Levy M, Gagnadoux M F, Beziau A, Habib R
Clin Nephrol. 1978 Oct;10(4):158-65.
The renal biopsy of a 3-year old boy with complete Fanconi syndrome showed the association of a membranous glomerulonephritis with severe tubulointerstial changes. Immunofluorescence microscopy disclosed linear and granular deposits of Ig along tubular basement membranes. The presence of anti-tubular basement membrane antibodies in the patient's serum was demonstrated by indirect immunofluorescence and radioimmunoassay. The child also developed pulmonary involvement associated with episodes of acute anemia. Anti-alveolar basement membrane antibodies were detected by indirect immunofluorescence. The present case is the first reported example of auto-immune disease characterized by the presence of anti-tubular and alveolar basement membrane antibodies associated with an immune complex glomerulonephritis.
一名患有完全型范科尼综合征的3岁男孩的肾活检显示，膜性肾小球肾炎与严重的肾小管间质改变相关。免疫荧光显微镜检查发现沿肾小管基底膜有Ig的线性和颗粒状沉积。通过间接免疫荧光和放射免疫测定法证实患者血清中存在抗肾小管基底膜抗体。该患儿还出现了与急性贫血发作相关的肺部受累情况。通过间接免疫荧光检测到抗肺泡基底膜抗体。本病例是首例报道的以抗肾小管和肺泡基底膜抗体伴免疫复合物性肾小球肾炎为特征的自身免疫性疾病。
