title: Kidney and liver transplantation in patients with autosomal recessive polycystic kidney disease: a multicentric study.
abstract: OBJECTIVE
  In contrast to the improvement in our understanding of the pathogenesis and presentation of autosomal recessive polycystic kidney disease (ARPKD), data regarding the issue of kidney and liver transplantation in patients with ARPKD remain particularly scarce. Here, we report the results and outcome of renal and/or liver transplantation in a series of patients with ARPKD.
  
  
  METHODS
  Fourteen ARPKD patients (age: 3-25 years) who underwent renal transplantation with or without liver transplantation were retrospectively identified in five French nephrology departments. The patients' medical charts were reviewed and relevant data were collected.
  
  
  RESULTS
  The clinical and radiological presentation of the 14 patients was highly variable illustrating the heterogeneity of ARPKD. Six patients underwent kidney and/or liver transplantation in adulthood. First renal graft survival was 92, 78 and 14% at 1, 5 and 10 years after renal transplantation, respectively. Mortality rate was relatively high (3/14; 21%) in these young patients and was directly related to infectious complications (recurrent angiocholitis) of severe Caroli's disease (dilatation of intra- and/or extra-hepatic bile ducts), a typical feature of ARPKD.
  
  
  CONCLUSIONS
  Our data suggest that ARPKD patients evaluated for renal transplantation should be carefully screened for severe Caroli's disease. Even though the limited number of patients included in our study precludes any definite recommendation, pre-emptive liver transplantation may be a therapeutic option in ARPKD patients with severe Caroli's disease evaluated for renal transplantation.
authors.affiliation: Nephrology Department, Institut de Transplantation Urologie et Nephrologie, CHU de Nantes, Nantes, France.
authors.forename: Marion
authors.initials: M
authors.lastname: Chapal
authors.forename: Agnès
authors.initials: A
authors.lastname: Debout
authors.forename: Alexandre
authors.initials: A
authors.lastname: Dufay
authors.forename: Rémi
authors.initials: R
authors.lastname: Salomon
authors.forename: Gwenaelle
authors.initials: G
authors.lastname: Roussey
authors.forename: Stéphane
authors.initials: S
authors.lastname: Burtey
authors.forename: Emma Allain
authors.initials: EA
authors.lastname: Launay
authors.forename: Cécile
authors.initials: C
authors.lastname: Vigneau
authors.forename: Gilles
authors.initials: G
authors.lastname: Blancho
authors.forename: Chantal
authors.initials: C
authors.lastname: Loirat
authors.forename: Maryvonne
authors.initials: M
authors.lastname: Hourmant
authors.forename: Fadi
authors.initials: F
authors.lastname: Fakhouri
mesh_terms: D000293:Adolescent; D000328:Adult; D016767:Caroli Disease / Q000209:etiology / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D019468:Disease Management; D005260:Female; D005602:France; D006801:Humans; D016030:Kidney Transplantation; D016031:Liver Transplantation; D008137:Longitudinal Studies; D008297:Male; D017044:Polycystic Kidney, Autosomal Recessive / Q000150:complications / Q000401:mortality / Q000601:surgery*; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome; D055815:Young Adult