Hirooka M, Hirota M
Int J Pediatr Nephrol. 1985 Apr-Jun;6(2):145-50.
A 19-year-old girl having a sporadic form of idiopathic multicentric osteolysis with nephropathy is described. She was first diagnosed as having sporadic carpotarsal osteolysis at the age of 6. Her proteinuria was first detected at 15 years of age and renal biopsy specimens obtained at the same age were compatible with those of focal segmental glomerulosclerosis with interstitial fibrosis and tubular atrophy. Immunofluorescence microscopy revealed negative deposits of Ig G, Ig A and C3 but a trace amount of mesangial Ig M in unsclerosed segments of the glomeruli. Electronmicroscopy showed no specific changes. Renal vascular changes such as proliferation of vascular intima and medial hypertrophy were not demonstrated in the present case. It is our conclusion that focal segmental glomerulosclerosis is part of the spectrum of nephropathy in the sporadic form of idiopathic multicentric osteolysis.
本文描述了一名患有散发性特发性多中心骨质溶解症合并肾病的19岁女孩。她6岁时首次被诊断为散发性腕跗关节骨质溶解症。蛋白尿于15岁时首次被发现，同年获取的肾活检标本符合局灶节段性肾小球硬化伴间质纤维化和肾小管萎缩的表现。免疫荧光显微镜检查显示，肾小球未硬化节段中IgG、IgA和C3沉积阴性，但有微量系膜IgM。电子显微镜检查未发现特异性改变。本病例未显示肾血管变化，如血管内膜增生和中层肥厚。我们的结论是，局灶节段性肾小球硬化是散发性特发性多中心骨质溶解症肾病谱的一部分。
