Non-ceruloplasmin copper and urinary copper in clinically stable Wilson disease: Alignment with recommended targets

Authors: Ott, Peter, Sandahl, Thomas, Ala, Aftab, Cassiman, David, Couchonnal-Bedoya, Eduardo, Cury, Rubens Gisbert, Czlonkowska, Anna, Denk, Gerald, D’Inca, Renata, de Assis Aquino Gondim, Francisco, Moore, Joanna, Poujois, Aurelia, Twardowschy, Carlos Alexandre, Weiss, Karl Heinz, Zuin, Massimo, Kamlin, C.Omar F., Schilsky, Michael L.

Source: In JHEP Reports August 2024 6(8)

Four-year overall survival update from the phase III HIMALAYA study of tremelimumab plus durvalumab in unresectable hepatocellular carcinoma

Authors: Azevedo, Sergio, Braghiroli, Maria Ignez, Girotto, Gustavo, Bragagnoli, Arinilda, Branco, Ricardo, Faccio, Adilson, Moretto, Andrea, Skare, Nils, Dutra, Jamille, Viola, Luciana, Vianna, Karina, Meton, Fernando, Sette, Claudia, Faulhaber, Amanda, Tam, Vincent C., Couture, Felix, Biagi, Jim, Castel, Helene, Mulder, Karen, Ko, Yoo-Joung, Zbuk, Kevin, Welch, Stephen, Beaudoin, Annie, Heurgué, Alexandra, Assenat, Eric, Archambeaud, Isabelle, Tougeron, David, Peron, Jean-Marie, Gilabert, Marine, Bronowicki, Jean-Pierre, Cattan, Stephane, Blanc, Jean-Frederic, Bouattour, Mohamed, Phelip, Jean-Marc, Boige, Valerie, Michel, Pierre, Frin, Anne-Claire, De Toni, Enrico N., Berres, Marie-Luise, Vogel, Arndt, Berg, Thomas, Ettrich, Thomas, Waldschmidt, Dirk, Wedemeyer, Hans Heinrich, Worns, Marcus-Alexander, Bitzer, Michael, Weiss, Karl-Heinz, Lau, George, Chan, Stephen L., Yau, Thomas, Tai, Yin Ping, Lee, Ann Shing, Thungappa, Satheesh Chiradoni, N, Lokesh K., Sureshchand Ostwal, Vikas, Ashok, Kattimani Kiran, Mittal, Sushant, Goyal, Hari, Srinivasan, Sankar, Biswas, Ghanashyam, Mohan, Mallavarapu, Limaye, Sewanti, Asarawala, Nirav, Rimassa, Lorenza, Falcone, Alfredo, Gianni, Luca, Gasbarrini, Antonio, Daniele, Bruno, Avallone, Antonio, Paolo Frassineti, Giovanni Luca, Roila, Fausto, Kudo, Masatoshi, Kawaoka, Tomokazu, Morimoto, Manabu, Takikawa, Yasuhiro, Kato, Naoya, Yamashita, Tatsuya, Osaki, Yukio, Motomura, Kenta, Tateishi, Ryosuke, Ohkawa, Kazuyoshi, Wada, Yoshiyuki, Onishi, Hideki, Sasahira, Naoki, Inaba, Yoshitaka, Kurosaki, Masayuki, Tsuji, Kunihiko, Takei, Yoshiyuki, Aramaki, Takeshi, Hagihara, Atsushi, Furuse, Junji, Kioka, Kiyohide, Koga, Hironori, Sasaki, Yutaka, Numata, Kazushi, Tada, Toshifumi, Kawaguchi, Yasunori, Nadano, Seijin, Vasilyev, Alexander, Breder, Valery, Lipatov, Oleg, Dvorkin, Mikhail, Zarubenkov, Oleg, Kutukova, Svetlana, Ponomarev, Roman, Shostka, Kirill, Alyasova, Anna, Topuzov, Eldar, Severtsev, Alexey, Petrov, Yuryi, Erygin, Dmitriy, Berdov, Boris, Kang, Yoon-Koo, Tak, Won-Young, Park, Joong-Won, Lim, Ho Yeong, Heo, Jeong, Kim, Jee Hyun, Kim, Tae-You, Choi, Hye Jin, Varela, María, Reig Monzon, María Elisa, Sangro, Bruno, Martin, Carlos Gómez, Ponce, Carmen Guillén, López, Carlos, Cheng, Ann-Lii, Chao, Yee, Feng, Yin-Hsun, Jeng, Long-Bin, Hung, Chao-Hung, Hou, Ming-Mo, Wang Tsang-En Wang, Jing-Houng, Yen, Chia-Jui, Sukeepaisarnjaroen, Wattana, Sunpaweravong, Patrapim, Charoentum, Chaiyut, Tanasanvimon, Suebpong, Sirachainan, Ekaphop, Ungtrakul, Teerapat, Prasongsook, Naiyarat, Maneenil, Kunlatida, Jitpewngarm, Wittawat, Ostapenko, Yurii, Skoryi, Denys, Bondarenko, Igor, Shparyk, Yaroslav, Trukhin, Dmytro, Hotko, Yevhen, Ursol, Grygorii, Kryzhanivska, Anna, Abou-Alfa, Ghassan K., Mody, Kabir, Dayyani, Farshid, Al-Rajabi, Raed, Yarchoan, Mark, Gandhi, Sunil, Crysler, Oxana, He, Aiwu Ruth, Reeves, James, Bahary, Nathan, Mahipal, Amit, Kelley, Robin Kate, Dasgupta, Anirudha, Rowe, Julie, Thota, Ramya, Beg, Muhammad, Morse, Michael, Choi, Sung-hee, Crocenzi, Todd, Somer, Bradley, Abrams, Thomas, Denlinger, Crystal, Zhang, Yue, Sharma, Nisha, Dao, Tu V., Thinh, Nguyen Tien, Tuyet Phuong, Le Thi, Sangro, B., Chan, S.L., Kelley, R.K., Lau, G., Kudo, M., Sukeepaisarnjaroen, W., Yarchoan, M., De Toni, E.N., Furuse, J., Kang, Y.K., Galle, P.R., Rimassa, L., Heurgué, A., Tam, V.C., Van Dao, T., Thungappa, S.C., Breder, V., Ostapenko, Y., Reig, M., Makowsky, M., Paskow, M.J., Gupta, C., Kurland, J.F., Negro, A., Abou-Alfa, G.K.

