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2. Incidence and predictors of hepatocellular carcinoma in patients with autoimmune hepatitis

Author

van Gerven, N., van Erpecum, K., Ouden, J den, Brouwer, J., Vrolijk, J., Gevers, T.J., Drenth, J., Guichelaar, M., Bouma, G., Schreuder, T.C.M.A., van der Wouden, E.J., Baak, L.C., Stadhouders, P., Klemt-Kropp, M., Verhagen, M., Bhalla, A., Kuijvenhoven, J., Almasio, P., Alvarez, F., Andrade, R., Arikan, C., Assis, D., Bardou-Jacquet, E., Biewenga, M., Cancado, E., Cazzagon, N., Chazouillères, O., Colloredo, G., Cuarterolo, M., Dalekos, G., Debray, D., Robles-Díaz, M., Dyson, J., Efe, C., Engel, B., Ferri, S., Fontana, R., Gatselis, N., Gerussi, A., Halilbasic, E., Halliday, N., Heneghan, M., Hirschfield, G., van Hoek, B., Hørby Jørgensen, M., Indolfini, G., Iorio, R., Invernizzi, P., Jeong, S., Jones, D., Kelly, D., Kerkar, N., Lacaille, F., Lammert, C., Leggett, B., Lenzi, M., Levy, C., Liberal, R., Lleo, A., Lohse, A., Lopez, S. Ines, de Martin, E., McLin, V., Mieli-Vergani, G., Milkiewicz, P., Mohan, N., Muratori, L., Nebbia, G., van Nieuwkerk, C., Oo, Y., Ortega, A., Páres, A., Pop, T., Pratt, D., Purnak, T., Ranucci, G., Rushbrook, S., Schramm, C., Stättermayer, A., Swain, M., Tanaka, A., Taubert, R., Terrabuio, D., Terziroli, B., Trauner, M., Valentino, P., van den Brand, F., Vergani, D., Villamil, A., Wahlin, S., Ytting, H., Zachou, K., Zeniya, M., Colapietro, Francesca, Maisonneuve, Patrick, Lytvyak, Ellina, Beuers, Ulrich, Verdonk, Robert C., van der Meer, Adriaan J., van Hoek, Bart, Kuiken, Sjoerd D., Brouwer, Johannes T., Muratori, Paolo, Aghemo, Alessio, Carella, Francesco, van den Berg, Ad P., Zachou, Kalliopi, Dalekos, George N., Di Zeo-Sánchez, Daniel E., Robles, Mercedes, Andrade, Raul J., Montano-Loza, Aldo J., van den Brand, Floris F., Slooter, Charlotte D., Macedo, Guilherme, Liberal, Rodrigo, de Boer, Ynto S., and Lleo, Ana

Published
2024
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3. Event-free survival of maralixibat-treated patients with Alagille syndrome compared to a real-world cohort from GALA

Author

Hansen, B, Vandriel, S, Vig, P, Garner, W, Mogul, D, Loomes, K, Piccoli, D, Rand, E, Jankowska, I, Czubkowski, P, Gliwicz-Miedzinska, D, Gonzales, E, Jacquemin, E, Bouligand, J, D'Antiga, L, Nicastro, E, Arnell, H, Fischler, B, Sokal, E, Demaret, T, Siew, S, Stormon, M, Karpen, S, Romero, R, Ebel, N, Feinstein, J, Roberts, A, Evans, H, Sundaram, S, Chaidez, A, Hardikar, W, Shankar, S, Fischer, R, Lacaille, F, Debray, D, Lin, H, Jensen, M, Jaramillo, C, Karthikeyan, P, Indolfi, G, Verkade, H, Larson-Nath, C, Quiros-Tejeira, R, Valentino, P, Rogalidou, M, Dezsofi, A, Squires, J, Schwarz, K, Calvo, P, Bernabeu, J, Zizzo, A, Nebbia, G, Bulut, P, Santos-Silva, E, Fawaz, R, Nastasio, S, Karnsakul, W, Tamara, M, Busoms, C, Kelly, D, Sandahl, T, Jimenez-Rivera, C, Banales, J, Mujawar, Q, Li, L, She, H, Wang, J, Kim, K, Oh, S, Sanchez, M, Cavalieri, M, Lee, W, Hajinicolaou, C, Lertudomphonwanit, C, Waisbourd-Zinman, O, Arikan, C, Alam, S, Carvalho, E, Melere, M, Eshun, J, Onal, Z, Desai, D, Wiecek, S, Pinto, R, Wolters, V, Garcia, J, Beretta, M, Kerkar, N, Brecelj, J, Rock, N, Lurz, E, Blondet, N, Shah, U, Thompson, R, Kamath, B, Hansen B. E., Vandriel S. M., Vig P., Garner W., Mogul D. B., Loomes K. M., Piccoli D. A., Rand E. B., Jankowska I., Czubkowski P., Gliwicz-Miedzinska D., Gonzales E. M., Jacquemin E., Bouligand J., D'Antiga L., Nicastro E., Arnell H., Fischler B., Sokal E., Demaret T., Siew S., Stormon M., Karpen S. J., Romero R., Ebel N. H., Feinstein J. A., Roberts A. J., Evans H. M., Sundaram S. S., Chaidez A., Hardikar W., Shankar S., Fischer R. T., Lacaille F., Debray D., Lin H. C., Jensen M. K., Jaramillo C., Karthikeyan P., Indolfi G., Verkade H. J., Larson-Nath C., Quiros-Tejeira R. E., Valentino P. L., Rogalidou M., Dezsofi A., Squires J. E., Schwarz K., Calvo P. L., Bernabeu J. Q., Zizzo A. N., Nebbia G., Bulut P., Santos-Silva E., Fawaz R., Nastasio S., Karnsakul W., Tamara M. L., Busoms C. M., Kelly D. A., Sandahl T. D., Jimenez-Rivera C., Banales J. M., Mujawar Q., Li L. -T., She H., Wang J. -S., Kim K. M., Oh S. H., Sanchez M. C., Cavalieri M. L., Lee W. S., Hajinicolaou C., Lertudomphonwanit C., Waisbourd-Zinman O., Arikan C., Alam S., Carvalho E., Melere M., Eshun J., Onal Z., Desai D. M., Wiecek S., Pinto R. B., Wolters V. M., Garcia J., Beretta M., Kerkar N., Brecelj J., Rock N., Lurz E., Blondet N., Shah U., Thompson R. J., Kamath B. M., Hansen, B, Vandriel, S, Vig, P, Garner, W, Mogul, D, Loomes, K, Piccoli, D, Rand, E, Jankowska, I, Czubkowski, P, Gliwicz-Miedzinska, D, Gonzales, E, Jacquemin, E, Bouligand, J, D'Antiga, L, Nicastro, E, Arnell, H, Fischler, B, Sokal, E, Demaret, T, Siew, S, Stormon, M, Karpen, S, Romero, R, Ebel, N, Feinstein, J, Roberts, A, Evans, H, Sundaram, S, Chaidez, A, Hardikar, W, Shankar, S, Fischer, R, Lacaille, F, Debray, D, Lin, H, Jensen, M, Jaramillo, C, Karthikeyan, P, Indolfi, G, Verkade, H, Larson-Nath, C, Quiros-Tejeira, R, Valentino, P, Rogalidou, M, Dezsofi, A, Squires, J, Schwarz, K, Calvo, P, Bernabeu, J, Zizzo, A, Nebbia, G, Bulut, P, Santos-Silva, E, Fawaz, R, Nastasio, S, Karnsakul, W, Tamara, M, Busoms, C, Kelly, D, Sandahl, T, Jimenez-Rivera, C, Banales, J, Mujawar, Q, Li, L, She, H, Wang, J, Kim, K, Oh, S, Sanchez, M, Cavalieri, M, Lee, W, Hajinicolaou, C, Lertudomphonwanit, C, Waisbourd-Zinman, O, Arikan, C, Alam, S, Carvalho, E, Melere, M, Eshun, J, Onal, Z, Desai, D, Wiecek, S, Pinto, R, Wolters, V, Garcia, J, Beretta, M, Kerkar, N, Brecelj, J, Rock, N, Lurz, E, Blondet, N, Shah, U, Thompson, R, Kamath, B, Hansen B. E., Vandriel S. M., Vig P., Garner W., Mogul D. B., Loomes K. M., Piccoli D. A., Rand E. B., Jankowska I., Czubkowski P., Gliwicz-Miedzinska D., Gonzales E. M., Jacquemin E., Bouligand J., D'Antiga L., Nicastro E., Arnell H., Fischler B., Sokal E., Demaret T., Siew S., Stormon M., Karpen S. J., Romero R., Ebel N. H., Feinstein J. A., Roberts A. J., Evans H. M., Sundaram S. S., Chaidez A., Hardikar W., Shankar S., Fischer R. T., Lacaille F., Debray D., Lin H. C., Jensen M. K., Jaramillo C., Karthikeyan P., Indolfi G., Verkade H. J., Larson-Nath C., Quiros-Tejeira R. E., Valentino P. L., Rogalidou M., Dezsofi A., Squires J. E., Schwarz K., Calvo P. L., Bernabeu J. Q., Zizzo A. N., Nebbia G., Bulut P., Santos-Silva E., Fawaz R., Nastasio S., Karnsakul W., Tamara M. L., Busoms C. M., Kelly D. A., Sandahl T. D., Jimenez-Rivera C., Banales J. M., Mujawar Q., Li L. -T., She H., Wang J. -S., Kim K. M., Oh S. H., Sanchez M. C., Cavalieri M. L., Lee W. S., Hajinicolaou C., Lertudomphonwanit C., Waisbourd-Zinman O., Arikan C., Alam S., Carvalho E., Melere M., Eshun J., Onal Z., Desai D. M., Wiecek S., Pinto R. B., Wolters V. M., Garcia J., Beretta M., Kerkar N., Brecelj J., Rock N., Lurz E., Blondet N., Shah U., Thompson R. J., and Kamath B. M.

