Figure 1734

Mutations have been identified in about half of patients with Alport's syndrome and are found in the genes for the a3, a4, or a5 chains of type IV collagen, which are the major constituents of the glomerular basement membrane. After transplantation, approximately 15% of patients develop linear deposition of immunoglobulin G (IgG) along the glomerular basement membrane (GBM), and circulating anti-GBM antibodies specific for the a3 or a5 chains of type IV collagen [42-44]. It is unclear why only some patients develop antibodies. Clinical disease, however, is rare. Only 20% of patients with antibody deposition develop urinary abnormalities from 1 month to 2 years after grafting. Those patients who do develop proteinuria or hematuria usually lose their grafts. In some cases, treatment with cyclophosphamide did not prevent graft loss.

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