Beaded bile ducts in primary sclerosing cholangitis

2019 ◽  
Vol 44 (3) ◽  
pp. 1195-1196 ◽  
Author(s):  
Dario Giambelluca ◽  
Claudio Leto ◽  
Francesco D’Arpa ◽  
Massimo Midiri ◽  
Giuseppe Salvaggio
2021 ◽  
Vol 3 (2) ◽  
pp. 20-24
Author(s):  
Aldona Wybraniec-Zaręba ◽  
Julia Tuchalska-Czuroń ◽  
Gabriela Półtorak-Szymczak ◽  
Mariusz Furmanek ◽  
Jerzy Walecki ◽  
...  

Primary sclerosing cholangitis (PSC) is a chronic liver disease in which there are inflammation and scarring of the bile ducts leading to fibrosis, destruction and narrowing of the bile ducts, resulting in cholestasis. In the long run, PSC can cause liver cirrhosis and failure. In clinical practice, the diagnosis of PSC is generally based on blood tests and imaging studies (currently preferably magnetic resonance cholangiopancreatography). To make a diagnosis of PSC it is necessary to exclude secondary causes of sclerosing cholangitis. The most common MRI features of PSC concerning bile ducts are: bile duct dilatation, beading, extrahepatic bile duct stenosis, wall enhancement and thickening. The most common MRI features of PSC concerning hepatic parenchyma are: rounded shape of the liver caused by hypertrophy of caudate lobe and left liver lobe, atrophy of the right lobe, enlargement of portal and/or portacaval lymph nodes, peripheral parenchymal inflammation, wedge-shaped confluent fibrosis, heterogeneity of the liver parenchyma, periportal oedema, cirrhosis with indirect signs of portal hypertension such as splenomegaly, ascites and collateral vasculature.


2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Yinka K. Davies ◽  
Cynthia J. Tsay ◽  
Dario V. Caccamo ◽  
Kathleen M. Cox ◽  
Ricardo O. Castillo ◽  
...  

Primary sclerosing cholangitis (PSC) is a progressive, cholestatic disease of the liver that is marked by inflammation of the bile ducts and damage to the hepatic biliary tree. Approximately 60–70% of patients also have inflammatory bowel disease and progression of PSC can lead to ulcerative colitis and cirrhosis of the liver. Due to limited understanding of the etiology and mechanism of PSC, the only existing treatment option is orthotopic liver transplantation (OLT); however, recurrence of PSC, after OLT is estimated to be between 5% and 35%. We discuss the successful treatment of a pediatric patient, with recurrent PSC, after OLT with oral Vancomycin.


1990 ◽  
Vol 11 (2) ◽  
pp. 196-199 ◽  
Author(s):  
Jean Genève ◽  
Nathalie Dubuc ◽  
Didier Mathieu ◽  
Elie Serge Zafrani ◽  
Daniel Dhumeaux ◽  
...  

2010 ◽  
Vol 2010 ◽  
pp. 1-4
Author(s):  
Siu-Tong Law ◽  
Wai-Ki Lee ◽  
Michael Kin-Kong Li ◽  
Ka-Ho Lok

Primary sclerosing cholangitis is a rare cause of cholestasis caused by progressive inflammation and fibrosis of both intrahepatic and extrahepatic bile ducts leading to multifocal ductal strictures. Herein, we report a case of primary sclerosing cholangitis and inflammatory bowel disease. The concomitant diagnosis of these two diseases is not typical. The management includes the treatment of inflammatory bowel disease and potential complications of primary sclerosing cholangitis, including dominant strictures of bile duct, portal hypertension, gallbladder diseases, cholangiocarcinoma, and colonoscopic surveillance.


2021 ◽  
Vol 13 (599) ◽  
pp. eabb3107
Author(s):  
Christine L. Zimmer ◽  
Erik von Seth ◽  
Marcus Buggert ◽  
Otto Strauss ◽  
Laura Hertwig ◽  
...  

The human biliary system, a mucosal barrier tissue connecting the liver and intestine, is an organ often affected by serious inflammatory and malignant diseases. Although these diseases are linked to immunological processes, the biliary system represents an unexplored immunological niche. By combining endoscopy-guided sampling of the biliary tree with a high-dimensional analysis approach, comprehensive mapping of the human biliary immunological landscape in patients with primary sclerosing cholangitis (PSC), a severe biliary inflammatory disease, was conducted. Major differences in immune cell composition in bile ducts compared to blood were revealed. Furthermore, biliary inflammation in patients with PSC was characterized by high presence of neutrophils and T cells as compared to control individuals without PSC. The biliary T cells displayed a CD103+CD69+ effector memory phenotype, a combined gut and liver homing profile, and produced interleukin-17 (IL-17) and IL-22. Biliary neutrophil infiltration in PSC associated with CXCL8, possibly produced by resident T cells, and CXCL16 was linked to the enrichment of T cells. This study uncovers the immunological niche of human bile ducts, defines a local immune network between neutrophils and biliary-resident T cells in PSC, and provides a resource for future studies of the immune responses in biliary disorders.


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