Dharnidharka V R, Takemoto C, Ewenstein B M, Rosen S, Harris H W
Department of Medicine, Children's Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA.
Pediatr Nephrol. 1998 Oct;12(8):654-7. doi: 10.1007/s004670050522.
Children with hemophilia B who receive exogenous factor IX infusions may rarely develop inhibitors to the exogenous factor IX and require desensitization. Nephrotic syndrome has recently been described in some of these children. We report the renal clinicopathological findings in a child with severe factor IX deficiency, requiring induction of an immune tolerance protocol, who developed nephrotic syndrome. Renal biopsy revealed peripheral capillary wall thickening and a spike appearance consistent with membranous glomerulonephritis. Electron microscopy showed prominent deposits throughout the thickness of the basement membrane. Factor IX dose reduction was accompanied by reversal of the child's nephrotic syndrome without relapses.
接受外源性凝血因子IX输注的B型血友病患儿很少会对外源性凝血因子IX产生抑制剂并需要脱敏治疗。最近在其中一些患儿中发现了肾病综合征。我们报告了一名患有严重凝血因子IX缺乏症、需要进行免疫耐受方案诱导治疗且发生了肾病综合征的患儿的肾脏临床病理结果。肾活检显示外周毛细血管壁增厚以及出现与膜性肾小球肾炎一致的钉突样表现。电子显微镜检查显示在整个基底膜厚度上有明显沉积物。凝血因子IX剂量减少伴随着患儿肾病综合征的逆转且未复发。