Source: In Annals of Oncology May 2024 35(5):448-457

Neurological worsening in Wilson disease – clinical classification and outcome

Authors: Mohr, Isabelle, Pfeiffenberger, Jan, Eker, Ecem, Merle, Uta, Poujois, Aurélia, Ala, Aftab, Weiss, Karl Heinz

Source: Journal of hepatology. 79(2):321-328

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100194209023.0x000001&indx=1&recIds=ETOCvdc_100194209023.0x000001

A multidisciplinary approach to the diagnosis and management of Wilson disease: Executive summary of the 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases

Authors: Schilsky, Michael L., Roberts, Eve A., Bronstein, Jeff M., Dhawan, Anil, Hamilton, James P., Rivard, Anne Marie, Washington, Mary Kay, Weiss, Karl Heinz, Zimbrean, Paula C.

Source: Hepatology. 77(4):1428-1455

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100187833713.0x000001&indx=1&recIds=ETOCvdc_100187833713.0x000001

Experience on switching trientine formulations in Wilson disease: Efficacy and safety after initiation of TETA 4HCl as substitute for TETA 2HCl

Authors: Mohr, Isabelle, Bourhis, Hélène, Woimant, France, Obadia, Mickael Alexandre, Morgil, Müzeyyen, Morvan, Erwan, Merle, Uta, Denk, Gerald, Poujois, Aurelia, Weiss, Karl Heinz

Source: Journal of gastroenterology and hepatology. 38(2):219-224

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100173472089.0x000001&indx=1&recIds=ETOCvdc_100173472089.0x000001

