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[Idiopathic nephrotic syndrome].

O Boyer1, V Baudouin2, E Bérard3

  • 1Service de néphrologie pédiatrique, centre de référence syndrome néphrotique idiopathique de l'enfant et de l'adulte, hôpital Necker-Enfants-Malades, institut Imagine, université Paris-Descartes, Assistance publique-Hôpitaux de Paris, 75015 Paris, France.

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|November 25, 2017
PubMed
Summary

Idiopathic nephrotic syndrome (INS) is a common kidney disease in children, often responding to steroid therapy. While most children recover, a small percentage develop steroid resistance, increasing the risk of kidney failure.

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Area of Science:

  • Pediatric Nephrology
  • Internal Medicine
  • Genetics

Background:

  • Nephrotic syndrome (NS) is characterized by proteinuria, hypoalbuminemia, edema, and hyperlipidemia.
  • Idiopathic nephrotic syndrome (INS) is the most frequent cause of NS in children, with an incidence of 1-4 per 100,000.
  • INS can be triggered by infections, drugs, allergies, vaccinations, and malignancies, typically affecting children aged 2-7 years.

Purpose of the Study:

  • To provide a comprehensive overview of idiopathic nephrotic syndrome in children.
  • To discuss the diagnostic criteria, management, and prognosis of INS.
  • To highlight the importance of steroid therapy and identify risk factors for steroid resistance.

Main Methods:

  • Review of existing literature on idiopathic nephrotic syndrome in pediatric populations.
  • Analysis of clinical presentation, diagnostic approaches, and treatment strategies.
  • Evaluation of factors influencing steroid responsiveness and long-term outcomes.

Main Results:

  • Most children (85-90%) with INS respond to initial corticosteroid treatment.
  • Relapses are common, but the majority of patients continue to respond to steroids in subsequent courses.
  • A small percentage (1-3%) of patients develop steroid resistance, posing a risk for end-stage kidney disease.

Conclusions:

  • Idiopathic nephrotic syndrome is a manageable condition in most children with prompt steroid therapy.
  • Close monitoring is essential to detect and manage steroid resistance and its complications.
  • Further research into the underlying mechanisms of INS and steroid resistance is warranted.