Martini A, Binda S, Mariani G, Scotta M S, Ruberto G
Acta Paediatr Scand. 1981;70(3):435-9. doi: 10.1111/j.1651-2227.1981.tb16581.x.
An 8-year-old girl with microhematuria of recent onset developed a picture of pulmonary hemosiderosis in the space of 2 months. Some months later while pulmonary involvement was improving severe extracapillary glomerulonephritis developed. Circulating anti-glomerular basement membrane antibody was detected and linear deposition of IgG along glomerular basement membrane was observed. A diagnosis of Goodpasture's syndrome was made and treatment was started with prednisone, cyclophosphamide and periodic plasmapheresis with complete progressive disappearance of circulating anti-glomerular basement membrane antibody. After suspension of plasmapheresis despite immunosuppressive therapy and lack of evidence for circulating anti-glomerular basement membrane antibody, the child went into terminal renal failure. The natural history of the disease in this case and the results of treatment are discussed. To our knowledge this is the first case of Goodpasture's syndrome reported in childhood with demonstration of the presence of anti-glomerular basement membrane antibody.
一名近期出现微血尿的8岁女孩在2个月内发展为肺含铁血黄素沉着症。几个月后，当肺部病变正在改善时，严重的毛细血管外肾小球肾炎出现了。检测到循环抗肾小球基底膜抗体，并观察到IgG沿肾小球基底膜呈线性沉积。诊断为Goodpasture综合征，并开始用泼尼松、环磷酰胺治疗以及定期进行血浆置换，循环抗肾小球基底膜抗体完全逐渐消失。尽管进行了免疫抑制治疗且没有循环抗肾小球基底膜抗体的证据，但在停止血浆置换后，该患儿进入终末期肾衰竭。本文讨论了该病例中疾病的自然史及治疗结果。据我们所知，这是儿童期报告的首例Goodpasture综合征病例，并证实了抗肾小球基底膜抗体的存在。
