Hellwege H H, Bläker F, Gebbers J O
Monatsschr Kinderheilkd (1902). 1976 Oct;124(10):706-11.
A 5 1/2 year old girl with hypocomplementemic membranoproliferative glomerulonephritis suffered from severe nephrotic syndrome. Despite intensive treatment with corticosteroids and immunosuppressive drugs the clinical state deteriorated. Three years after clinical onset of the disease the girl entered our regular hemodialysis program because of terminal renal insufficiency. After two weeks of intermittent hemodialysis she presented intestinal bleeding, which could not be stopped. One week later complete ileus developed and the child died. Before the onset of melaena no occult blood or mucus could be detected in the faeces. The autopsy revealed a severe ulcerative colitis with pseudopolyposis of the whole colon. In serum specimens still available colonic antigen could be detected by means of immunodiffusion using a rabbit antiserum against fetal colonic extract. Immunofluorescence studies showed granular deposits of immunoglobulins and complement along the glomerular capillary walls suggesting an immunogenesis of the glomerulonephritis by circulating immune complexes. The possibility of an interrelationship in the pathogenesis of both diseases is discussed. It should not be excluded that immune complexes formed in excess of colonic antigen have caused or perpetuated chronic glomerulonephritis.
一名患有低补体血症性膜增生性肾小球肾炎的5岁半女孩患有严重的肾病综合征。尽管使用皮质类固醇和免疫抑制药物进行了强化治疗，但临床状况仍恶化。疾病临床发作三年后，由于终末期肾功能不全，该女孩进入了我们的常规血液透析项目。在进行了两周的间歇性血液透析后，她出现了无法止血的肠道出血。一周后出现了完全性肠梗阻，孩子死亡。在出现黑便之前，粪便中未检测到潜血或黏液。尸检显示为严重的溃疡性结肠炎，整个结肠有假息肉形成。在仍可获得的血清标本中，使用抗胎儿结肠提取物的兔抗血清通过免疫扩散法可检测到结肠抗原。免疫荧光研究显示免疫球蛋白和补体沿肾小球毛细血管壁呈颗粒状沉积，提示肾小球肾炎是由循环免疫复合物引起的免疫发病机制。本文讨论了两种疾病发病机制之间相互关联的可能性。不应排除结肠抗原过量形成的免疫复合物导致或使慢性肾小球肾炎持续存在的可能性。
