先予激素治疗,如出现激素耐药再行肾Objective To analyze the clinical and pathological features of adolescent-onset primary nephrotic syndrome(PNS)in children(10 years≤age≤18 years),so as to explore the renal biopsy indications in adolescent-onset PNS.Methods It was a single-center retrospective observational study.The clinical and pathological data of adolescent-onset PNS(age≥10 years)who underwent renal biopsy in Children's Hospital Affiliated to Nanjing Medical University from December 2004 to June 2022 were analyzed retrospectively.Results A total of 110 children were included in the study,including 76 males(69.1%)and 34 females(30.9%),with the onset age ranging from 10 years to 14 years and 9 months.Forty-nine cases(44.5%)were accompanied by hematuria,including 14 cases(12.7%)of gross hematuria and 35 cases(31.8%)of microscopic hematuria.Twenty-five cases(22.7%)had hypertension,19 cases(17.3%)had renal insufficiency,and 4 cases(3.6%)had low complement C3 at the onset.Fifty-two cases(47.3%)were steroid sensitive nephrotic syndrome and 58 cases(52.7%)were steroid resistant nephrotic syndrome.Biopsy results showed that minimal change disease(MCD)was the most common histopathological subtype(47.3%,52 case),followed by focal segmental glomerulosclerosis(FSGS)in 22 cases(20.0%),IgA nephropathy(IgAN)in 17 cases(15.5%),membranous nephropathy(MN)in 7 cases(6.4%),mesangial proliferative glomerulonephritis in 5 cases(4.5%),IgM nephropathy in 4 cases(3.6%),membranous proliferative glomerulonephritis in 2 cases(1.8%),and C1q nephropathy in 1 case(0.9%).Among 44 children with simple type nephrotic syndrome,the pathological type was mainly MCD(77.3%),and 66 children with nephritic type nephrotic syndrome were mostly non-MCD(72.7%),such as IgAN,FSGS,MN,etc.If there are two or more clinical manifestations of persistent hematuria,hypertension,renal insufficiency or low C3 levels,the proportion of non-MCD would further increase to 92.0%(23/25).The pathological type of patient with gross hematuria or low C3-emia was non-MCD.The frequency of hematuria(69.0%v

关 键 词：肾病综合征 青少年 活组织检查 病理学 临床 

分 类 号：R726.9[医药卫生—儿科]