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Lifetime Data and Concepts
Published in Prabhanjan Narayanachar Tattar, H. J. Vaman, Survival Analysis, 2022
Prabhanjan Narayanachar Tattar, H. J. Vaman
Mayo Clinic has conducted many clinical trials and a popular dataset widely used is the primary biliary cirrhosis (PBC) of the liver conducted during the period 1974–84. The primary biliary cirrhosis problem is known in recent times as primary biliary cholangitis. It is the autoimmune disease of liver. This problem arises because of a slow and progressive destruction of the small bile ducts of the liver leading to a build-up of bile and other toxins in the liver.
Inflammation and immunology
Published in C. Simon Herrington, Muir's Textbook of Pathology, 2020
Christopher Bellamy, Stephen J. Jenkins, Henry J. McSorley, David A. Dorward, Timothy J. Kendall
Primary biliary cholangitis (formerly called primary biliary cirrhosis) is a chronic progressive disease of the liver with good evidence of an autoimmune aetiology. Although autoantibodies to mitochondria are present, the pattern of destruction of intrahepatic bile ducts is typical of type IV hypersensitivity. Autoreactive T lymphocytes directed against antigens on the epithelium of the bile duct trigger the activation of macrophages and formation of a granulomatous response. This leads to progressive destruction of bile ducts, obstruction to biliary secretion, and fibrosis leading to cirrhosis and liver failure.
Health-related quality of life questionnaires used in primary biliary cholangitis: a systematic review
Published in Scandinavian Journal of Gastroenterology, 2022
Xin Ai, Xian Yang, Hai-yan Fu, Jia-min Xu, Ying-mei Tang
Primary biliary cholangitis (PBC) is an autoimmune disease that is characterized by interlobular cholangitis with progressive destruction, cholestasis, and fibrosis, and which may eventually develop into end-stage liver disease [1,2]. High-titre specific anti-mitochondrial antibodies and the anti-nuclear antibodies GP120 and SP100 appear in the serum of PBC patients, and the level of IgM in peripheral blood increases correspondingly [3]. The most common symptoms of primary biliary cholangitis are fatigue and pruritus, which seriously affect patients’ quality of life. Ursodeoxycholic acid (UDCA) is the first valid drug in the treatment of PBC, but 30% of patients with PBC do not respond to treatment with it [4,5]. The World Health Organization (WHO) defines quality of life as individuals’ perception of their position in life in the context of the culture and value systems in which they live and in relation to their goals, expectations, standards and concerns [6]. More specifically, health-related quality of life (HRQOL) is defined as the influence of health and disease on individual’s quality of life [7]. HRQOL measures are necessary for chronic disease management as they assess the overall impact of these diseases on health from the patient’s perspective. A range of different questionnaires, both disease-specific and generic, have been used to evaluate HRQOL in PBC; however, there is no current guidance as to which HRQOL questionnaires clinicians and researchers should implement. The aim of this review was to evaluate the suitability of questionnaires used to assess HRQOL in primary biliary cholangitis.
Pharmaceutical suspensions of ursodeoxycholic acid for pediatric patients: in vitro and in vivo studies
Published in Pharmaceutical Development and Technology, 2021
Oriana Boscolo, Leandro Salvo, Cecilia Dobrecky, Eliana N. Fissore, Fabian Buontempo, Valeria Tripodi, Silvia E. Lucangioli
Ursodeoxycholic acid (UDCA) (3α, 7β-dihydroxy-5β-cholan-24-oic acid), or ursodiol is approved as therapeutic agent for hepatobiliary disorders. Treatment with oral UDCA ameliorates histological features of liver diseases and restores the biochemical parameters in preschoolers and children with a diversity of cholestatic disorders (Setchell et al. 2005), such as benign recurrent intrahepatic cholestasis, progressive familiar intrahepatic cholestasis, biliary atresia, Alagille syndrome, bile acid synthesis impairment causing cholestasis inborn, long-term parenteral nutrition and cystic fibrosis-associated liver disease. Primary biliary cholangitis pathology (Melchor-Mendoza et al. 2017; Lindor et al. 2019) is an autoimmune type disease in which the progressive destruction of the intrahepatic bile ducts that can cause biliary cirrhosis, portal inflammation and finally liver failure. Up to 2016, UDCA was the only drug granted approval by the U.S. Food and Drug Administration for treatment of primary biliary cholangitis. In this case, UDCA administration improves survival without transplantation and is consequently the preliminary drug of choice for this pathology (Melchor-Mendoza et al. 2017; Lindor et al. 2019).
Old and novel prognostic biomarkers in primary biliary cholangitis
Published in Expert Opinion on Orphan Drugs, 2021
G Mulinacci, A Palermo, Pietro Invernizzi, Marco Carbone
Primary biliary cholangitis (PBC) is a chronic liver pathology characterized by intrahepatic biliary obstruction and chronic cholestasis potentially leading to end-stage liver disease and its associated complications [1,2]. It is clinically characterized by chronic cholestasis, serologic reactivity to antimitochondrial antibodies (AMA) or specific antinuclear antibody (ANA), gp210 and sp100, and histologic evidence of chronic non-suppurative, granulomatous cholangitis. The disease is generally slowly progressive, but many patients eventually develop end-stage liver disease with attendant need for liver transplantation (LT). Epidemiologic studies report heterogeneous incidence rates of 0.33 to 5.8 per 100.000 inhabitants per year, and prevalence rates of 1.9 to 40.2 per 100.000 inhabitants. Such figures, in particular the prevalence, have shown some increasing in the last decades. Improvement of diagnostic tools, increasing disease awareness, and digitalized patient registration with easing of case-findings, along with an improved survival likely contributed to the rising prevalence rates.