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Recommended procedure of the Czech Society of Hepatology for the diagnosis and treatment of acute porphyrias – update 2022


Authors: Brůha R. 1;  T. Fejfar 2;  Fraňková S. 3;  Husová L. 4,5;  Husa P. 6;  Hůlek P. 2,7;  Šperl J. 3;  Urbánek P. 8;  Vaňásek T. 2;  Vítek L. 9
Authors‘ workplace: IV. interní klinika – klinika gastroenterologie a hepatologie 1. LF UK a VFN v Praze 1;  II. interní gastroenterologická klinika, FN Hradec Králové 2;  Klinika hepatogastroenterologie, Transplantcentrum, IKEM, Praha 3;  Interní gastroenterologická klinika LF MU a FN Brno 4;  Centrum kardiovaskulární a transplantační chirurgie Brno 5;  Klinika infekčních chorob LF MU a FN Brno 6;  Katedra interních oborů, LF OU a FN Ostrava 7;  Interní klinika 1. LF UK a ÚVN Praha 8;  IV. interní klinika a Ústav lékařské bio­chemie a laboratorní dia­gnostiky, 1. LF UK a VFN v Praze 9
Published in: Gastroent Hepatol 2022; 76(2): 143-144
Category: Guidelines
doi: https://doi.org/10.48095/ccgh2022143


Sources

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2. Hirschfield GM, Mason A, Luketic V et al. Efficacy of obeticholic acid in patients with primary biliary cirrhosis and inadequate response to ursodeoxycholic acid. Gastroenterology 2015; 148 (4): 751–761. doi: 10.1053/j.gastro.2014.12.005.

3. Nevens F, Andreone P, Mazzella G et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. N Engl J Med 2016; 375 (7): 631–643. doi: 10.1056/NEJMoa1509840.

4. Kowdley KV, Luketic V, Chapman R et al. Obeticholic Acid PBC Monotherapy Study Group. A randomized trial of obeticholic acid monotherapy in patients with primary biliary cholangitis. Hepatology 2018; 67 (5): 1890–1902. doi: 10.1002/hep.29569.

5. Hirschfield GM, Beuers U, Corpechot C et al. EASL Clinical Practice Guidelines: the dia­gnosis and management of patients with primary biliary cholangitis. J Hepatol 2017; 67 (1): 145–172. doi: 10.1016/j.jhep.2017.03.022.

6. Lindor KD, Bowlus CL, Boyer J et al. Primary biliary cholangitis: 2021 practice guidance update from the American Association for the Study of Liver Diseases. Hepatology 2022; 75 (4): 1012–1013. doi: 10.1002/hep.32117.

7. D’Amato D, De Vincentis A, Malinverno F et al. Real-world experience with obeticholic acid in patients with primary biliary cholangitis. JHEP Rep 2021; 3 (2): 100248. doi: 10.1016/ j.jhepr.2021.100248.

8. Trauner M, Nevens F, Shiffman ML et al. Long-term efficacy and safety of obeticholic acid for patients with primary biliary cholangitis: 3-year results of an international open-label extension study. Lancet Gastroenterol Hepatol 2019; 4 (6): 445–453. doi: 10.1016/S2468-1253 (19) 30094-9.

9. Lindor KD, Bowlus CL, Boyer J et al. Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases. Hepatology 2019; 69 (1): 394–419. doi: 10.1002/hep.30145.

10. Gideon M, Hirschfield GM, Dyson JK et al. The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut 2018; 67 (9): 1568–1594. doi: 10.1136/gutjnl-2017-315259.

11. National Institute for Health and Care Excellence. Obeticholic acid for treating primary biliary cholangitis. Technology appraisal guidance [TA443]. [online]. Dostupné z: https: //www.nice.org.uk/guidance/ta443/.

12. Harms MH, Hirschfield GM, Florean A et al. Obeticholic acid is associated with improvements in AST-to-platelet ratio index and GLOBE score in patients with primary biliary cholangitis. JHEP Rep 2020; 3 (1): 100191. doi: 10.1016/ j.jhepr.2020.100191.

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Paediatric gastroenterology Gastroenterology and hepatology Surgery

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Gastroenterology and Hepatology

Issue 2

2022 Issue 2

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