Trientine tetrahydrochloride versus penicillamine for maintenance therapy in Wilson disease (CHELATE): a randomised, open-label, non-inferiority, phase 3 trial

Authors: To, Uyen, Patel, Amar, Hettiarachchi, Daksshi, Giorgini, Alessia, Monico, Sara, Litwin, Tomasz, Piechal, Agnieszka, Skowronska, Marta, Lachaux, Alain, Belmalih, Abdelouahed, Boogers, Alexandra, Mohr, Isabelle, Langel, Andrea, Freitas, Christian, Barbosa, Egberto Reis, Sandahl, Thomas D, Gerdes, Lisbet, Obadia, Alexandre, Rahli, Djamila, Cosgrove, Jeremy, Schilsky, Michael L ^*, Czlonkowska, Anna, Zuin, Massimo, Cassiman, David, Twardowschy, Carlos, Poujois, Aurelia, Gondim, Francisco de Assis A, Denk, Gerald, Cury, Rubens G, Ott, Peter, Moore, Joanna, Ala, Aftab, D'Inca, Renata, Couchonnal-Bedoya, Eduardo, D'Hollander, Koenraad, Dubois, Nicolas, Kamlin, C Omar F, Weiss, Karl Heinz

Source: In The Lancet Gastroenterology & Hepatology December 2022 7(12):1092-1102

Trientine tetrahydrochloride versus penicillamine for maintenance therapy in Wilson disease (CHELATE): a randomised, open-label, non-inferiority, phase 3 trial

Authors: Schilsky, Michael L, Czlonkowska, Anna, Zuin, Massimo, Cassiman, David, Twardowschy, Carlos, Poujois, Aurelia, Gondim, Francisco de Assis A, Denk, Gerald, Cury, Rubens G, Ott, Peter, Moore, Joanna, Ala, Aftab, D'Inca, Renata, Couchonnal-Bedoya, Eduardo, D'Hollander, Koenraad, Dubois, Nicolas, Kamlin, C Omar F, Weiss, Karl Heinz

Source: Lancet.Gastroenterology & hepatology. 7(12):1092-1102

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100183240859.0x000001&indx=1&recIds=ETOCvdc_100183240859.0x000001

Multicentre, retrospective study to assess long-term outcomes of chelator based treatment with trientine in Wilson disease patients withdrawn from therapy with d-penicillamine

Authors: Weiss, Karl Heinz, Kruse, Carlot, Manolaki, Nina, Zuin, Massimo, Ferenci, Peter, van Scheppingen, Daphne, Wijnberg, Larissa, de Koning, Constance E., Dhawan, Anil

Source: European journal of gastroenterology & hepatology. 34(9):940-947

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100168783137.0x000001&indx=1&recIds=ETOCvdc_100168783137.0x000001

Synonymous mutation in adenosine triphosphatase copper‐transporting beta causes enhanced exon skipping in Wilson disease

Authors: Panzer, Marlene, Viveiros, André, Schaefer, Benedikt, Baumgartner, Nadja, Seppi, Klaus, Djamshidian, Atbin, Todorov, Theodor, Griffiths, William J. H., Schott, Eckart, Schuelke, Markus, Eurich, Dennis, Stättermayer, Albert Friedrich, Bomford, Adrian, Foskett, Pierre, Vodopiutz, Julia, Stauber, Rudolf, Pertler, Elke, Morell, Bernhard, Tilg, Herbert, Müller, Thomas, Kiechl, Stefan, Jimenez‐Heredia, Raul, Weiss, Karl Heinz, Hahn, Si Houn, Janecke, Andreas, Ferenci, Peter, Zoller, Heinz

Source: Hepatology communications. 6(7):1611-1619

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100154052116.0x000001&indx=1&recIds=ETOCvdc_100154052116.0x000001

Hepatitis E seroprevalence, cases and management in a large German centre for liver transplantation

Authors: Nkongolo, Shirin, Mohr, Isabelle, Wenzel, Jürgen J., Khalid, Dina, Mieth, Markus, Mehrabi, Arianeb, Weiss, Karl Heinz, Schnitzler, Paul