Abstract

BACKGROUND AND AIMS: Alagille syndrome (ALGS) is characterized by chronic cholestasis with associated pruritus and extrahepatic anomalies. Maralixibat, an ileal bile acid transporter inhibitor, is an approved pharmacologic therapy for cholestatic pruritus in ALGS. Since long-term placebo-controlled studies are not feasible or ethical in children with rare diseases, a novel approach was taken comparing 6-year outcomes from maralixibat trials with an aligned and harmonized natural history cohort from the G lobal AL agille A lliance (GALA) study. APPROACH AND RESULTS: Maralixibat trials comprise 84 patients with ALGS with up to 6 years of treatment. GALA contains retrospective data from 1438 participants. GALA was filtered to align with key maralixibat eligibility criteria, yielding 469 participants. Serum bile acids could not be included in the GALA filtering criteria as these are not routinely performed in clinical practice. Index time was determined through maximum likelihood estimation in an effort to align the disease severity between the two cohorts with the initiation of maralixibat. Event-free survival, defined as the time to first event of manifestations of portal hypertension (variceal bleeding, ascites requiring therapy), surgical biliary diversion, liver transplant, or death, was analyzed by Cox proportional hazards methods. Sensitivity analyses and adjustments for covariates were applied. Age, total bilirubin, gamma-glutamyl transferase, and alanine aminotransferase were balanced between groups with no statistical differences. Event-free survival in the maralixibat cohort was significantly better than the GALA cohort (HR, 0.305; 95% CI, 0.189-0.491; p <0.0001). Multiple sensitivity and subgroup analyses (including serum bile acid availability) showed similar findings. CONCLUSIONS: This study demonstrates a novel application of a robust statistical method to evaluate outcomes in long-term intervention studies where placebo comparisons are not feasible, p

