Loughlin G M, Taussig L M, Murphy S A, Strunk R C, Kohnen P W
J Pediatr. 1978 Aug;93(2):181-4. doi: 10.1016/s0022-3476(78)80491-0.
Two female children whose clinical presentations and renal light-microscopic findings were consistent with Goodpasture syndrome are described. Immunopathologic studies demonstrated granular deposition of immunoglobulins and complement, suggesting that the renal disease was mediated by circulating immune complexes and not by anti-glomerular basement membrane antibody. Anti-GBM antibody was absent in both patients. These patients represent the first report in children of idiopathic nephritis due to immune complexes with associated pulmonary hemorrhage. The findings raise some doubt as to the accuracy of previous reports of Goodpasture syndrome in children, and also demonstrate the diagnostic and therapeutic importance of evaluation the renal immunopathology in the child with nephritis and pulmonary hemorrhage.
本文描述了两名女童,她们的临床表现和肾脏光学显微镜检查结果与Goodpasture综合征相符。免疫病理学研究显示免疫球蛋白和补体呈颗粒状沉积,提示肾脏疾病是由循环免疫复合物介导,而非抗肾小球基底膜抗体。两名患者均未检测到抗GBM抗体。这些患者代表了首例关于儿童免疫复合物相关性特发性肾炎伴肺出血的报道。这些发现对既往儿童Goodpasture综合征报道的准确性提出了质疑,同时也证明了评估肾炎伴肺出血患儿肾脏免疫病理学在诊断和治疗中的重要性。