Source: Liver Cancer International. 3(2):72-81

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100154067882.0x000001&indx=1&recIds=ETOCvdc_100154067882.0x000001

The rs429358 Locus in Apolipoprotein E Is Associated With Hepatocellular Carcinoma in Patients With Cirrhosis

Authors: Innes, Hamish, Nischalke, Hans Dieter, Guha, Indra Neil, Weiss, Karl Heinz, Irving, Will, Gotthardt, Daniel, Barnes, Eleanor, Fischer, Janett, Ansari, M. Azim, Rosendahl, Jonas, Lin, Shang‐Kuan, Marot, Astrid, Pedergnana, Vincent, Casper, Markus, Benselin, Jennifer, Lammert, Frank, McLauchlan, John, Lutz, Philip L., Hamill, Victoria, Mueller, Sebastian, Morling, Joanne R., Semmler, Georg, Eyer, Florian, von Felden, Johann, Link, Alexander, Vogel, Arndt, Marquardt, Jens U., Sulk, Stefan, Trebicka, Jonel, Valenti, Luca, Datz, Christian, Reiberger, Thomas, Schafmayer, Clemens, Berg, Thomas, Deltenre, Pierre, Hampe, Jochen, Stickel, Felix, Buch, Stephan

Source: Hepatology communications. 6(5):1213-1226

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100148394997.0x000001&indx=1&recIds=ETOCvdc_100148394997.0x000001

A comparative analysis in monitoring 24-hour urinary copper in wilson disease: sampling on or off treatment?

Authors: Mohr, Isabelle, Lamade, Patrick, Weber, Christophe, Leidner, Viola, Köhrer, Sebastian, Olkus, Alexander, Lang, Matthias, Langel, Andrea, Dankert, Patrischia, Greibich, Melanie, Wolf, Silke, Zimmer, Holger, Michl, Patrick, Poujois, Aurélia, Weiss, Karl Heinz, Merle, Uta

Source: Orphanet Journal of Rare Diseases; 1/21/2025, Vol. 20 Issue 1, p1-11, 11p

Subject Terms: STRUCTURED treatment interruption, COPPER, BIOMARKERS, MEDICAL sciences, TREATMENT duration

Infigratinib (BGJ398) in previously treated patients with advanced or metastatic cholangiocarcinoma with FGFR2 fusions or rearrangements: mature results from a multicentre, open-label, single-arm, phase 2 study

Authors: Javle, Milind ^*, Roychowdhury, Sameek, Kelley, Robin Kate, Sadeghi, Saeed, Macarulla, Teresa, Weiss, Karl Heinz, Waldschmidt, Dirk-Thomas, Goyal, Lipika, Borbath, Ivan, El-Khoueiry, Anthony, Borad, Mitesh J, Yong, Wei Peng, Philip, Philip A, Bitzer, Michael, Tanasanvimon, Surbpong, Li, Ai, Pande, Amit, Soifer, Harris S, Shepherd, Stacie Peacock, Moran, Susan, Zhu, Andrew X, Bekaii-Saab, Tanios S, Abou-Alfa, Ghassan K

Source: In The Lancet Gastroenterology & Hepatology October 2021 6(10):803-815

Refining prediction of survival after TIPS with the novel Freiburg index of post-TIPS survival

Authors: Bettinger, Dominik, Sturm, Lukas, Pfaff, Lena, Hahn, Felix, Kloeckner, Roman, Volkwein, Lara, Praktiknjo, Michael, Lv, Yong, Han, Guohong, Huber, Jan Patrick, Boettler, Tobias, Reincke, Marlene, Klinger, Christoph, Caca, Karel, Heinzow, Hauke, Seifert, Leon Louis, Weiss, Karl Heinz, Rupp, Christian, Piecha, Felix, Kluwe, Johannes, Zipprich, Alexander, Luxenburger, Hendrik, Neumann-Haefelin, Christoph, Schmidt, Arthur, Jansen, Christian, Meyer, Carsten, Uschner, Frank E., Brol, Maximilian J., Trebicka, Jonel, Rössle, Martin, Thimme, Robert, Schultheiss, Michael

