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

Andrew Wehrman, Alyssa Anne Kriegermeier, Jessica Wen*

*Corresponding author for this work

Research output: Contribution to journalShort survey

6 Citations (Scopus)

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 (US)
Article number124
JournalFrontiers in Pediatrics
Volume5
DOIs
StatePublished - May 29 2017

Fingerprint

Autosomal Recessive Polycystic Kidney
Liver Diseases
Cholangitis
Portal Hypertension
Liver Transplantation
Liver
Splenomegaly
Biliary Tract
Thrombocytopenia
Hemorrhage
Anti-Bacterial Agents
Kidney

Keywords

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

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Cite this

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title = "Diagnosis and management of hepatobiliary complications in autosomal recessive polycystic kidney disease",
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.",
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Diagnosis and management of hepatobiliary complications in autosomal recessive polycystic kidney disease. / Wehrman, Andrew; Kriegermeier, Alyssa Anne; Wen, Jessica.

In: Frontiers in Pediatrics, Vol. 5, 124, 29.05.2017.

Research output: Contribution to journalShort survey

TY - JOUR

T1 - Diagnosis and management of hepatobiliary complications in autosomal recessive polycystic kidney disease

AU - Wehrman, Andrew

AU - Kriegermeier, Alyssa Anne

AU - Wen, Jessica

PY - 2017/5/29

Y1 - 2017/5/29

N2 - 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.

AB - 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.

KW - Autosomal recessive polycystic kidney disease

KW - Cholangitis

KW - Congenital hepatic fibrosis

KW - Esophageal varices

KW - Portal hypertension

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