Diagnosis and management of hepatobiliary complications in autosomal recessive polycystic kidney disease

Andrew Wehrman, Alyssa Kriegermeier, Jessica Wen

Research output: Contribution to journalShort surveypeer-review

19 Scopus citations

Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is a congenital hepatorenal fibrocystic disease. The hepatic manifestations of ARPKD can range from asymptomatic to portal hypertension and massively dilated biliary system that results in liver transplantation. Hepatic complications of ARPKD typically present with signs of portal hypertension (splenomegaly and thrombocytopenia) or cholangitis. Liver disease in ARPKD does not always correlate with severity of renal disease. Management of ARPKD-related liver disease is largely treating specific symptoms, such as antibiotics for cholangitis or endoscopic treatment for variceal bleeding. If complications cannot be managed medically, liver transplantation may be indicated. This mini-review will discuss the clinical manifestations and management of children with ARPKD liver disease.

Original languageEnglish
Article number124
JournalFrontiers in Pediatrics
Volume5
DOIs
StatePublished - May 29 2017
Externally publishedYes

Keywords

  • Autosomal recessive polycystic kidney disease
  • Cholangitis
  • Congenital hepatic fibrosis
  • Esophageal varices
  • Portal hypertension

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