Genetics of Congenital and Early Infantile Nephrotic Syndromes

  • Olli Koskimies
Part of the Topics in Renal Medicine book series (TIRM, volume 9)


The congenital nephrotic syndrome (CoNS) is characterized by the presence of proteinuria at birth. Marked glomerular proteinuria leads to protein depletion, a low serum protein level, edema, dyslipidemia, and disturbances in fluid and electrolyte homeostasis. Overt nephrotic syndrome develops; the infant fails to thrive, and her/his psychomotor development is disturbed. It was thought that the age of three months separates congenital from infantile forms of NS. This division proved to be arbitrary. The diagnosis has to be based on clinical, laboratory, and histological criteria, rather than age of onset.


Nephrotic Syndrome Prenatal Diagnosis Autosomal Recessive Disease Renal Vein Thrombosis Congenital Nephrotic Syndrome 
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© Kluwer Academic Publishers 1990

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  • Olli Koskimies

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