Andreoli S P, Yum M N, Bergstein J M
Am J Nephrol. 1986;6(1):28-33. doi: 10.1159/000167049.
Seventeen children with IgA nephropathy were grouped according to the absence (group I, n = 10) or presence (group II, n = 7) of glomerular basement membrane (GBM) deposition of IgA to determine whether GBM deposition of IgA correlated with laboratory or pathologic data at diagnosis or clinical status at follow-up. Children in group II had significantly (p less than 0.01) more proteinuria at diagnosis than children in group I. The percentage of glomeruli demonstrating crescent formation was significantly (p less than 0.05) higher in group II biopsies. Chronic changes of fibrous crescents, segmental sclerosis, global obsolescence, tubular atrophy, and interstitial fibrosis were also significantly (p less than 0.001) more common in group II biopsies. After a mean follow-up period of 2 years, all children in group II have persistent proteinuria of more than 1 g/24 h, and 3 of 5 have renal insufficiency (2 require dialysis). In contrast, 2 of 9 group I children have proteinuria exceeding 1 g/24 h, and only 1 has renal insufficiency. We conclude that, as compared to children with IgA localized to the mesangium, children with IgA nephropathy and GBM deposition of IgA have a higher urinary protein excretion at the time of diagnosis, more severe histologic alterations including a greater percentage of glomeruli demonstrating crescent formation, more chronic changes of segmental or global sclerosis, tubular atrophy, and interstitial fibrosis. Such children usually have persistent proteinuria and are more likely to develop progressive renal disease.
17例IgA肾病患儿根据肾小球基底膜(GBM)有无IgA沉积分为两组(I组,无GBM沉积,n = 10;II组,有GBM沉积,n = 7),以确定IgA在GBM上的沉积是否与诊断时的实验室或病理数据或随访时的临床状况相关。II组患儿诊断时的蛋白尿明显(p<0.01)多于I组患儿。II组活检中显示新月体形成的肾小球百分比明显(p<0.05)更高。II组活检中纤维性新月体、节段性硬化、球性废弃、肾小管萎缩和间质纤维化等慢性改变也明显(p<0.001)更常见。平均随访2年后,II组所有患儿均持续存在蛋白尿>1g/24h,5例中有3例出现肾功能不全(2例需要透析)。相比之下,I组9例患儿中有2例蛋白尿超过1g/24h,只有1例出现肾功能不全。我们得出结论,与IgA局限于系膜区的患儿相比,IgA肾病且有IgA在GBM沉积的患儿在诊断时尿蛋白排泄更高,组织学改变更严重,包括显示新月体形成的肾小球百分比更高,节段性或球性硬化、肾小管萎缩和间质纤维化等慢性改变更多。此类患儿通常有持续性蛋白尿,更易发展为进行性肾病。