Transplantation in autosomal recessive polycystic kidney disease: liver and/or kidney?

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8 Scopus citations


Autosomal recessive polycystic kidney disease (ARPKD) is characterized by enlarged kidneys with dilated collecting ducts and congenital hepatic fibrosis. There is a variable rate of progression of kidney and liver disease. Portal hypertension and Caroli’s disease occur from liver involvement that contributes to morbidity and mortality. Approximately 40 % of patients have a severe disease phenotype leading to rapid onset of end-stage kidney disease (ESKD) and signs of portal hypertension and the rest may have predominant involvement of either the kidney or liver. It is important for the physician to establish the extent of organ involvement before deciding on the ultimate plan of management, especially when transplantation is required. Isolated renal transplantation can be considered when liver involvement is minimal. If hepatobiliary disease is prominent, and kidney function is preserved, management options are based on individual characteristics. In the presence of significant liver disease and ESKD, consideration should be given to combined liver kidney transplantation, which can be beneficial in eliminating the consequences of both kidney and liver disease. However, this is a complex surgical procedure that needs to be performed at experienced transplant centers. Improvement in surgical techniques has considerably improved short-term graft survival with the added advantage of the liver offering immunologic protection to the kidney allograft.

Original languageEnglish (US)
Pages (from-to)1233-1242
Number of pages10
JournalPediatric Nephrology
Issue number8
StatePublished - Aug 29 2015


  • Children
  • Combined liver kidney transplant
  • Congenital hepatic fibrosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Nephrology


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