Published
2024

4. Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study

Author

Vandriel, S, Li, L, She, H, Wang, J, Gilbert, M, Jankowska, I, Czubkowski, P, Gliwicz-Miedzinska, D, Gonzales, E, Jacquemin, E, Bouligand, J, Spinner, N, Loomes, K, Piccoli, D, D'Antiga, L, Nicastro, E, Sokal, A, Demaret, T, Ebel, N, Feinstein, J, Fawaz, R, Nastasio, S, Lacaille, F, Debray, D, Arnell, H, Fischler, B, Siew, S, Stormon, M, Karpen, S, Romero, R, Kim, K, Baek, W, Hardikar, W, Shankar, S, Roberts, A, Evans, H, Jensen, M, Kavan, M, Sundaram, S, Chaidez, A, Karthikeyan, P, Sanchez, M, Cavalieri, M, Verkade, H, Lee, W, Squires, J, Hajinicolaou, C, Lertudomphonwanit, C, Fischer, R, Larson-Nath, C, Mozer-Glassberg, Y, Arikan, C, Lin, H, Bernabeu, J, Alam, S, Kelly, D, Carvalho, E, Ferreira, C, Indolfi, G, Quiros-Tejeira, R, Bulut, P, Calvo, P, Anal, Z, Valentino, P, Desai, D, Eshun, J, Rogalidou, M, Dezsofi, A, Wiecek, S, Nebbia, G, Pinto, R, Wolters, V, Tamara, M, Zizzo, A, Garcia, J, Schwarz, K, Beretta, M, Sandahl, T, Jimenez-Rivera, C, Kerkar, N, Brecelj, J, Mujawar, Q, Rock, N, Busoms, C, Karnsakul, W, Lurz, E, Santos-Silva, E, Blondet, N, Bujanda, L, Shah, U, Thompson, R, Hansen, B, Kamath, B, Vandriel S. M., Li L. -T., She H., Wang J. -S., Gilbert M. A., Jankowska I., Czubkowski P., Gliwicz-Miedzinska D., Gonzales E. M., Jacquemin E., Bouligand J., Spinner N. B., Loomes K. M., Piccoli D. A., D'Antiga L., Nicastro E., Sokal A., Demaret T., Ebel N. H., Feinstein J. A., Fawaz R., Nastasio S., Lacaille F., Debray D., Arnell H., Fischler B., Siew S., Stormon M., Karpen S. J., Romero R., Kim K. M., Baek W. Y., Hardikar W., Shankar S., Roberts A. J., Evans H. M., Jensen M. K., Kavan M., Sundaram S. S., Chaidez A., Karthikeyan P., Sanchez M. C., Cavalieri M. L., Verkade H. J., Lee W. S., Squires J. E., Hajinicolaou C., Lertudomphonwanit C., Fischer R. T., Larson-Nath C., Mozer-Glassberg Y., Arikan C., Lin H. C., Bernabeu J. Q., Alam S., Kelly D. A., Carvalho E., Ferreira C. T., Indolfi G., Quiros-Tejeira R. E., Bulut P., Calvo P. L., Anal Z., Valentino P. L., Desai D. M., Eshun J., Rogalidou M., Dezsofi A., Wiecek S., Nebbia G., Pinto R. B., Wolters V. M., Tamara M. L., Zizzo A. N., Garcia J., Schwarz K., Beretta M., Sandahl T. D., Jimenez-Rivera C., Kerkar N., Brecelj J., Mujawar Q., Rock N., Busoms C. M., Karnsakul W., Lurz E., Santos-Silva E., Blondet N., Bujanda L., Shah U., Thompson R. J., Hansen B. E., Kamath B. M., Vandriel, S, Li, L, She, H, Wang, J, Gilbert, M, Jankowska, I, Czubkowski, P, Gliwicz-Miedzinska, D, Gonzales, E, Jacquemin, E, Bouligand, J, Spinner, N, Loomes, K, Piccoli, D, D'Antiga, L, Nicastro, E, Sokal, A, Demaret, T, Ebel, N, Feinstein, J, Fawaz, R, Nastasio, S, Lacaille, F, Debray, D, Arnell, H, Fischler, B, Siew, S, Stormon, M, Karpen, S, Romero, R, Kim, K, Baek, W, Hardikar, W, Shankar, S, Roberts, A, Evans, H, Jensen, M, Kavan, M, Sundaram, S, Chaidez, A, Karthikeyan, P, Sanchez, M, Cavalieri, M, Verkade, H, Lee, W, Squires, J, Hajinicolaou, C, Lertudomphonwanit, C, Fischer, R, Larson-Nath, C, Mozer-Glassberg, Y, Arikan, C, Lin, H, Bernabeu, J, Alam, S, Kelly, D, Carvalho, E, Ferreira, C, Indolfi, G, Quiros-Tejeira, R, Bulut, P, Calvo, P, Anal, Z, Valentino, P, Desai, D, Eshun, J, Rogalidou, M, Dezsofi, A, Wiecek, S, Nebbia, G, Pinto, R, Wolters, V, Tamara, M, Zizzo, A, Garcia, J, Schwarz, K, Beretta, M, Sandahl, T, Jimenez-Rivera, C, Kerkar, N, Brecelj, J, Mujawar, Q, Rock, N, Busoms, C, Karnsakul, W, Lurz, E, Santos-Silva, E, Blondet, N, Bujanda, L, Shah, U, Thompson, R, Hansen, B, Kamath, B, Vandriel S. M., Li L. -T., She H., Wang J. -S., Gilbert M. A., Jankowska I., Czubkowski P., Gliwicz-Miedzinska D., Gonzales E. M., Jacquemin E., Bouligand J., Spinner N. B., Loomes K. M., Piccoli D. A., D'Antiga L., Nicastro E., Sokal A., Demaret T., Ebel N. H., Feinstein J. A., Fawaz R., Nastasio S., Lacaille F., Debray D., Arnell H., Fischler B., Siew S., Stormon M., Karpen S. J., Romero R., Kim K. M., Baek W. Y., Hardikar W., Shankar S., Roberts A. J., Evans H. M., Jensen M. K., Kavan M., Sundaram S. S., Chaidez A., Karthikeyan P., Sanchez M. C., Cavalieri M. L., Verkade H. J., Lee W. S., Squires J. E., Hajinicolaou C., Lertudomphonwanit C., Fischer R. T., Larson-Nath C., Mozer-Glassberg Y., Arikan C., Lin H. C., Bernabeu J. Q., Alam S., Kelly D. A., Carvalho E., Ferreira C. T., Indolfi G., Quiros-Tejeira R. E., Bulut P., Calvo P. L., Anal Z., Valentino P. L., Desai D. M., Eshun J., Rogalidou M., Dezsofi A., Wiecek S., Nebbia G., Pinto R. B., Wolters V. M., Tamara M. L., Zizzo A. N., Garcia J., Schwarz K., Beretta M., Sandahl T. D., Jimenez-Rivera C., Kerkar N., Brecelj J., Mujawar Q., Rock N., Busoms C. M., Karnsakul W., Lurz E., Santos-Silva E., Blondet N., Bujanda L., Shah U., Thompson R. J., Hansen B. E., and Kamath B. M.

