Abstract | BACKGROUND AND OBJECTIVES: 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:
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Authors | Marion Chapal, Agnès Debout, Alexandre Dufay, Rémi Salomon, Gwenaelle Roussey, Stéphane Burtey, Emma Allain Launay, Cécile Vigneau, Gilles Blancho, Chantal Loirat, Maryvonne Hourmant, Fadi Fakhouri |
Journal | Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
(Nephrol Dial Transplant)
Vol. 27
Issue 5
Pg. 2083-8
(May 2012)
ISSN: 1460-2385 [Electronic] England |
PMID | 22076432
(Publication Type: Journal Article, Multicenter Study)
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Topics |
- Adolescent
- Adult
- Caroli Disease
(etiology, mortality, surgery)
- Child
- Child, Preschool
- Cohort Studies
- Disease Management
- Female
- France
- Humans
- Kidney Transplantation
- Liver Transplantation
- Longitudinal Studies
- Male
- Polycystic Kidney, Autosomal Recessive
(complications, mortality, surgery)
- Retrospective Studies
- Survival Rate
- Treatment Outcome
- Young Adult
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