Reichel W, Köbberling J, Fischbach H, Scheler F
Klin Wochenschr. 1976 Jan 15;54(2):75-81. doi: 10.1007/BF01468772.
The course of disease of a patient with membranoproliferative glomerulonephritis and partial lipodystrophy is described. The case is further characterized by a deficiency of C3 and C3- activator, by normal values of C4, by evidence of the nephritogenic factor, by raised fibrin degradation products and by an unselective proteinuria. The course of the glomerulonephritis runs parallel to a pronounced susceptibility to infection (at first varicella, tonsillitis and measles, later pneumonia, meningitis, encephalitis and hepatitis). On account of a nephrotic syndrome and an initative impairment of the renal function, a cytostatic treatment was begun, which although raising the C3 level did not influence the further course of the disease. As the patient has a healthy identical twin sister without lipodystrophy, who shows no reduction in C3 and no nephritogenic factor, this case proves that these diseases are acquired and not genetically determined.
本文描述了一名患有膜增生性肾小球肾炎和部分脂肪营养不良患者的病程。该病例的进一步特征为C3和C3激活剂缺乏、C4值正常、存在致肾炎因子证据、纤维蛋白降解产物升高以及非选择性蛋白尿。肾小球肾炎的病程与明显的感染易感性平行（起初为水痘、扁桃体炎和麻疹，后来为肺炎、脑膜炎、脑炎和肝炎）。由于肾病综合征和肾功能的初始损害，开始了细胞抑制治疗，尽管该治疗提高了C3水平，但并未影响疾病的进一步发展。由于患者有一名健康的同卵双胞胎姐妹，无脂肪营养不良，C3无降低且无致肾炎因子，该病例证明这些疾病是后天获得的，而非由基因决定。
