Tolvaptan treatment for severe neonatal autosomal-dominant polycystic kidney disease

Pediatr Nephrol. 2017 May;32(5):893-896. doi: 10.1007/s00467-017-3584-9. Epub 2017 Feb 13.

Abstract

Background: Severe neonatal autosomal-dominant polycystic kidney disease (ADPKD) is rare and easily confused with recessive PKD. Managing such infants is difficult and often unsuccessful.

Case diagnosis/treatment: A female infant with massive renal enlargement, respiratory compromise and hyponatraemia was treated with the arginine vasopressin receptor 2 antagonist tolvaptan. This resolved hyponatraemia, and there was no further increase in renal size.

Conclusion: Tolvaptan may be a useful treatment for severe neonatal PKD.

Keywords: Autosomal dominant polycystic kidney disease; Infant; Tolvaptan.

Publication types

  • Case Reports

MeSH terms

  • Benzazepines / therapeutic use*
  • Edema / drug therapy
  • Edema / etiology
  • Female
  • Glomerular Filtration Rate
  • Humans
  • Infant, Newborn
  • Kidney Function Tests
  • Parenteral Nutrition
  • Polycystic Kidney, Autosomal Dominant / congenital
  • Polycystic Kidney, Autosomal Dominant / drug therapy*
  • Polycystic Kidney, Autosomal Dominant / genetics
  • Pregnancy
  • Receptors, Vasopressin / genetics
  • TRPP Cation Channels / genetics
  • Tolvaptan
  • Treatment Outcome
  • Young Adult

Substances

  • AVPR2 protein, human
  • Benzazepines
  • Receptors, Vasopressin
  • TRPP Cation Channels
  • polycystic kidney disease 1 protein
  • Tolvaptan