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Up-to-Date Snapshot of Current and Emerging Medical Therapies in Primary Biliary Cholangitis.
J Pers Med
November 2024
Healthpoint Hospital, Abu Dhabi 112308, United Arab Emirates.
Primary biliary cholangitis (PBC) is an autoimmune chronic cholestatic disease of the liver that symptomatically can present with pruritus and fatigue. Its established first- and second-line therapies are ursodeoxycholic acid (UDCA) and obeticholic acid (OCA) although they provide limited symptom management. Liver transplantation offers a potentially curative therapeutic option in refractory cases progressing to cirrhosis.
View Article and Find Full Text PDFSecond-Line Treatment for Patients With Primary Biliary Cholangitis: A Systematic Review With Network Meta-Analysis.
Liver Int
January 2025
Liver Center, Digestive Diseases Section, Department of Internal Medicine, Yale University School of Medicine, New Haven, Connecticut, USA.
Background & Aims: Approximately 40% of patients with Primary Biliary Cholangitis (PBC) show incomplete response to ursodeoxycholic acid, thus needing second-line treatment to prevent disease progression. As no head-to-head comparison study is available, we used a network meta-analysis (NMA) to compare efficacy and safety of available second-line therapies.
Methods: We performed a systematic literature review including randomised, placebo-controlled trials of patients with PBC and incomplete response, or intolerance, to ursodeoxycholic acid, and compared relative risks (RRs) for primary (biochemical response at 52-week) and secondary outcomes [incidence of new-onset pruritus and serious adverse events (SAEs)].
Primary biliary cholangitis: Personalizing second-line therapies.
Hepatology
November 2024
Department of Medicine, Division of Gastroenterology and Hepatology, University of California Davis School of Medicine, Sacramento, California, USA.
Article Synopsis
- Primary biliary cholangitis (PBC) is an autoimmune disease primarily affecting middle-aged women, characterized by damage to small bile ducts, leading to liver issues like cholestasis and cirrhosis.
- The main treatment for PBC has been ursodeoxycholic acid, which helps slow disease progression but doesn't alleviate symptoms; new second-line therapies are being developed for patients who don't respond well.
- Recent medications like obeticholic acid, elafibranor, and seladelpar show promise in improving liver function and reducing cholestasis, while considerations for personalized treatment based on patient features are essential.
Protective effects of the PPAR agonist bezafibrate against disruption of redox and energy homeostasis, neuronal death, astroglial reactivity, and neuroinflammation induced in vivo by D-2-hydroxyglutaric acid in rat brain.
Eur J Pharmacol
January 2025
Postgraduation Program in Biological Sciences: Biochemistry, Institute of Basic Health Sciences, Universidade Federal do Rio Grande do Sul, Porto Alegre, RS, Brazil; Medical Genetics Service, Hospital de Clínicas de Porto Alegre, Porto Alegre, RS, 90035-007, Brazil. Electronic address:
The biochemical hallmark of D-2-hydroxyglutaric aciduria is brain accumulation of D-2-hydroxyglutaric acid (D2HG). Patients present predominantly neurological manifestations, whose pathogenesis is still unknown. Thus, we examined the impact of elevated brain levels of D2HG, induced by intracerebral injection of this metabolite in juvenile rats, on redox and mitochondrial homeostasis and histochemical landmarks in the cerebral cortex.
View Article and Find Full Text PDFReview of Current and Upcoming Second-Line Treatments for Primary Biliary Cholangitis.
Dig Dis Sci
January 2025
Department of Medicine and Surgery, David Geffen School of Medicine at UCLA, Los Angeles, CA, USA.
Background: Treatment for primary biliary cholangitis (PBC) was defined by its singular relationship with ursodeoxycholic acid (UDCA) for decades. However, nearly 40% of patients fail to achieve adequate biochemical response with UDCA, necessitating second-line therapies.
Aims: The aim of our review was to assess the efficacy and safety of second-line therapies for PBC from phase three trials.
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