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SummaryCongenital Nephrotic Syndrome of the Finish type (CNF) is a rare and severe disease. A neonate with CNF is described. The diagnosis carries a dramatically poorer prognosis than nephrotic syndrome diagnosed after one year. The clinical course is one of persistent oedema and recurrent infections leading to death. The gene for the Finish type has been mapped to the long arm of chromosome 19. Case reports show it to be responsive to captopril and indomethacin. It is uniformly resistant to steroids and immunosuppressive drugs.

Congenital Nephrotic Syndrome of the Finnish Type