Steroid-resistant nephrotic syndrome: a persistent challenge for pediatric nephrology

Pediatr Nephrol. 2017 Jun;32(6):965-974. doi: 10.1007/s00467-016-3459-5. Epub 2016 Oct 26.

Abstract

Steroid-resistant nephrotic syndrome remains a challenge to treat, but various efforts are underway to better understand the pathogenesis and improve patient outcomes. This review provides an update on the newer advances in understanding the molecular etiologies for a variety of podocyte abnormalities, potential circulating factors that may initiate and sustain the steroid-resistant state, genetic mutations, and precision medicine treatment modalities in this continuously perplexing disorder.

Keywords: Focal segmental glomerulosclerosis; Genetic testing; Glomerular filtration barrier; Minimal change disease; Podocyte; Steroid-resistant nephrotic syndrome.

Publication types

  • Review
  • Research Support, N.I.H., Extramural

MeSH terms

  • Adrenocorticotropic Hormone / therapeutic use
  • Age Factors
  • Biopsy
  • Calcineurin Inhibitors / therapeutic use
  • Child, Preschool
  • Drug Resistance
  • Genetic Testing
  • Glomerulosclerosis, Focal Segmental / pathology
  • Glucocorticoids / pharmacology
  • Glucocorticoids / therapeutic use*
  • Humans
  • Immunosuppressive Agents / therapeutic use*
  • Infant
  • Infant, Newborn
  • Microscopy, Electron
  • Mutation
  • Nephrotic Syndrome / classification
  • Nephrotic Syndrome / drug therapy*
  • Nephrotic Syndrome / etiology*
  • Nephrotic Syndrome / pathology
  • Podocytes / pathology*
  • Podocytes / ultrastructure
  • Receptors, Urokinase Plasminogen Activator / blood
  • Treatment Outcome

Substances

  • Calcineurin Inhibitors
  • Glucocorticoids
  • Immunosuppressive Agents
  • PLAUR protein, human
  • Receptors, Urokinase Plasminogen Activator
  • Adrenocorticotropic Hormone