title: Tolvaptan treatment for severe neonatal autosomal-dominant polycystic kidney disease.
abstract: Severe neonatal autosomal-dominant polycystic kidney disease (ADPKD) is rare and easily confused with recessive PKD. Managing such infants is difficult and often unsuccessful.
  
  
  
  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.
  
  
  
  Tolvaptan may be a useful treatment for severe neonatal PKD.
authors.affiliation: Regional Paediatric Nephro-Urology Unit, Southampton Children's Hospital, Tremona Road, Southampton, SO16 6YD, Hampshire, UK. Rodney.Gilbert@uhs.nhs.uk.|Faculty of Medicine, University of Southampton, Southampton, UK. Rodney.Gilbert@uhs.nhs.uk.
authors.forename: Rodney D
authors.initials: RD
authors.lastname: Gilbert
authors.affiliation: Respiratory Unit, Southampton Children's Hospital, Southampton, UK.
authors.forename: Hazel
authors.initials: H
authors.lastname: Evans
authors.affiliation: Pharmacy, Southampton University Hospital, Southampton, UK.
authors.forename: Kazeem
authors.initials: K
authors.lastname: Olalekan
authors.affiliation: Regional Paediatric Nephro-Urology Unit, Southampton Children's Hospital, Tremona Road, Southampton, SO16 6YD, Hampshire, UK.
authors.forename: Arvind
authors.initials: A
authors.lastname: Nagra
authors.affiliation: Regional Paediatric Nephro-Urology Unit, Southampton Children's Hospital, Tremona Road, Southampton, SO16 6YD, Hampshire, UK.
authors.forename: Mushfequr R
authors.initials: MR
authors.lastname: Haq
authors.affiliation: Radiology Department, Southampton Children's Hospital, Southampton, UK.
authors.forename: Mark
authors.initials: M
authors.lastname: Griffiths
mesh_terms: D001552:Benzazepines / Q000627:therapeutic use*; D004487:Edema / Q000188:drug therapy / Q000209:etiology; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D007231:Infant, Newborn; D007677:Kidney Function Tests; D010288:Parenteral Nutrition; D016891:Polycystic Kidney, Autosomal Dominant / Q000151:congenital / Q000188:drug therapy* / Q000235:genetics; D011247:Pregnancy; D017483:Receptors, Vasopressin / Q000235:genetics; D050396:TRPP Cation Channels / Q000235:genetics; D000077602:Tolvaptan; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D001552:Benzazepines; D017483:Receptors, Vasopressin; D050396:TRPP Cation Channels; D000077602:Tolvaptan; C585768:AVPR2 protein, human
keywords: Autosomal dominant polycystic kidney disease; Infant; Tolvaptan