Abstract

Background and Aims: Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, and real-world data are lacking. This study aimed to elucidate the natural history of liver disease in a contemporary, international cohort of children with ALGS. Approach and Results: This was a multicenter retrospective study of children with a clinically and/or genetically confirmed ALGS diagnosis, born between January 1997 and August 2019. Native liver survival (NLS) and event-free survival rates were assessed. Cox models were constructed to identify early biochemical predictors of clinically evident portal hypertension (CEPH) and NLS. In total, 1433 children (57% male) from 67 centers in 29 countries were included. The 10 and 18-year NLS rates were 54.4% and 40.3%. By 10 and 18 years, 51.5% and 66.0% of children with ALGS experienced ≥1 adverse liver-related event (CEPH, transplant, or death). Children (>6 and ≤12 months) with median total bilirubin (TB) levels between ≥5.0 and <10.0 mg/dl had a 4.1-fold (95% confidence interval [CI], 1.6-10.8), and those ≥10.0 mg/dl had an 8.0-fold (95% CI, 3.4-18.4) increased risk of developing CEPH compared with those <5.0 mg/dl. Median TB levels between ≥5.0 and <10.0 mg/dl and >10.0 mg/dl were associated with a 4.8 (95% CI, 2.4-9.7) and 15.6 (95% CI, 8.7-28.2) increased risk of transplantation relative to <5.0 mg/dl. Median TB <5.0 mg/dl were associated with higher NLS rates relative to ≥5.0 mg/dl, with 79% reaching adulthood with native liver (p < 0.001). Conclusions: In this large international cohort of ALGS, only 40.3% of children reach adulthood with their native liver. A TB <5.0 mg/dl between 6 and 12 months of age is associated with better hepatic outcomes. These thresholds provide clinicians with an objective tool to assist with clinical decision-making and in the evaluation of therapies.

Published
2023

5. Incidence and predictors of hepatocellular carcinoma in patients with autoimmune hepatitis

Author

Colapietro, Francesca, primary, Maisonneuve, Patrick, additional, Lytvyak, Ellina, additional, Beuers, Ulrich, additional, Verdonk, Robert C., additional, van der Meer, Adriaan J., additional, van Hoek, Bart, additional, Kuiken, Sjoerd D., additional, Brouwer, Johannes T., additional, Muratori, Paolo, additional, Aghemo, Alessio, additional, Carella, Francesco, additional, van den Berg, Ad P., additional, Zachou, Kalliopi, additional, Dalekos, George N., additional, Di Zeo-Sánchez, Daniel E., additional, Robles, Mercedes, additional, Andrade, Raul J., additional, Montano-Loza, Aldo J., additional, van den Brand, Floris F., additional, Slooter, Charlotte D., additional, Macedo, Guilherme, additional, Liberal, Rodrigo, additional, de Boer, Ynto S., additional, Lleo, Ana, additional, van Gerven, N., additional, van Erpecum, K., additional, Ouden, J den, additional, Brouwer, J., additional, Vrolijk, J., additional, Gevers, T.J., additional, Drenth, J., additional, Guichelaar, M., additional, Bouma, G., additional, Schreuder, T.C.M.A., additional, van der Wouden, E.J., additional, Baak, L.C., additional, Stadhouders, P., additional, Klemt-Kropp, M., additional, Verhagen, M., additional, Bhalla, A., additional, Kuijvenhoven, J., additional, Almasio, P., additional, Alvarez, F., additional, Andrade, R., additional, Arikan, C., additional, Assis, D., additional, Bardou-Jacquet, E., additional, Biewenga, M., additional, Cancado, E., additional, Cazzagon, N., additional, Chazouillères, O., additional, Colloredo, G., additional, Cuarterolo, M., additional, Dalekos, G., additional, Debray, D., additional, Robles-Díaz, M., additional, Dyson, J., additional, Efe, C., additional, Engel, B., additional, Ferri, S., additional, Fontana, R., additional, Gatselis, N., additional, Gerussi, A., additional, Halilbasic, E., additional, Halliday, N., additional, Heneghan, M., additional, Hirschfield, G., additional, van Hoek, B., additional, Hørby Jørgensen, M., additional, Indolfini, G., additional, Iorio, R., additional, Invernizzi, P., additional, Jeong, S., additional, Jones, D., additional, Kelly, D., additional, Kerkar, N., additional, Lacaille, F., additional, Lammert, C., additional, Leggett, B., additional, Lenzi, M., additional, Levy, C., additional, Liberal, R., additional, Lleo, A., additional, Lohse, A., additional, Lopez, S. Ines, additional, de Martin, E., additional, McLin, V., additional, Mieli-Vergani, G., additional, Milkiewicz, P., additional, Mohan, N., additional, Muratori, L., additional, Nebbia, G., additional, van Nieuwkerk, C., additional, Oo, Y., additional, Ortega, A., additional, Páres, A., additional, Pop, T., additional, Pratt, D., additional, Purnak, T., additional, Ranucci, G., additional, Rushbrook, S., additional, Schramm, C., additional, Stättermayer, A., additional, Swain, M., additional, Tanaka, A., additional, Taubert, R., additional, Terrabuio, D., additional, Terziroli, B., additional, Trauner, M., additional, Valentino, P., additional, van den Brand, F., additional, Vergani, D., additional, Villamil, A., additional, Wahlin, S., additional, Ytting, H., additional, Zachou, K., additional, and Zeniya, M., additional

Published
2023
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6. Systematic review of response criteria and endpoints in autoimmune hepatitis by the International Autoimmune Hepatitis Group

