Higginbottom M C, Griswold W R, Jones K L, Vasquez M D, Mendoza S A, Wilson C B
Pediatrics. 1979 Dec;64(6):929-34.
Three children with renal disease, hypertension, and the Cockayne syndrome were evaluated. All patients had severe hypertension; peripheral vein renin was elevated in two patients. Renal biopsy specimens from two patients were studied by light microscopy, electron microscopy, and immunofluoresence. Immunohistologic studies demonstrated deposits of immunoglobulin and complement in the vessels and glomeruli of the first patient; deposits of immunoglobulin and complement were seen in the glomeruli of the third patient. Also electron-dense deposits were seen in the glomerular basement membrane of the third patient. Circulating immune complexes were detected by the Raji cell and Clq binding techniques in this child as well. Both hypertension and renal disease are frequent complications of the Cockayne syndrome.
对三名患有肾病、高血压和科凯恩综合征的儿童进行了评估。所有患者均患有严重高血压；两名患者外周静脉肾素升高。对两名患者的肾活检标本进行了光镜、电镜和免疫荧光检查。免疫组织学研究显示，第一名患者的血管和肾小球中有免疫球蛋白和补体沉积；第三名患者的肾小球中可见免疫球蛋白和补体沉积。第三名患者的肾小球基底膜中也可见电子致密沉积物。通过Raji细胞和Clq结合技术在该患儿中也检测到循环免疫复合物。高血压和肾病都是科凯恩综合征的常见并发症。
