Imaging of fibropolycystic liver disease.
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 u...
| Publicado en: | Abdominal Radiology Vol. 47; no. 7; pp. 2356 - 2371 |
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| Autores principales: | , , , , |
| Formato: | Journal Article |
| Publicado: |
Springer Nature
Jul2022
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| Acceso en línea: | Ver este registro en EBSCOhost |
| fields | @attributes: recordID: 1 pdfLink: plink: https://search.ebscohost.com/login.aspx?direct=true&db=ccm&AN=157613535&site=ehost-live header: @attributes: shortDbName: ccm uiTerm: 157613535 longDbName: CINAHL Complete uiTag: AN controlInfo: bkinfo: dissinfo: jinfo: jid: 2366004X JT14 jtl: Abdominal Radiology issn: 2366004X maglogo: N pubinfo: dt: Jul2022 vid: 47 iid: 7 pid: 237 pub: Springer Nature place: New York, New York artinfo: ui: 157613535 157299854 10.1007/s00261-022-03565-7 157613535 ppf: 2356 ppct: 15 formats: fmt: – @attributes: type: T – @attributes: type: P tig: atl: Imaging of fibropolycystic liver disease. aug: au: Sharbidre, Kedar Zahid, Mohd Venkatesh, Sudhakar Kundapur Bhati, Chandra Lalwani, Neeraj affil: Department of Abdominal Imaging, University of Alabama at Birmingham, Birmingham, AB, USA sug: ab: 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. pubtype: Academic Journal doctype: Journal Article ougenre: Article language: English refInfo: holdings: @attributes: islocal: N |
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