Evaluation and management of steroid-unresponsive nephrotic syndrome

Research output: Contribution to journalReview article

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Abstract

PURPOSE OF REVIEW: Idiopathic nephrotic syndrome in children is commonly associated with minimal change disease and response to steroid therapy. Steroid-unresponsive nephrotic syndrome is often characterized by persistent proteinuria and progression to chronic kidney disease. Focal segmental glomerulosclerosis is the leading cause of steroid-unresponsive nephrotic syndrome in childhood. There is no uniformed consensus as to the treatment of steroid-unresponsive nephrotic syndrome. Advances in the pathogenesis, genetics and biomarkers or surrogate markers may be useful for the diagnosis and identification of patients with steroid-unresponsive nephrotic syndrome, severity of disease, progression and response to therapy. RECENT FINDINGS: This review is intended to describe some of the recent changes in the epidemiology of steroid-unresponsive nephrotic syndrome, in particular focal segmental glomerulosclerosis, its pathogenesis and alternative therapies. SUMMARY: Recent studies in both children and adults have shown an increase in the incidence of focal segmental sclerosis as a cause of steroid-unresponsive nephrotic syndrome. Advances in the pathogenesis and noninvasive methods of diagnosis may allow for the identification of steroid-responsive patients.

Original languageEnglish (US)
Pages (from-to)151-156
Number of pages6
JournalCurrent Opinion in Pediatrics
Volume20
Issue number2
DOIs
StatePublished - Apr 1 2008

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Keywords

  • Focal segmental glomerulosclerosis
  • Nephrotic syndrome
  • Steroid-resistant nephrotic syndrome
  • Steroid-sensitive nephrotic syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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