Abstract
A 27 yr old woman with maturity-onset diabetes of the young had a nephrotic syndrome and congestive heart failure. Studies disclosed severe diabetic retinopathy and nephropathy. The patient''s HLA type at the A and B loci did not contain markers previously associated with diabetic vascular disease. This is the 1st patient with maturity-onset diabetes of the young to be described with serious vascular disease and absence of previously described HLA markers for microangiopathy.