Ziółkowska Helena, Adamczuk Dominika, Leszczyńska Beata, Roszkowska-Blaim Maria
Department of Pediatrics and Nephrology, Medical University of Warsaw.
Pol Merkur Lekarski. 2009 Apr;26(154):301-5.
The aim of the study was assessment of the frequency of glomerular diseases (GD) as a cause of end-stage renal disease (ESRD) in children.
Retrospectively, causes of ESRD, frequency, duration of the disease until diagnosis of ESRD in 195 children treated in 1973-2008 were analysed.
Among children with ESRD, GD were diagnosed in 94 (48.2%)--mean age 10.9 +/- 4.3 years, congenital abnormalities of urinary tract in 61 (31.2%) aged 10.6 +/- 4.3 years and other causes in 40 (20.5%). Among 94 children with GD the 49 (52%) patients had primary glomerulonephritis (GN): 24--crescentic GN, 9--focal segmental glomerulosclerosis, 8--mesangioproliferative GN, 5--IgA nephropathy, 3--membranoproliferative GN. In 37 (39.5%) the causes of ESRD were secondary glomerulopathies: 20--amyloidosis, 10--hemolytic-uremic syndrome, 6--Schönlein-Henoch nephropathy, 1--Wegener granulomatosis. In 8 (8.5%) patients causes of ESRD were: Alport syndrome in 2, congenital nephrotic syndrome in 1 and in 5 type of glomerulopathy was unknown. Time between diagnosis of nephropathy and start of dialysis was 0-13.75 years, median 1.1 in patients with GD and was significantly shorter (p<0.003) than this time in the group with congenital abnormalities of the urinary tract (0-14.9 years; median 3.83). There was no difference between primary GN and secondary glomerulopathies.
Chronic glomerulopathies were the most frequent cause of ESRD in investigated group. Time between diagnosis of chronic renal disease and initiation of renal replacement therapy was significantly shorter in children with glomerulopathies than with congenital abnormalities of urinary tract.
本研究的目的是评估肾小球疾病(GD)作为儿童终末期肾病(ESRD)病因的频率。
回顾性分析了1973年至2008年接受治疗的195名儿童ESRD的病因、频率以及疾病至ESRD诊断的持续时间。
在患有ESRD的儿童中,94例(48.2%)诊断为GD,平均年龄10.9±4.3岁;61例(31.2%)为尿路先天性异常,年龄10.6±4.3岁;40例(20.5%)为其他病因。在94例患有GD的儿童中,49例(52%)患有原发性肾小球肾炎(GN):24例为新月体性GN,9例为局灶节段性肾小球硬化,8例为系膜增生性GN,5例为IgA肾病,3例为膜增生性GN。37例(39.5%)ESRD的病因是继发性肾小球病:20例为淀粉样变性,10例为溶血尿毒综合征,6例为过敏性紫癜性肾病,1例为韦格纳肉芽肿病。8例(8.5%)患者ESRD的病因是:2例为Alport综合征,1例为先天性肾病综合征,5例肾小球病类型不明。肾病诊断至开始透析的时间为0至13.75年,GD患者中位数为1.1年,显著短于(p<0.003)尿路先天性异常组(0至14.9年;中位数3.83)。原发性GN和继发性肾小球病之间无差异。
慢性肾小球病是研究组中ESRD最常见的病因。慢性肾病诊断至开始肾脏替代治疗的时间,患有肾小球病的儿童明显短于尿路先天性异常的儿童。