ABSTRACT
To investigate the association of glomerular involvement with biliary atresia, we carried out the following studies: (1) review of the clinical records of 120 patients, (2) histologic study of the kidneys obtained at autopsy from 28 patients, and (3) measurements of circulating immune complexes. Of 90 patients with adequate follow-up information, 40 (44.4%) had hematuria, proteinuria, or both. All the kidney specimens showed a wide variety of mesangial proliferation, and immunoglobulins were present in 23 of 26 cases. There was a good correlation between the glomerular alterations and the period of reduced hepatic function. When IgA was present in the mesangium, IgA2 and secretory components were detected. Elevated serum IgA and circulating IgA-containing immune complex levels were found in patients with prolonged obstructive jaundice. These findings indicate that glomerular alterations may occur subsequently in patients with biliary atresia, and that IgA of intestinal mucosal origin plays some role in the development of these lesions.