Primary sclerosing cholangitis (PSC) is a serious disease that not only affects quality of life but can also have a significant effect on patient survival. The treatment for PSC is primarily supportive with the aim of controlling cholestatic symptoms and preventing complications. Ursodeoxycholic acid may induce biochemical improvements in affected patients; however, long-term pediatric studies to determine its possible benefits in young patients are lacking. Thus, the treatment of pediatric PSC remains a significant clinical challenge. We describe a patient with PSC and undetermined colitis who was treated with a combination of a steroid, salazosulfapyridine, and a probiotic. This treatment provided benefits both for PSC and the undetermined colitis. These findings suggest that bacterial flora and gut inflammation are closely associated with the pathogenesis of inflammatory bowel disease-related PSC. Suppression of bowel inflammation and maintenance of bacterial homeostasis may be important for treating PSC.
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http://dx.doi.org/10.1159/000338834 | DOI Listing |
Endoscopy
January 2025
Department of Gastroenterology, HUS Abdominal Center, Helsinki University Central Hospital, Helsinki, Finland.
Primary sclerosing cholangitis (PSC) is associated with a high risk of hepatobiliary malignancy, especially cholangiocarcinoma (CCA). There are no good tumor markers to screen for CCA, and current recommendations for PSC monitoring are mainly based on expert opinions. The optimal strategy to assess disease progression and screen for CCA - the main cause of death of PSC patients - remains unclear.
View Article and Find Full Text PDFNat Rev Drug Discov
January 2025
Institute of Cell Growth Factor, Oujiang Laboratory, Zhejiang Lab for Regenerative Medicine, Vision, and Brain Health, Wenzhou, Zhejiang, China.
The fibroblast growth factor (FGF) family comprises 15 paracrine-acting and 3 endocrine-acting polypeptides, which govern a multitude of processes in human development, metabolism and tissue homeostasis. Therapeutic endocrine FGFs have recently advanced in clinical trials, with FGF19 and FGF21-based therapies on the cusp of approval for the treatment of primary sclerosing cholangitis and metabolic syndrome-associated steatohepatitis, respectively. By contrast, while paracrine FGFs were once thought to be promising drug candidates for wound healing, burns, tissue repair and ischaemic ailments based on their potent mitogenic and angiogenic properties, repeated failures in clinical trials have led to the widespread perception that the development of paracrine FGF-based drugs is not feasible.
View Article and Find Full Text PDFThe liver lymphatic system plays a critical role in maintaining interstitial fluid balance and immune regulation. Efficient lymphatic drainage is essential for liver homeostasis, but its role in liver disease progression remains poorly understood. In cirrhosis, lymphangiogenesis initially compensates for increased lymph production, but impaired lymphatic drainage in advanced stages may lead to complications such as ascites and portal hypertension.
View Article and Find Full Text PDFFront Biosci (Landmark Ed)
January 2025
Department of Surgery, School of Nutrition and Translational Research in Metabolism, Maastricht University, 6200 MD Maastricht, The Netherlands.
Sulfatides or 3-O-sulfogalactosylceramide are negatively charged sulfated glycosphingolipids abundant in the brain and kidneys and play crucial roles in nerve impulse conduction and urinary pH regulation. Sulfatides are present in the liver, specifically in the biliary tract. Sulfatides are self-lipid antigens presented by cholangiocytes to activate cluster of differentiation 1d (CD1d)-restricted type II natural killer T (NKT) cells.
View Article and Find Full Text PDFJ Clin Med
January 2025
Division of Gastroenterology and Hepatology, McGill University Health Centre, Montreal, QC H3G 1A4, Canada.
Inflammatory bowel diseases (IBDs), encompassing Ulcerative Colitis (UC) and Crohn's Disease (CD), are chronic inflammatory disorders affecting the gastrointestinal tract. The association between IBD and colorectal cancer (CRC) is well-documented. Multiple factors have been identified as contributors to the risk of developing CRC in patients with IBD, including duration of disease, disease extension, family history of CRC, co-existance of primary sclerosing cholangitis (PSC), and potentially the presence of post-inflammatory polyps (PIPs).
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