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本文报告肺-肾综合征7例,5例行双侧肾切除,3例肾切除后成功地进行了肾移植并进行了长期随访。作者提出了肾移植的时机及肾移植前双侧肾切除的论据。肺-肾综合征最初的含义为肾小球肾炎合并肺出血。其后证明抗肾小球基底膜抗体(简称AGBMA)在肾小球肾炎发病中的病因学作用。目前以肾小球肾炎、肺泡内出血及AGBMA的存在三联征作为诊断本病的依据。早期报告本病预后不良,可能由于只将暴发型病例诊断为肺-肾综合征所致。随着AGBMA敏感试验的发展。对于肾脏受累极轻又无肺部症状的病例亦能辨认。 7例中男性5例,女性2例,年龄14～21岁。
This article reports 7 cases of pulmonary-renal syndrome, 5 cases of bilateral nephrectomy, 3 cases of nephrectomy after kidney transplantation and long-term follow-up. The authors propose the timing of kidney transplantation and bilateral nephrectomy before kidney transplantation. Lung-kidney syndrome was originally defined as glomerulonephritis with pulmonary hemorrhage. Subsequent demonstration of anti-glomerular basement membrane antibody (AGBMA) etiology in the pathogenesis of glomerulonephritis. At present, glomerulonephritis, alveolar hemorrhage and the presence of AGBMA triad as the basis for the diagnosis of the disease. Early reports of poor prognosis of the disease may be due to only the outbreak of cases diagnosed as lung-kidney syndrome. With the development of AGBMA-sensitive tests. For patients with minimal renal involvement and no symptoms of lung can be identified. Of the 7 patients, 5 were male and 2 were female, ranging in age from 14 to 21 years.