Imaging of fibropolycystic liver disease.
Caroli
Congenital hepatic fibrosis
Ductal plate
Fibrocystic
Journal
Abdominal radiology (New York)
ISSN: 2366-0058
Titre abrégé: Abdom Radiol (NY)
Pays: United States
ID NLM: 101674571
Informations de publication
Date de publication:
07 2022
07 2022
Historique:
received:
22
03
2022
accepted:
17
05
2022
revised:
14
05
2022
pubmed:
8
6
2022
medline:
28
6
2022
entrez:
7
6
2022
Statut:
ppublish
Résumé
Fibropolycystic liver diseases (FLDs) make up a rare spectrum of heritable hepatobiliary diseases resulting from congenital ductal plate malformations (DPMs) due to the dysfunction of proteins expressed on the primary cilia of cholangiocytes. The embryonic development of the ductal plate is key to understanding this spectrum of diseases. In particular, DPMs can result in various degrees of intrahepatic duct involvement and a wide spectrum of cholangiopathies, including congenital hepatic fibrosis, Caroli disease, polycystic liver disease, and Von Meyenberg complexes. The most common clinical manifestations of FLDs are portal hypertension, cholestasis, cholangitis, and (in rare cases) cholangiocarcinoma. This article reviews recent updates in the pathophysiology, imaging, and clinical management of FLDs.
Identifiants
pubmed: 35670875
doi: 10.1007/s00261-022-03565-7
pii: 10.1007/s00261-022-03565-7
doi:
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
2356-2370Informations de copyright
© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.
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