title: Early bilateral nephrectomy in infantile autosomal recessive polycystic kidney disease.
abstract: The management of neonatal autosomal recessive polycystic kidney disease (ARPKD) complicated by severe pulmonary insufficiency presents complex clinical challenges. Where massive nephromegaly exists, early bilateral nephrectomy, supportive peritoneal dialysis and early aggressive nutrition can minimise infant mortality. Consensus, however, is lacking on the role and optimal timing of nephrectomy, with decision-making driven by the patient's clinical condition and the expertise of the centre. We report on our experience of an infant with ARPKD requiring neonatal renal replacement therapy and survival at 14 months following early bilateral nephrectomy.
authors.affiliation: Department of Paediatric Nephrology, Royal Belfast Hospital for Sick Children, Belfast, UK Department of Paediatric Nephrology, Royal Bristol Hospital for Children, Bristol, UK.
authors.forename: Tamara Mary
authors.initials: TM
authors.lastname: Mallett
authors.affiliation: Department of Paediatric Nephrology, Royal Belfast Hospital for Sick Children, Belfast, UK Department of Paediatric Nephrology, Royal Manchester Children's Hospital, Manchester, UK.
authors.forename: Emma
authors.initials: E
authors.lastname: O'Hagan
authors.affiliation: Department of Paediatric Nephrology, Royal Belfast Hospital for Sick Children, Belfast, UK.
authors.forename: Karl Gerard
authors.initials: KG
authors.lastname: McKeever
mesh_terms: D004452:Echocardiography; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009392:Nephrectomy / Q000379:methods; D010530:Peritoneal Dialysis / Q000379:methods; D017044:Polycystic Kidney, Autosomal Recessive / Q000175:diagnosis / Q000601:surgery*; D011247:Pregnancy; D016896:Treatment Outcome