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Page 1
Fazirsiran for Liver Disease Associated with Alpha1-Antitrypsin Deficiency.
Strnad P, Mandorfer M, Choudhury G, Griffiths W, Trautwein C, Loomba R, Schluep T, Chang T, Yi M, Given BD, Hamilton JC, San Martin J, Teckman JH. Strnad P, et al. Among authors: mandorfer m. N Engl J Med. 2022 Aug 11;387(6):514-524. doi: 10.1056/NEJMoa2205416. Epub 2022 Jun 25. N Engl J Med. 2022. PMID: 35748699 Clinical Trial.
Heterozygous carriage of the alpha1-antitrypsin Pi*Z variant increases the risk to develop liver cirrhosis.
Strnad P, Buch S, Hamesch K, Fischer J, Rosendahl J, Schmelz R, Brueckner S, Brosch M, Heimes CV, Woditsch V, Scholten D, Nischalke HD, Janciauskiene S, Mandorfer M, Trauner M, Way MJ, McQuillin A, Reichert MC, Krawczyk M, Casper M, Lammert F, Braun F, von Schönfels W, Hinz S, Burmeister G, Hellerbrand C, Teufel A, Feldman A, Schattenberg JM, Bantel H, Pathil A, Demir M, Kluwe J, Boettler T, Ridinger M, Wodarz N, Soyka M, Rietschel M, Kiefer F, Weber T, Marhenke S, Vogel A, Hinrichsen H, Canbay A, Schlattjan M, Sosnowsky K, Sarrazin C, von Felden J, Geier A, Deltenre P, Sipos B, Schafmayer C, Nothnagel M, Aigner E, Datz C, Stickel F, Morgan MY, Hampe J, Berg T, Trautwein C. Strnad P, et al. Among authors: mandorfer m. Gut. 2019 Jun;68(6):1099-1107. doi: 10.1136/gutjnl-2018-316228. Epub 2018 Aug 1. Gut. 2019. PMID: 30068662
Liver Fibrosis and Metabolic Alterations in Adults With alpha-1-antitrypsin Deficiency Caused by the Pi*ZZ Mutation.
Hamesch K, Mandorfer M, Pereira VM, Moeller LS, Pons M, Dolman GE, Reichert MC, Schneider CV, Woditsch V, Voss J, Lindhauer C, Fromme M, Spivak I, Guldiken N, Zhou B, Arslanow A, Schaefer B, Zoller H, Aigner E, Reiberger T, Wetzel M, Siegmund B, Simões C, Gaspar R, Maia L, Costa D, Bento-Miranda M, van Helden J, Yagmur E, Bzdok D, Stolk J, Gleiber W, Knipel V, Windisch W, Mahadeva R, Bals R, Koczulla R, Barrecheguren M, Miravitlles M, Janciauskiene S, Stickel F, Lammert F, Liberal R, Genesca J, Griffiths WJ, Trauner M, Krag A, Trautwein C, Strnad P; European Alpha1-Liver Study Group. Hamesch K, et al. Among authors: mandorfer m. Gastroenterology. 2019 Sep;157(3):705-719.e18. doi: 10.1053/j.gastro.2019.05.013. Epub 2019 May 20. Gastroenterology. 2019. PMID: 31121167 Free article.
Liver Phenotypes of European Adults Heterozygous or Homozygous for Pi∗Z Variant of AAT (Pi∗MZ vs Pi∗ZZ genotype) and Noncarriers.
Schneider CV, Hamesch K, Gross A, Mandorfer M, Moeller LS, Pereira V, Pons M, Kuca P, Reichert MC, Benini F, Burbaum B, Voss J, Gutberlet M, Woditsch V, Lindhauer C, Fromme M, Kümpers J, Bewersdorf L, Schaefer B, Eslam M, Bals R, Janciauskiene S, Carvão J, Neureiter D, Zhou B, Wöran K, Bantel H, Geier A, Dirrichs T, Stickel F, Teumer A, Verbeek J, Nevens F, Govaere O, Krawczyk M, Roskams T, Haybaeck J, Lurje G, Chorostowska-Wynimko J, Genesca J, Reiberger T, Lammert F, Krag A, George J, Anstee QM, Trauner M, Datz C, Gaisa NT, Denk H, Trautwein C, Aigner E, Strnad P; European Alpha-1 Liver Study Group. Schneider CV, et al. Among authors: mandorfer m. Gastroenterology. 2020 Aug;159(2):534-548.e11. doi: 10.1053/j.gastro.2020.04.058. Epub 2020 May 4. Gastroenterology. 2020. PMID: 32376409
The PREDICT study uncovers three clinical courses of acutely decompensated cirrhosis that have distinct pathophysiology.
