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Review
. 2011 Dec;4(6):460-5.
doi: 10.1111/j.1752-8062.2011.00306.x. Epub 2011 Dec 7.

Congenital hepatic fibrosis in autosomal recessive polycystic kidney disease

Jessica Wen  1
Affiliations
Review

Congenital hepatic fibrosis in autosomal recessive polycystic kidney disease

Jessica Wen. Clin Transl Sci. 2011 Dec.

Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is a developmental disorder that mainly affects the kidneys and the biliary tract. Affected patients often have massively enlarged cystic kidneys as well as congenital hepatic fibrosis (CHF) characterized by dilated bile ducts and associated peribiliary fibrosis. This review will examine what is known about ARPKD-associated liver disease and will highlight areas of ongoing research into its pathogenesis and potential treatment.

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Figures

Figure 1
Figure 1
Liver explant specimen from a patient with ARPKD/CHF. The bile ducts are in a ring‐like pattern surrounding the portal vein. The portal tract is expanded with dilated bile ducts surrounded by fibrous tissue. The liver parenchyma is well preserved. This is typical of ductal plate malformation. H&E100× magnification.
See this image and copyright information in PMC

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