ADULT FAMILIAL NEPHROTIC SYNDROME - BALKAN VARIANT OF CONGENITAL NEPHROTIC SYNDROME

We report on two families from the southern parr of Former Yugoslav Re public of Macedonia (close to Mediterranean area) with autosomal domin ant inherited nephrotic syndrome, documented with renal biopsy in thre e family members. Histopathological examination confirmed microcystic tubular changes and focal segmental glomerulosclerosis. In biopsied pa tients proteinuria was noted in the first decade, or second, but it wa s quantitatively documented as nephrotic in the second and third decad e, The prognosis of the patients was poor: one patient died because of intracranial hemorrhage before developing end stage renal disease; th e other two cases developed end stage chronic renal failure in the thi rd and fourth decade.