Am J Kidney Dis. 1986 Feb;7(2):115-24.
This retrospective multicenter study has revealed 68 children with membranous glomerulonephropathy (MGN), accounting for 5.5% of all patients (pts) with nephrotic syndrome who were biopsied during the period of study. The total group includes 54 pts with idiopathic MGN (IMGN), 10 with lupus MGN (LMGN), and 4 who were ANA-positive but had no other features of systemic lupus erythematosus (SLE). Renal biopsies were examined by light (LM), immunofluorescent (IF), and electron microscopy (EM), and the findings compared with clinical features within and between the IMGN and LMGN groups. The LMGN pts tended to be more frequently female and older, and differed significantly from the IMGN pts by being more frequently hypocomplementemic (70% v 4%, p less than 0.001), and having higher levels of total serum protein (6.6 +/- 1.2 v 5.1 +/- 1.0, p less than 0.03), and serum albumin (2.9 +/- 0.7 v 2.2 +/- 0.8, p = 0.03). There was no significant difference in glomerular filtration rate (GFR) or the frequency of hypertension or hematuria between the two groups. Pathologic features that differed between LMGN and IMGN included diffuse mesangial hypercellularity (44% v 7%, p = 0.01), glomerular electron-dense subendothelial deposits (78% v 13%, p = 0.001), and mesangial deposits (100% v 31%, p = 0.002). The frequency of focal mesangial hypercellularity and of mesangial sclerosis, tubulointerstitial disease, and frequency of glomerular immunoreactants did not differ between the groups. Limited follow-up of the pts has revealed no difference in outcome between the IMGN and LMGN pts. We conclude that differentiation between IMGN and LMGN in children, as in adults, may be difficult on pathologic grounds alone and that the separation can only be made by established clinical and laboratory criteria of SLE.
这项回顾性多中心研究发现了68例膜性肾小球肾病(MGN)患儿,占研究期间接受肾活检的所有肾病综合征患者的5.5%。总共有54例特发性MGN(IMGN)患者、10例狼疮性MGN(LMGN)患者以及4例ANA阳性但无其他系统性红斑狼疮(SLE)特征的患者。对肾活检组织进行了光镜(LM)、免疫荧光(IF)和电子显微镜(EM)检查,并将检查结果与IMGN组和LMGN组内部及两组之间的临床特征进行比较。LMGN患者女性更为常见且年龄更大,与IMGN患者相比,LMGN患者低补体血症更为常见(70%对4%,p<0.001),总血清蛋白水平更高(6.6±1.2对5.1±1.0,p<0.03),血清白蛋白水平更高(2.9±0.7对2.2±0.8,p = 0.03)。两组间肾小球滤过率(GFR)、高血压或血尿的发生率无显著差异。LMGN和IMGN之间不同的病理特征包括弥漫性系膜细胞增多(44%对7%,p = 0.01)、肾小球电子致密内皮下沉积物(78%对13%,p = 0.001)和系膜沉积物(100%对31%,p = 0.002)。两组间局灶性系膜细胞增多、系膜硬化、肾小管间质疾病的发生率以及肾小球免疫反应物的发生率无差异。对患者的有限随访显示,IMGN和LMGN患者的预后无差异。我们得出结论,儿童IMGN和LMGN的鉴别,如同成人一样,仅基于病理依据可能较为困难,只能通过已确立的SLE临床和实验室标准来区分。