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Genetics of Congenital and Early Infantile Nephrotic Syndromes

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Inheritance of Kidney and Urinary Tract Diseases

Part of the book series: Topics in Renal Medicine ((TIRM,volume 9))

Abstract

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.

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Authors
  1. Olli Koskimies
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Editors and Affiliations

  1. Albert Einstein College of Medicine, Bronx, New York, USA

    Adrian Spitzer

  2. Children’s Hospital and Medical Center, Seattle, Washington, USA

    Ellis D. Avner

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© 1990 Kluwer Academic Publishers

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Koskimies, O. (1990). Genetics of Congenital and Early Infantile Nephrotic Syndromes. In: Spitzer, A., Avner, E.D. (eds) Inheritance of Kidney and Urinary Tract Diseases. Topics in Renal Medicine, vol 9. Springer, Boston, MA. https://doi.org/10.1007/978-1-4613-1603-9_7

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  • DOI: https://doi.org/10.1007/978-1-4613-1603-9_7

  • Publisher Name: Springer, Boston, MA

  • Print ISBN: 978-1-4612-8887-9

  • Online ISBN: 978-1-4613-1603-9

  • eBook Packages: Springer Book Archive

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