Prognosis, treatment and outcome of childhood mesangiocapillary (membranoproliferative) glomerulonephritis

Prognosis, treatment and outcome of childhood mesangiocapillary (membranoproliferative) glomerulonephritis
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DOI:
10.1093/ndt/gfh484
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发表时间:
2004-11-01
影响因子:
6.1
通讯作者:
Taylor, CM
Taylor, CM
中科院分区:
医学1区
文献类型:
--
作者:
Cansick, JC;Lennon, R;Taylor, CM

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背景资料。儿童系膜毛细血管肾炎(MCGN)的预后因素和预后尚不完全清楚。本研究旨在将肾脏预后与临床和组织病理学变量相关联。我们对患有MCN的儿童进行了双中心的回顾性分析。53例患儿平均年龄8.8岁(13个月~15岁)。随访时间中位数为3.5年(0~17年)。组织学分型:1型31例,2型14例,3型2例,未定型6例。平均肾脏存活时间[终末期肾功能衰竭(ESRF)]预计为12.2年[可信区间(0):9.7-14.6年]。5年和10年肾存活率分别为92%(CI:88-100%)和83%(CI:74-92%)。初发肾病综合征患者的平均肾存活时间为8.9年(CI:7.1年~10.7年),而非肾病综合征患者的平均肾脏存活时间为13.6年(CI:10.8年~16.5年)(P=0.047)。进展为终末期肾衰的患者1年后的平均估计肾小球滤过率为52vs98ml/分钟/1.73m(2),未进展为终末期肾衰竭的患者(P<0.001)。31名儿童(一个中心)首次活检时的慢性损害评分与5年后的不良肾脏结局呈正相关:
Background. Prognostic factors and outcome are incompletely known in childhood mesangiocapillary glomerulonephritis (MCGN). This study aimed to correlate renal outcome with clinical and histopathological variables.Methods. We conducted a two-centre retrospective analysis of children with MCGN.Results. Fifty-three children presented at a mean age of 8.8 years (range: 13 months-15 years). They were followed for a median of 3.5 years (range: 0-17 years). Histological classification identified 31 type 1, 14 type 2, two type 3 and six undetermined type. Mean renal survival [time to end-stage renal failure (ESRF)] was projected to be 12.2 years [confidence interval (0): 9.7-14.6 years]. Five and 10 year renal survival was 92% (CI: 88-100%) and 83% (CI: 74-92%), respectively. Those with nephrotic syndrome at presentation had mean renal survival of 8.9 years (CI: 7.1-10.7 years) vs 13.6 years for those without (CI: 10.8-16.5 years) (P = 0.047). The mean estimated glomerular filtration rate (eGFR) at 1 year in those who progressed to ESRF was 52 vs 98 ml/min/1.73 m(2) in those who did not (P < 0.001). Chronic damage scored on the first biopsy in 31 children (one centre) was positively associated with adverse renal Outcome at 5 years: