Adult Familial Nephrotic Syndrome—Balkan Variant of Congenital Nephrotic Syndrome

Abstract

We report on two families from the southern part of Former Yugoslav Republic of Macedonia (close to Mediterranean area) with autosomal dominant inherited nephrotic syndrome, documented with renal biopsy in three family members. Histopathological examination confirmed microcystic tubular changes and focal segmental glomerulosclerosis. In biopsied patients proteinuria was noted in the first decade, or second, but it was quantitatively documented as nephrotic in the second and third decade. The prognosis of the patients was poor: one patient died because ofintracranial hemorrhage before developing end stage renal disease; the other two cases developed end stage chronic renal failure in the third and fourth decade.