Variability of the Antigenicity of the Glomerular Basement Membrane in Nail-patella Syndrome

摘要

The difficulty of diagnosing Alport-type nephritis, and the possibility of altered antigenicity of the glomerular basement membrane in Alport-type nephritis is discussed. Attention is focused on the monoclonal antibody MCA-PI as a specific tool in studying the Goodpasture antigenetic determinant in renal biopsy specimens. The clinical details and the results of MCA-PI binding studies for three patients with nail-patella syndrome are presented. Two of these patients proved to have an abnormal antigenicity of the glomerular basement membrane as demonstrated by the absence of binding of MCA-PI. This is the first time that such an abnormality has been demonstrated in patients other than those with Alport-type nephritis. The significance of these results is discussed.