Sakano T, Hamasaki T, Mori M, Ohta T, Ueda K, Ishigame K, Kawamura M, Takeda N
Department of Paediatrics, Hiroshima University School of Medicine, Japan.
Eur J Pediatr. 1988 Jun;147(5):516-7. doi: 10.1007/BF00441978.
A 14-year-old boy had persistent haematuria along with complete C7 deficiency. No significant changes in glomeruli and tubules were found in a renal biopsy specimen by light microscopy and immunofluorescence gave negative results for immune deposits. Electron microscopic examination demonstrated an attenuation of the glomerular capillary basement membrane without lamination and a diagnosis of thin basement membrane disease was made. It would be difficult to conclude that patients with C7 deficiency were predisposed to develop glomerulonephritis caused by immunologic aberrations. A family study failed to provide evidence of an association of C7 deficiency and thin basement membrane disease.
一名14岁男孩患有持续性血尿并伴有C7完全缺乏。肾活检标本经光学显微镜检查,肾小球和肾小管未发现明显变化,免疫荧光检查免疫沉积物呈阴性。电子显微镜检查显示肾小球毛细血管基底膜变薄且无分层,诊断为薄基底膜病。很难得出结论认为C7缺乏患者易患由免疫异常引起的肾小球肾炎。一项家族研究未能提供C7缺乏与薄基底膜病相关联的证据。