Congenital nephrotic syndrome

ABSTRACT

Congenital Nephrotic Syndrome is a rare disorder in which inheritance is probably autosomal recessive, The most striking characteristic of congenital nephrotic syndrome, in contrast to that occuring later during childhood, is the resistance to corticosteroid therapy and the almost invariable fatal outcome. It appears to have a peculiarly high incidence in Scandinavian countries, particularly Finland. Often it is familial; maternal antibodies may be responsible as in Rh- incompatibility, it appear before the child first year; sometimes during the first few weeks of life. The course is progressive and few patients survive the first year of life. Death is usually due to renal failure or secondary infection. In this report, an Iraqi family with congenital nephrotic syndrome was thoroughly studied in one of them renal biopsy was done in UK. that confirmed the diagnosis