Huttunen N P
Scand J Clin Lab Invest. 1977 Apr;37(2):133-8. doi: 10.3109/00365517709156067.
The endogenous creatinine clearance and urinary excretion rate of glomerular basement membrane (GBM) antigens were followed from 2 to 19 months in fifteen patients with congenital nephrotic syndrome (CNF). The quantitative examination of renal morphology was made on fourteen of these patients. Creatinine clearance increased during the first few months of life and thereafter gradually decreased. The urinary excretion rate of GBM antigens rose during the course of the disease. The creatinine clearance did not correlate significantly with glomerular fibrosis but it did correlate with tubular atrophy and interstitial fibrosis. The urinary excretion of GBM antigens correlated significantly with glomerular and interstitial fibrosis and with tubular atrophy. It is concluded that there is a clear progress in the disease and the renal histological changes probably are caused by accumulation of GBM material in glomeruli.
对15例先天性肾病综合征（CNF）患者从2个月至19个月跟踪观察其内生肌酐清除率及肾小球基底膜（GBM）抗原的尿排泄率。对其中14例患者进行了肾脏形态学的定量检查。肌酐清除率在生命的最初几个月增加，此后逐渐下降。GBM抗原的尿排泄率在疾病过程中上升。肌酐清除率与肾小球纤维化无显著相关性，但与肾小管萎缩和间质纤维化相关。GBM抗原的尿排泄与肾小球和间质纤维化以及肾小管萎缩显著相关。结论是该疾病有明显进展，肾脏组织学变化可能是由GBM物质在肾小球中的积聚引起的。
