Primary biliary cirrhosis

Primary biliary cirrhosis (PBC) is an immune-mediated chronic cholestatic liver disease with a slowly progressive course. Without treatment, most patients eventually develop fibrosis and cirrhosis of the liver and may need liver transplantation in the late stage of disease. PBC primarily affects women (female preponderance 9–10:1) with a prevalence of up to 1 in 1,000 women over 40 years of age. Common symptoms of the disease are fatigue and pruritus, but most patients are asymptomatic at first presentation. The diagnosis is based on sustained elevation of serum markers of cholestasis, i.e., alkaline phosphatase and gamma-glutamyl transferase, and the presence of serum antimitochondrial antibodies directed against the E2 subunit of the pyruvate dehydrogenase complex. Histologically, PBC is characterized by florid bile duct lesions with damage to biliary epithelial cells, an often dense portal inflammatory infiltrate and progressive loss of small intrahepatic bile ducts. Although the insight into pathogenetic aspects of PBC has grown enormously during the recent decade and numerous genetic, environmental, and infectious factors have been disclosed which may contribute to the development of PBC, the precise pathogenesis remains enigmatic. Ursodeoxycholic acid (UDCA) is currently the only FDA-approved medical treatment for PBC. When administered at adequate doses of 13–15 mg/kg/day, up to two out of three patients with PBC may have a normal life expectancy without additional therapeutic measures. The mode of action of UDCA is still under discussion, but stimulation of impaired hepatocellular and cholangiocellular secretion, detoxification of bile, and antiapoptotic effects may represent key mechanisms. One out of three patients does not adequately respond to UDCA therapy and may need additional medical therapy and/or liver transplantation. This review summarizes current knowledge on the clinical, diagnostic, pathogenetic, and therapeutic aspects of PBC

Erratum to: 36th International Symposium on Intensive Care and Emergency Medicine

[This corrects the article DOI: 10.1186/s13054-016-1208-6.]

Variation in Transfusion Practice and How to Influence Clinicians' Use of Blood in Hospitals

Blood components are scarce and costly resources that are required for almost every area of hospital practice. Most quality improvement activity in transfusion has centred on small incremental improvements in safety of donated blood for transfusion, although increasing attention is now focusing on improving blood use by clinicians to be consistent with evidence-based recommendations. Audits of transfusion provide information on (variable) compliance with standards and levels of unnecessary transfusions and findings continue to show unnecessary use of blood outside established guidelines. The translation of research findings into hospital practice is often slow and haphazard and this applies to transfusion as well as other branches of healthcare. Many different interventions are undertaken by hospitals to change transfusion practice, but there are real uncertainties about their effectiveness and durability. There is a need for research to define the determinants of transfusion behaviour to guide the design and selection of interventions better in order to deliver optimal change in transfusion practice. This edition first published 2013 © 2001, 2005, 2009, 2013 John Wiley and Sons Ltd

Variation in Transfusion Practice and How to Influence Clinicians' Use of Blood in Hospitals

Blood components are scarce and costly resources that are required for almost every area of hospital practice. Most quality improvement activity in transfusion has centred on small incremental improvements in safety of donated blood for transfusion, although increasing attention is now focusing on improving blood use by clinicians to be consistent with evidence-based recommendations. Audits of transfusion provide information on (variable) compliance with standards and levels of unnecessary transfusions and findings continue to show unnecessary use of blood outside established guidelines. The translation of research findings into hospital practice is often slow and haphazard and this applies to transfusion as well as other branches of healthcare. Many different interventions are undertaken by hospitals to change transfusion practice, but there are real uncertainties about their effectiveness and durability. There is a need for research to define the determinants of transfusion behaviour to guide the design and selection of interventions better in order to deliver optimal change in transfusion practice. This edition first published 2013 © 2001, 2005, 2009, 2013 John Wiley and Sons Ltd