Trebicka J, Fernandez J, Papp M, Caraceni P, Laleman W, Gambino C, Giovo I, Uschner FE, Jimenez C, Mookerjee R, Gustot T, Albillos A, Bañares R, Janicko M, Steib C, Reiberger T, Acevedo J, Gatti P, Bernal W, Zeuzem S, Zipprich A, Piano S, Berg T, Bruns T, Bendtsen F, Coenraad M, Merli M, Stauber R, Zoller H, Ramos JP, Solè C, Soriano G, de Gottardi A, Gronbaek H, Saliba F, Trautwein C, Özdogan OC, Francque S, Ryder S, Nahon P, Romero-Gomez M, Van Vlierberghe H, Francoz C, Manns M, Garcia E, Tufoni M, Amoros A, Pavesi M, Sanchez C, Curto A, Pitarch C, Putignano A, Moreno E, Shawcross D, Aguilar F, Clària J, Ponzo P, Jansen C, Vitalis Z, Zaccherini G, Balogh B, Vargas V, Montagnese S, Alessandria C, Bernardi M, Ginès P, Jalan R, Moreau R, Angeli P, Arroyo V; PREDICT STUDY group of the EASL-CLIF Consortium. Trebicka J, et al. J Hepatol. 2020 Oct;73(4):842-854. doi: 10.1016/j.jhep.2020.06.013. Epub 2020 Jul 13. J Hepatol. 2020. PMID: 32673741 Free article.
Serum keratin 19 (CYFRA21-1) links ductular reaction with portal hypertension and outcome of various advanced liver diseases.
Hamesch K, Guldiken N, Aly M, Hüser N, Hartmann D, Rufat P, Ziol M, Remih K, Lurje G, Scheiner B, Trautwein C, Mandorfer M, Reiberger T, Mueller S, Bruns T, Nahon P, Strnad P. Hamesch K, et al. Among authors: mandorfer m. BMC Med. 2020 Nov 12;18(1):336. doi: 10.1186/s12916-020-01784-7. BMC Med. 2020. PMID: 33176798 Free PMC article.
PREDICT identifies precipitating events associated with the clinical course of acutely decompensated cirrhosis.
Trebicka J, Fernandez J, Papp M, Caraceni P, Laleman W, Gambino C, Giovo I, Uschner FE, Jansen C, Jimenez C, Mookerjee R, Gustot T, Albillos A, Bañares R, Jarcuska P, Steib C, Reiberger T, Acevedo J, Gatti P, Shawcross DL, Zeuzem S, Zipprich A, Piano S, Berg T, Bruns T, Danielsen KV, Coenraad M, Merli M, Stauber R, Zoller H, Ramos JP, Solé C, Soriano G, de Gottardi A, Gronbaek H, Saliba F, Trautwein C, Kani HT, Francque S, Ryder S, Nahon P, Romero-Gomez M, Van Vlierberghe H, Francoz C, Manns M, Garcia-Lopez E, Tufoni M, Amoros A, Pavesi M, Sanchez C, Praktiknjo M, Curto A, Pitarch C, Putignano A, Moreno E, Bernal W, Aguilar F, Clària J, Ponzo P, Vitalis Z, Zaccherini G, Balogh B, Gerbes A, Vargas V, Alessandria C, Bernardi M, Ginès P, Moreau R, Angeli P, Jalan R, Arroyo V; PREDICT STUDY group of the EASL-CLIF CONSORTIUM. Trebicka J, et al. J Hepatol. 2021 May;74(5):1097-1108. doi: 10.1016/j.jhep.2020.11.019. Epub 2020 Nov 20. J Hepatol. 2021. PMID: 33227350 Free article.
Hepatobiliary phenotypes of adults with alpha-1 antitrypsin deficiency.
Fromme M, Schneider CV, Pereira V, Hamesch K, Pons M, Reichert MC, Benini F, Ellis P, H Thorhauge K, Mandorfer M, Burbaum B, Woditsch V, Chorostowska-Wynimko J, Verbeek J, Nevens F, Genesca J, Miravitlles M, Nuñez A, Schaefer B, Zoller H, Janciauskiene S, Abreu N, Jasmins L, Gaspar R, Liberal R, Macedo G, Mahadeva R, Gomes C, Schneider KM, Trauner M, Krag A, Gooptu B, Thorburn D, Marshall A, Hurst JR, Lomas DA, Lammert F, Gaisa NT, Clark V, Griffiths W, Trautwein C, Turner AM, McElvaney NG, Strnad P. Fromme M, et al. Among authors: mandorfer m. Gut. 2022 Feb;71(2):415-423. doi: 10.1136/gutjnl-2020-323729. Epub 2021 Feb 25. Gut. 2022. PMID: 33632708
Alpha-1 Antitrypsin Augmentation and the Liver Phenotype of Adults With Alpha-1 Antitrypsin Deficiency (Genotype Pi∗ZZ).
Fromme M, Hamesch K, Schneider CV, Mandorfer M, Pons M, Thorhauge KH, Pereira V, Sperl J, Frankova S, Reichert MC, Benini F, Burbaum B, Kleinjans M, Amzou S, Rademacher L, Bewersdorf L, Verbeek J, Nevens F, Genesca J, Miravitlles M, Nuñez A, Schaefer B, Zoller H, Janciauskiene S, Waern J, Oliveira A, Maia L, Simões C, Mahadeva R, Fraughen DD, Trauner M, Krag A, Lammert F, Bals R, Gaisa NT, Aigner E, Griffiths WJ, Denk H, Teumer A, McElvaney NG, Turner AM, Trautwein C, Strnad P. Fromme M, et al. Among authors: mandorfer m. Clin Gastroenterol Hepatol. 2024 Feb;22(2):283-294.e5. doi: 10.1016/j.cgh.2023.08.038. Epub 2023 Sep 15. Clin Gastroenterol Hepatol. 2024. PMID: 37716616
302 results