Author

Pape, S, Snijders, R, Gevers, T, Chazouilleres, O, Dalekos, G, Hirschfield, G, Lenzi, M, Trauner, M, Manns, M, Vierling, J, Montano-Loza, A, Lohse, A, Schramm, C, Drenth, J, Heneghan, M, Almasio, P, Alvarez, F, Andrade, R, Arikan, C, Assis, D, Bardou-Jacquet, E, Biewenga, M, Cancado, E, Cazzagon, N, Colloredo, G, Cuarterolo, M, Debray, D, Robles-Diaz, M, Dyson, J, Efe, C, Engel, B, Ferri, S, Fontana, R, Gatselis, N, Gerussi, A, Halilbasic, E, Halliday, N, van Hoek, B, Horby Jorgensen, M, Indolfini, G, Iorio, R, Jeong, S, Jones, D, Kelly, D, Kerkar, N, Lacaille, F, Lammert, C, Leggett, B, Levy, C, Liberal, R, Lleo, A, Ines Lopez, S, de Martin, E, Mclin, V, Mieli-Vergani, G, Milkiewicz, P, Mohan, N, Muratori, L, Nebbia, G, van Nieuwkerk, C, Oo, Y, Ortega, A, Pares, A, Pop, T, Pratt, D, Purnak, T, Ranucci, G, Rushbrook, S, Stattermayer, A, Swain, M, Tanaka, A, Taubert, R, Terrabuio, D, Terziroli, B, Valentino, P, van den Brand, F, Villamil, A, Wahlin, S, Ytting, H, Zachou, K, Zeniya, M, Pape S., Snijders R. J. A. L. M., Gevers T. J. G., Chazouilleres O., Dalekos G. N., Hirschfield G. M., Lenzi M., Trauner M., Manns M. P., Vierling J. M., Montano-Loza A. J., Lohse A. W., Schramm C., Drenth J. P. H., Heneghan M. A., Almasio P., Alvarez F., Andrade R., Arikan C., Assis D., Bardou-Jacquet E., Biewenga M., Cancado E., Cazzagon N., Colloredo G., Cuarterolo M., Dalekos G., Debray D., Robles-Diaz M., Drenth J., Dyson J., Efe C., Engel B., Ferri S., Fontana R., Gatselis N., Gerussi A., Halilbasic E., Halliday N., Heneghan M., Hirschfield G., van Hoek B., Horby Jorgensen M., Indolfini G., Iorio R., Jeong S., Jones D., Kelly D., Kerkar N., Lacaille F., Lammert C., Leggett B., Levy C., Liberal R., Lleo A., Lohse A., Ines Lopez S., de Martin E., McLin V., Mieli-Vergani G., Milkiewicz P., Mohan N., Muratori L., Nebbia G., van Nieuwkerk C., Oo Y., Ortega A., Pares A., Pop T., Pratt D., Purnak T., Ranucci G., Rushbrook S., Stattermayer A., Swain M., Tanaka A., Taubert R., Terrabuio D., Terziroli B., Valentino P., van den Brand F., Villamil A., Wahlin S., Ytting H., Zachou K., Zeniya M., Pape, S, Snijders, R, Gevers, T, Chazouilleres, O, Dalekos, G, Hirschfield, G, Lenzi, M, Trauner, M, Manns, M, Vierling, J, Montano-Loza, A, Lohse, A, Schramm, C, Drenth, J, Heneghan, M, Almasio, P, Alvarez, F, Andrade, R, Arikan, C, Assis, D, Bardou-Jacquet, E, Biewenga, M, Cancado, E, Cazzagon, N, Colloredo, G, Cuarterolo, M, Debray, D, Robles-Diaz, M, Dyson, J, Efe, C, Engel, B, Ferri, S, Fontana, R, Gatselis, N, Gerussi, A, Halilbasic, E, Halliday, N, van Hoek, B, Horby Jorgensen, M, Indolfini, G, Iorio, R, Jeong, S, Jones, D, Kelly, D, Kerkar, N, Lacaille, F, Lammert, C, Leggett, B, Levy, C, Liberal, R, Lleo, A, Ines Lopez, S, de Martin, E, Mclin, V, Mieli-Vergani, G, Milkiewicz, P, Mohan, N, Muratori, L, Nebbia, G, van Nieuwkerk, C, Oo, Y, Ortega, A, Pares, A, Pop, T, Pratt, D, Purnak, T, Ranucci, G, Rushbrook, S, Stattermayer, A, Swain, M, Tanaka, A, Taubert, R, Terrabuio, D, Terziroli, B, Valentino, P, van den Brand, F, Villamil, A, Wahlin, S, Ytting, H, Zachou, K, Zeniya, M, Pape S., Snijders R. J. A. L. M., Gevers T. J. G., Chazouilleres O., Dalekos G. N., Hirschfield G. M., Lenzi M., Trauner M., Manns M. P., Vierling J. M., Montano-Loza A. J., Lohse A. W., Schramm C., Drenth J. P. H., Heneghan M. A., Almasio P., Alvarez F., Andrade R., Arikan C., Assis D., Bardou-Jacquet E., Biewenga M., Cancado E., Cazzagon N., Colloredo G., Cuarterolo M., Dalekos G., Debray D., Robles-Diaz M., Drenth J., Dyson J., Efe C., Engel B., Ferri S., Fontana R., Gatselis N., Gerussi A., Halilbasic E., Halliday N., Heneghan M., Hirschfield G., van Hoek B., Horby Jorgensen M., Indolfini G., Iorio R., Jeong S., Jones D., Kelly D., Kerkar N., Lacaille F., Lammert C., Leggett B., Levy C., Liberal R., Lleo A., Lohse A., Ines Lopez S., de Martin E., McLin V., Mieli-Vergani G., Milkiewicz P., Mohan N., Muratori L., Nebbia G., van Nieuwkerk C., Oo Y., Ortega A., Pares A., Pop T., Pratt D., Purnak T., Ranucci G., Rushbrook S., Stattermayer A., Swain M., Tanaka A., Taubert R., Terrabuio D., Terziroli B., Valentino P., van den Brand F., Villamil A., Wahlin S., Ytting H., Zachou K., and Zeniya M.

Abstract

Background & Aims: Autoimmune hepatitis (AIH) has been well characterised and codified through the development of diagnostic criteria. These criteria have been adapted and simplified and are widely used in clinical practice. However, there is a need to update and precisely define the criteria for both treatment response and treatment. Methods: A systematic review was performed and a modified Delphi consensus process was used to identify and redefine the response criteria in autoimmune hepatitis. Results: The consensus process initiated by the International Autoimmune Hepatitis Group proposes that the term ‘complete biochemical response’ defined as ‘normalization of serum transaminases and IgG below the upper limit of normal’ be adopted to include a time point at 6 months after initiation of treatment. An insufficient response by 6 months was a failure to meet the above definition. Non-response was defined as ‘<50% decrease of serum transaminases within 4 weeks after initiation of treatment’. Remission is defined as liver histology with a Hepatitis Activity Index <4/18. Intolerance to treatment was agreed to stand for ‘any adverse event possibly related to treatment leading to potential drug discontinuation’. Conclusions: These definitions provide a simple and reproducible framework to define treatment response and non-response, irrespective of the therapeutic intervention. A consensus on endpoints is urgently required to set a global standard for the reporting of study results and to enable inter-study comparisons. Future prospective database studies are needed to validate these endpoints. Lay summary: Consensus among international experts on response criteria and endpoints in autoimmune hepatitis is lacking. A consensus on endpoints is urgently required to set a global standard for the reporting of study results and to enable the comparison of results between clinical trials. Therefore, the International Autoimmune Hepatitis Group (IAIHG) herein presents

Published
2022

7. Natural history of liver disease in a large international cohort of children with Alagille syndrome: Results from the GALA study

