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Congenital nephrotic syndrome

C Holmberg1, J Laine, K Rönnholm

  • 1Division of Pediatric Nephrology, Children's Hospital, University of Helsinki, Finland.

Kidney International. Supplement
|January 1, 1996
PubMed
Summary

Finnish-type congenital nephrotic syndrome (CNF) is a severe kidney disorder. Early diagnosis and treatment, including kidney transplantation, lead to normal growth and high patient survival rates.

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Area of Science:

  • Nephrology
  • Genetics
  • Pediatrics

Background:

  • Congenital nephrotic syndrome (CNS) presents with massive proteinuria from birth.
  • Finnish-type CNS (CNF) is the most common form, inherited in an autosomal recessive pattern.
  • The exact pathogenesis of CNF remains unclear despite gene localization.

Purpose of the Study:

  • To describe the diagnostic criteria and treatment outcomes for Finnish-type congenital nephrotic syndrome (CNF).

Main Methods:

  • Diagnosis based on intrauterine proteinuria, family history, placental weight, and exclusion of other CNS types.
  • Treatment involved albumin substitution, nutrition, thyroxine, anticoagulation, bilateral nephrectomy, peritoneal dialysis, and renal transplantation (Tx).

Main Results:

  • Forty-six CNF patients were treated; 34 underwent renal transplantation at a mean age of 2.2 years.
  • Post-transplant patient survival was 97%, with graft survival at 94%, 81%, and 81% at 1, 3, and 5 years, respectively.
  • Post-transplant GFR averaged 75 ml/min/1.73 m2, with normal growth and development observed.

Conclusions:

  • Early diagnosis and comprehensive management, including renal transplantation, are crucial for improving outcomes in Finnish-type congenital nephrotic syndrome.
  • Successful renal transplantation enables normal growth, development, and high long-term survival for CNF patients.

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