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[一名患有I型膜增生性肾小球肾炎男孩的第二次肾活检中检测到显著的上皮下沉积物:病例报告]

[Prominent subepithelial deposits detected on 2nd renal biopsy in a boy with membranoproliferative glomerulonephritis type I: a case report].

作者信息

Motoyama O, Ohshima M, Kawamura S, Iitaka K

机构信息

1st Department of Pediatrics, Toho University School of Medicine, Tokyo, Japan.

出版信息

Nihon Jinzo Gakkai Shi. 1995 Apr;37(4):247-52.

PMID:7602812
Abstract

The first renal biopsy of a 7-year-old boy with microscopic hematuria, hypocomplementemia (CH50 20 U/ml, C3 33 mg/dl, C4 13 mg/dl) and otherwise normal renal function revealed lobular, but not diffuse, expansion of glomerular mesangial areas showing mild to marked mesangial cell proliferation and thickening of the capillary walls with double contours as seen on light microscopic examination. Granular C3 deposition along the capillary wall and in the mesangium was observed on immunofluorescent microscopy. Further, electron microscopy showed subendothelial dense deposits and circumferential mesangial interposition. Thus, he was diagnosed as membranoproliferative glomerulonephritis (MPGN) type I. Thereafter, high-dose, alternate-day administration of prednisolone was started. A few months after treatment, microscopic hematuria disappeared and hypocomplementemia improved. The second renal biopsy at 11 years of age showed markedly decreased mesangial cell proliferation and subendothelial dense deposits. However, C3 deposition still persisted and subepithelial dense deposits had increased in number prominently at this time. Whether MPGN type III is a distinct disease entity or a variant of MPGN type I has not yet been established. The morphological changes in this reported case might justify the notion that MPGN type III is a variant form of MPGN type I.

摘要

一名7岁男孩,有镜下血尿、低补体血症(CH50 20 U/ml,C3 33 mg/dl,C4 13 mg/dl),肾功能其他方面正常,其首次肾活检显示肾小球系膜区呈小叶状而非弥漫性扩张,光镜检查可见系膜细胞轻度至显著增生,毛细血管壁增厚并呈双轨征。免疫荧光显微镜检查观察到沿毛细血管壁和系膜区有颗粒状C3沉积。此外,电镜显示内皮下致密沉积物和系膜周围插入。因此,他被诊断为I型膜增生性肾小球肾炎(MPGN)。此后,开始隔日给予大剂量泼尼松龙。治疗数月后,镜下血尿消失,低补体血症改善。11岁时的第二次肾活检显示系膜细胞增生和内皮下致密沉积物明显减少。然而,此时C3沉积仍然存在,上皮下致密沉积物数量显著增加。III型MPGN是一种独特的疾病实体还是I型MPGN的一种变体尚未确定。该报道病例的形态学变化可能支持III型MPGN是I型MPGN变体形式的观点。

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