RRML - Congenital Nephrotic Syndrome of Finnish Type. A Report of Two Cases

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Nr. 17(4)/2009

Congenital Nephrotic Syndrome of Finnish Type. A Report of Two Cases

Carmen Duicu, Eva Kiss, Claudia Bănescu, Virginia Bodescu, Zsuzsanna Moreh, Emöke Horvath

Abstract:

Congenital nephrotic syndrome of the Finnish type (CNSF) is a rare autosomal recessive disease with high infant mortality without aggressive treatment. CNSF is characterized by massive in utero proteinuria and nephrosis at birth. The majority of cases are caused by genetic defects in the components of the glomerular filtration barrier, especially nephrin and podocin. Congenital nephrotic syndrome (CNS) may also be a part of a more generalized syndrome or caused by a perinatal infection. Immunosuppressive medication is not helpful in the genetic forms of CNS, and kidney transplantation is the only curative therapy. This paper describes the clinical features and outcome of the last 2 patients from 6 with CNSF who have been admitted to Pediatric Clinics from Târgu Mures in the last 15 years.

Keywords: congenital nephrotic syndrome,proteinuria

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Duicu C, Kiss E, Bănescu C, Bodescu V, Moreh Z, Horvath E. Congenital Nephrotic Syndrome of Finnish Type. A Report of Two Cases . Rev Romana Med Lab. 2009;17(4):43-9