Author

Vandriel, SM, Li, L-T, She, H, Wang, J-S, Gilbert, MA, Jankowska, I, Czubkowski, P, Gliwicz-Miedzinska, D, Gonzales, EM, Jacquemin, E, Bouligand, J, Spinner, NB, Loomes, KM, Piccoli, DA, D'Antiga, L, Nicastro, E, Sokal, E, Demaret, T, Ebel, NH, Feinstein, JA, Fawaz, R, Nastasio, S, Lacaille, F, Debray, D, Arnell, H, Fischler, B, Siew, S, Stormon, M, Karpen, SJ, Romero, R, Kim, KM, Baek, WY, Hardikar, W, Shankar, S, Roberts, AJ, Evans, HM, Jensen, MK, Kavan, M, Sundaram, SS, Chaidez, A, Karthikeyan, P, Sanchez, MC, Cavalieri, ML, Verkade, HJ, Lee, WS, Squires, JE, Hajinicolaou, C, Lertudomphonwanit, C, Fischer, RT, Larson-Nath, C, Mozer-Glassberg, Y, Arikan, C, Lin, HC, Bernabeu, JQ, Alam, S, Kelly, DA, Carvalho, E, Ferreira, CT, Indolfi, G, Quiros-Tejeira, RE, Bulut, P, Calvo, PL, Onal, Z, Valentino, PL, Desai, DM, Eshun, J, Rogalidou, M, Dezsofi, A, Wiecek, S, Nebbia, G, Pinto, RB, Wolters, VM, Tamara, ML, Zizzo, AN, Garcia, J, Schwarz, K, Beretta, M, Sandahl, TD, Jimenez-Rivera, C, Kerkar, N, Brecelj, J, Mujawar, Q, Rock, N, Busoms, CM, Karnsakul, W, Lurz, E, Santos-Silva, E, Blondet, N, Bujanda, L, Shah, U, Thompson, RJ, Hansen, BE, Kamath, BM, Vandriel, SM, Li, L-T, She, H, Wang, J-S, Gilbert, MA, Jankowska, I, Czubkowski, P, Gliwicz-Miedzinska, D, Gonzales, EM, Jacquemin, E, Bouligand, J, Spinner, NB, Loomes, KM, Piccoli, DA, D'Antiga, L, Nicastro, E, Sokal, E, Demaret, T, Ebel, NH, Feinstein, JA, Fawaz, R, Nastasio, S, Lacaille, F, Debray, D, Arnell, H, Fischler, B, Siew, S, Stormon, M, Karpen, SJ, Romero, R, Kim, KM, Baek, WY, Hardikar, W, Shankar, S, Roberts, AJ, Evans, HM, Jensen, MK, Kavan, M, Sundaram, SS, Chaidez, A, Karthikeyan, P, Sanchez, MC, Cavalieri, ML, Verkade, HJ, Lee, WS, Squires, JE, Hajinicolaou, C, Lertudomphonwanit, C, Fischer, RT, Larson-Nath, C, Mozer-Glassberg, Y, Arikan, C, Lin, HC, Bernabeu, JQ, Alam, S, Kelly, DA, Carvalho, E, Ferreira, CT, Indolfi, G, Quiros-Tejeira, RE, Bulut, P, Calvo, PL, Onal, Z, Valentino, PL, Desai, DM, Eshun, J, Rogalidou, M, Dezsofi, A, Wiecek, S, Nebbia, G, Pinto, RB, Wolters, VM, Tamara, ML, Zizzo, AN, Garcia, J, Schwarz, K, Beretta, M, Sandahl, TD, Jimenez-Rivera, C, Kerkar, N, Brecelj, J, Mujawar, Q, Rock, N, Busoms, CM, Karnsakul, W, Lurz, E, Santos-Silva, E, Blondet, N, Bujanda, L, Shah, U, Thompson, RJ, Hansen, BE, and Kamath, BM

Abstract

BACKGROUND AND AIMS: Alagille syndrome (ALGS) is a multisystem disorder, characterized by cholestasis. Existing outcome data are largely derived from tertiary centers, and real-world data are lacking. This study aimed to elucidate the natural history of liver disease in a contemporary, international cohort of children with ALGS. APPROACH AND RESULTS: This was a multicenter retrospective study of children with a clinically and/or genetically confirmed ALGS diagnosis, born between January 1997 and August 2019. Native liver survival (NLS) and event-free survival rates were assessed. Cox models were constructed to identify early biochemical predictors of clinically evident portal hypertension (CEPH) and NLS. In total, 1433 children (57% male) from 67 centers in 29 countries were included. The 10 and 18-year NLS rates were 54.4% and 40.3%. By 10 and 18 years, 51.5% and 66.0% of children with ALGS experienced ≥1 adverse liver-related event (CEPH, transplant, or death). Children (>6 and ≤12 months) with median total bilirubin (TB) levels between ≥5.0 and <10.0 mg/dl had a 4.1-fold (95% confidence interval [CI], 1.6-10.8), and those ≥10.0 mg/dl had an 8.0-fold (95% CI, 3.4-18.4) increased risk of developing CEPH compared with those <5.0 mg/dl. Median TB levels between ≥5.0 and <10.0 mg/dl and >10.0 mg/dl were associated with a 4.8 (95% CI, 2.4-9.7) and 15.6 (95% CI, 8.7-28.2) increased risk of transplantation relative to <5.0 mg/dl. Median TB <5.0 mg/dl were associated with higher NLS rates relative to ≥5.0 mg/dl, with 79% reaching adulthood with native liver ( p < 0.001). CONCLUSIONS: In this large international cohort of ALGS, only 40.3% of children reach adulthood with their native liver. A TB <5.0 mg/dl between 6 and 12 months of age is associated with better hepatic outcomes. These thresholds provide clinicians with an objective tool to assist with clinical decision-making and in the evaluation of therapies.

Published
2023

8. Risk factors and outcomes associated with recurrent autoimmune hepatitis following liver transplantation

Author

Montano-Loza, A.J., Ronca, V., Ebadi, M., Hansen, B.E., Hirschfield, G., Elwir, S., Alsaed, M., Milkiewicz, P., Janik, M.K., Marschall, H.U., Burza, M.A., Efe, C., Caliskan, A.R., Harputluoglu, M., Kabacam, G., Terrabuio, D., Onofrio, F.D., Selzner, N., Bonder, A., Pares, A., Llovet, L., Akyildiz, M., Arikan, C., Manns, M.P., Taubert, R., Weber, A.L., Schiano, T.D., Haydel, B., Czubkowski, P., Socha, P., Oldak, N., Akamatsu, N., Tanaka, A., Levy, C., Martin, E.F., Goel, A., Sedki, M., Jankowska, I., Ikegami, T., Rodriguez, M., Sterneck, M., Weiler-Normann, C., Schramm, C., Donato, M.F., Lohse, A., Andrade, R.J., Patwardhan, V.R., Hoek, B. van, Biewenga, M., Kremer, A.E., Ueda, Y., Deneau, M., Pedersen, M., Mayo, M.J., Floreani, A., Burra, P., Secchi, M.F., Beretta-Piccoli, B.T., Sciveres, M., Maggiore, G., Jafri, S.M., Debray, D., Girard, M., Lacaille, F., Lytvyak, E., Mason, A.L., Heneghan, M., Oo, Y.H., and Int Autoimmune Hepatitis Grp IAIHG