Source: In Journal of Hepatology June 2021 74(6):1362-1372

HBV-infection rate and long-term outcome after liver-transplantation of anti-HBc-positive liver-grafts to HBV-naïve recipients: A retrospective study

Authors: Hornuss, Daniel, Rudi, Anna, Koerner, Lucas, Mohr, Isabelle, Merle, Uta, Wehling, Cyrill, Rupp, Christian, Dill, Michael T., Golriz, Mohammad, Schnitzler, Paul, Brenner, Thorsten, Mehrabi, Arianeb, Weiss, Karl Heinz, Mieth, Markus ^*

Source: In Clinics and Research in Hepatology and Gastroenterology March 2021 45(2)

Designing Clinical Trials in Wilson's Disease

Authors: Ott, Peter, Ala, Aftab, Askari, Frederick K., Czlonkowska, Anna, Hilgers, Ralf‐Dieter, Poujois, Aurélia, Roberts, Eve A., Sandahl, Thomas Damgaard, Weiss, Karl Heinz, Ferenci, Peter, Schilsky, Michael L.

Source: Hepatology. 74(6):3460-3471

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100135784932.0x000001&indx=1&recIds=ETOCvdc_100135784932.0x000001

Variants in PCSK7, PNPLA3 and TM6SF2 are risk factors for the development of cirrhosis in hereditary haemochromatosis

Authors: Buch, Stephan, Sharma, Aneesh, Ryan, Eleanor, Datz, Christian, Griffiths, William J. H., Way, Michael, Buckley, Thomas W. M., Ryan, John D., Stewart, Stephen, Wright, Callum, Dongiovanni, Paola, Fracanzani, Anna, Zwerina, Jochen, Merle, Uta, Weiss, Karl Heinz, Aigner, Elmar, Krones, Elisabeth, Dejaco, Christian, Fischer, Janett, Berg, Thomas, Valenti, Luca, Zoller, Heinz, McQuillin, Andrew, Hampe, Jochen, Stickel, Felix, Morgan, Marsha Y.

Source: Alimentary pharmacology & therapeutics. 53(7):830-843

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100116432211.0x000001&indx=1&recIds=ETOCvdc_100116432211.0x000001

Optimized Trientine-dihydrochloride Therapy in Pediatric Patients With Wilson Disease: Is Weight-based Dosing Justified?

Authors: Mayr, Toni, Ferenci, Peter, Weiler, Markus, Fichtner, Alexander, Mehrabi, Arianeb, Hoffmann, Georg Friedrich, Mohr, Isabelle, Pfeiffenberger, Jan, Weiss, Karl Heinz, Teufel-Schäfer, Ulrike

Source: Journal of pediatric gastroenterology and nutrition. 72(1):115-122

Availability: http://explore.bl.uk/primo_library/libweb/action/display.do?tabs=detailsTab&gathStatTab=true&ct=display&fn=search&doc=ETOCvdc_100115649782.0x000001&indx=1&recIds=ETOCvdc_100115649782.0x000001

Clinical experience on switching trientine tetrahydrochloride to trientine dihydrochloride in Wilson disease patients.

Authors: Mohr, Isabelle, Schmitt, Timo, Weber, Christophe, Schall, Nicolas, Leidner, Viola Yuriko, Langel, Andrea, Langel, Jessica, Poujois, Aurélia, Weiss, Karl Heinz, Merle, Uta

Source: Journal of Inherited Metabolic Disease Reports; Nov2024, Vol. 65 Issue 6, p406-416, 11p

The MBOAT7 rs641738 variant is associated with an improved outcome in primary sclerosing cholangitis

Authors: Freund, Cora, Wahlers, Arne, Begli, Nima Haji, Leopold, Yvonne, Klöters-Plachky, Petra, Mehrabi, Arianeb, Mohr, Isabelle, Sander, Julia, Rupp, Christian, Gotthardt, Daniel Nils, Weiss, Karl Heinz ^*

Source: In Clinics and Research in Hepatology and Gastroenterology October 2020 44(5):646-652