Glomerular lesions in the branchio-oto-renal (BOR) syndrome

Three cases: two adults and one child, with histologically confirmed branchio-oto-renal syndrome showed prominent glomerular lesions. Typically there was segmental and focal hyalinization associated with dense deposits along the basement membrane and in the mesangium. Variable deposits of immunoglobulins IgG, IgM, and IgA as well as C3 were present. The branchio-oto-renal syndrome appears to be a familial nephropathy which may lead to renal failure.