Subjects
Adult, Male, Hepatology, liver transplantation, recurrent disease, autoimmune liver disease, graft survival, Mycophenolic Acid, survival, Hepatitis, Autoimmune, Recurrence, Risk Factors, Immunoglobulin G, Humans, Female, Immunosuppressive Agents
Abstract

Background & Aims: Autoimmune hepatitis can recur after liver transplantation (LT), though the impact of recurrence on patient and graft survival has not been well characterized. We evaluated a large, international, multicenter cohort to identify the probability and risk factors associated with recurrent AIH and the association between recurrent disease and patient and graft survival.Methods: We included 736 patients (77% female, mean age 42 +/- 1 years) with AIH who underwent LT from January 1987 through June 2020, among 33 centers in North America, South America, Europe and Asia. Clinical data before and after LT, biochemical data within the first 12 months after LT, and immunosuppression after LT were analyzed to identify patients at higher risk of AIH recurrence based on histological diagnosis.Results: AIH recurred in 20% of patients after 5 years and 31% after 10 years. Age at LT

Published
2022

9. Systematic review of response criteria and endpoints in autoimmune hepatitis by the International Autoimmune Hepatitis Group

Author

Pape, Simon, primary, Snijders, Romée J.A.L.M., additional, Gevers, Tom J.G., additional, Chazouilleres, Oliver, additional, Dalekos, George N., additional, Hirschfield, Gideon M., additional, Lenzi, Marco, additional, Trauner, Michael, additional, Manns, Michael P., additional, Vierling, John M., additional, Montano-Loza, Aldo J., additional, Lohse, Ansgar W., additional, Schramm, Christoph, additional, Drenth, Joost P.H., additional, Heneghan, Michael A., additional, Almasio, P., additional, Alvarez, F., additional, Andrade, R., additional, Arikan, C., additional, Assis, D., additional, Bardou-Jacquet, E., additional, Biewenga, M., additional, Cancado, E., additional, Cazzagon, N., additional, Chazouillères, O., additional, Colloredo, G., additional, Cuarterolo, M., additional, Dalekos, G., additional, Debray, D., additional, Robles-Díaz, M., additional, Drenth, J., additional, Dyson, J., additional, Efe, C., additional, Engel, B., additional, Ferri, S., additional, Fontana, R., additional, Gatselis, N., additional, Gerussi, A., additional, Halilbasic, E., additional, Halliday, N., additional, Heneghan, M., additional, Hirschfield, G., additional, van Hoek, B., additional, Hørby Jørgensen, M., additional, Indolfini, G., additional, Iorio, R., additional, Jeong, S., additional, Jones, D., additional, Kelly, D., additional, Kerkar, N., additional, Lacaille, F., additional, Lammert, C., additional, Leggett, B., additional, Lenzi, M., additional, Levy, C., additional, Liberal, R., additional, Lleo, A., additional, Lohse, A., additional, Ines Lopez, S., additional, de Martin, E., additional, McLin, V., additional, Mieli-Vergani, G., additional, Milkiewicz, P., additional, Mohan, N., additional, Muratori, L., additional, Nebbia, G., additional, van Nieuwkerk, C., additional, Oo, Y., additional, Ortega, A., additional, Páres, A., additional, Pop, T., additional, Pratt, D., additional, Purnak, T., additional, Ranucci, G., additional, Rushbrook, S., additional, Schramm, C., additional, Stättermayer, A., additional, Swain, M., additional, Tanaka, A., additional, Taubert, R., additional, Terrabuio, D., additional, Terziroli, B., additional, Trauner, M., additional, Valentino, P., additional, van den Brand, F., additional, Villamil, A., additional, Wahlin, S., additional, Ytting, H., additional, Zachou, K., additional, and Zeniya, M., additional

Published
2022
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13. trauma in the emergency department: A randomised clinical trial

Author

Yilmaz, A, Sabirli, R, Ozen, M, Turkcuer, I, Erdur, B, Arikan, C, Demirozogul, E, Sarohan, A, Seyit, M, and Ok, N

Abstract

Introduction: Musculoskeletal system traumas are among the most common presentations in the emergency departments. In the treatment of traumatic musculoskeletal pain, paracetamol and non-steroidal anti-inflammatory analgesics (NSAID) are frequently used. Our aim in this study is to compare the efficacy of intravenous dexketoprofen and paracetamol in the treatment of traumatic musculoskeletal pain. Methods: This prospective, randomised, double blind, controlled study was conducted in a tertiary care emergency unit. The participating patients were randomised into two groups to receive either 50 mg of dexketoprofen or 1000 mg of paracetamol intravenously by rapid infusion in 150 mL of normal saline. Visual analogue scale (VAS), Numeric Rating Scala (NRS) and Verbal Rating Scale (VRS) was employed for pain measurement at baseline, after 15, after 30 and after 60 mins. Results: 200 patients were included in the final analysis. The median age of the paracetamol group was 34 (24-48), while that of the dexketoprofen group was 35 (23-50), and 63% (n = 126) of them consisted of men. Paracetamol and dexketoprofen administration reduced VAS pain scores over time (p = 0.0001). Median reduction in VAS score at 60 min was 55 (IQR 30-65) for the paracetamol group and 50(IQR 30.25-60) for the dexketoprofen group. There was no statistically significant difference between the paracetamol and dexketoprofen groups in terms of VAS reductions (p = 0.613). Conclusion: Intravenous paracetamol and dexketoprofen seem to produce equivalent pain relief for acute musculoskeletal trauma in the emergency department. (C) 2018 Elsevier Inc. All rights reserved. C1 [Yilmaz, Atakan; Ozen, Mert; Turkcuer, Ibrahim; Erdur, Bulent; Seyit, Murat] Pamukkale Univ, Med Fac, Dept Emergency Med, TR-20070 Denizli, Turkey. [Sabirli, Ramazan] Servergazi State Hosp, Emergency Serv, Denizli, Turkey. [Arikan, Cuneyt] Urla State Hosp, Emergency Serv, Izmir, Turkey. [Demirozogul, Ezgi] Gaziantep 25 Aralik State Hosp, Emergency Serv, Antakya, Turkey. [Sarohan, Ahmet] Hatay State Hosp, Emergency Serv, Antakya, Turkey. [Ok, Nusret] Pamukkale Univ, Med Fac, Dept Orthoped & Traumatol, Denizli, Turkey.

Published
2019

14. Intravenous dexketoprofen versus paracetamol in non-traumatic

Author

Demirozogul, E, Yilmaz, A, Ozen, M, Turkcuer, I, Seyit, M, and Arikan, C

Subjects
Dexketoprofen, Paracetamol, Intravenous, Pain relief
Abstract

Introduction: Although acute musculoskeletal pain has a wide range of causes from tendinitis, muscle spasm, to bone and joint injuries, it is a frequent occurrence in emergency services. Paracetamol and non-steroidal anti-inflammatory analgesics (NSAID) are common used in the treatment of musculoskeletal pain. This study sets out to compare the effectiveness of intravenous dexketoprofen and paracetamol in musculoskeletal pain relief. Methods: This prospective, randomized, double blind, controlled study was carried out in a university emergency room. The participating patients were randomized into two groups to receive either 50 mg of dexketoprofen or 1000 mg of paracetamol intravenously by rapid infusion in 150 ml of normal saline. Visual analogue scale (VAS), Numeric Rating Scala (NRS) was employed for pain measurement at baseline, after 15, after 30 and after 60 mins. Results: 200 patients were included in the study, excluding 7342 of them. The mean age of the patients was calculated as 32,6. Paracetamol and dexketoprofen intervention decreases NRS pain scores over time. When compared to all pain locations, the NRS pain score of the patients was found to be statistically more effective in dexketoprofen than in paracetamol (p = 0.001). Paracetamol and dexketoprofen intervention reduces pain VAS scores over time. When the VAS pain score of the patients was compared to all pain locations, dexketoprofen was found to be statistically more effective than paracetamol (p = 0.001). Conclusion: Intravenous dexketoprofen seemed to achieve superior analgesia to intravenous paracetamol when compared with all pain locations in patients with non-traumatic musculoskeletal pain. (C) 2019 Elsevier Inc. All rights reserved. C1 [Demirozogul, Ezgi] Gaziantep 25 Aralik State Hosp, Emergency Serv, Gaziantep, Turkey. [Yilmaz, Atakan; Ozen, Mert; Turkcuer, Ibrahim; Seyit, Murat] Pamukkale Univ, Med Fac, Dept Emergency Med, TR-20070 Denizli, Turkey. [Arikan, Cuneyt] Urla State Hosp, Emergency Serv, Izmir, Turkey.

Published
2019

23. The natural course of FIC1 deficiency and BSEP deficiency: Initial results from the NAPPED-consortium (Natural course and Prognosis of PFIC and Effect of biliary Diversion)

Author

van Wessel, D., primary, Thompson, R., additional, Grammatikopoulos, T., additional, Kadaristiana, A., additional, Jankowska, I., additional, Lipiński, P., additional, Czubkowski, P., additional, Gonzales, E., additional, Jacquemin, E., additional, Spraul, A., additional, Sokal, E., additional, Shagrani, M.A., additional, Broering, D.C., additional, Algoufi, T., additional, Mazhar, N., additional, Nicastro, E., additional, Kelly, D., additional, Nebbia, G., additional, Arnell, H., additional, Fischler, B., additional, Sankaranarayanan, S., additional, Hulscher, J., additional, Serranti, D., additional, Arikan, C., additional, Polat, E., additional, Debray, D., additional, Lacaille, F., additional, Goncalves, C., additional, Hierro, L., additional, Bartolo, G.M., additional, Mozer-Glassberg, Y., additional, Azaz, A., additional, Brecelj, J., additional, Dezsőfi, A., additional, Calvo, P.L., additional, Pizzol, A., additional, Schmitt, D., additional, Sturm, E., additional, van der Woerd, W., additional, Hansen, B., additional, and Verkade, H., additional

Published
2018
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26. SAT-048 - The natural course of FIC1 deficiency and BSEP deficiency: Initial results from the NAPPED-consortium (Natural course and Prognosis of PFIC and Effect of biliary Diversion)

Author

van Wessel, D., Thompson, R., Grammatikopoulos, T., Kadaristiana, A., Jankowska, I., Lipiński, P., Czubkowski, P., Gonzales, E., Jacquemin, E., Spraul, A., Sokal, E., Shagrani, M.A., Broering, D.C., Algoufi, T., Mazhar, N., Nicastro, E., Kelly, D., Nebbia, G., Arnell, H., Fischler, B., Sankaranarayanan, S., Hulscher, J., Serranti, D., Arikan, C., Polat, E., Debray, D., Lacaille, F., Goncalves, C., Hierro, L., Bartolo, G.M., Mozer-Glassberg, Y., Azaz, A., Brecelj, J., Dezsőfi, A., Calvo, P.L., Pizzol, A., Schmitt, D., Sturm, E., van der Woerd, W., Hansen, B., and Verkade, H.

Published
2018
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27. A COMPARISON OF NOVEL SERUM MARKERS FOR THE ASSESSMENT OF LIVER FIBROSIS IN PATIENTS WITH BETA THALASSEMIA MAJOR TREATED WITH DEFERASIROX

Author

Aydinok, Y., Arikan, C., Karakas, Z., Sasmaz, I., Canatan, D., Agaoglu, L., Kilinc, Y., Canatar, A., Ocak, O., Alnigenis, E., and Ege Üniversitesi

Subjects
health services administration, population characteristics, geographic locations
Abstract

15th Annual Meeting of the European-Hematology-Association -- JUN 10-13, 2010 -- Barcelona, SPAIN, WOS: 000279051302488, European Hematol Assoc

Published
2010

29. Surface Modification of Polyamide via Grafted Chitosan-Based Responsive Hydrogels

Author

Glampedaki, P., de Klein, J., Warmoeskerken, M.M.C.G., Jocic, D., H. Kadoglu, H., Perrin Akcakoca Kumbasar, E., Celik, P., Okan Arikan, C., Cay, A., and Damci, G.

Subjects
Chitosan, technology, industry, and agriculture, Polyamide, Responsive hydrogels, sense organs, skin and connective tissue diseases
Abstract

This study focuses on the surface modification of polyamide via chitosan-based hydrogels, which are responsive to pH and temperature changes. SEM and DSC were used for the hydrogels' characterization; Pore Volume Distribution measurements were realized for the polyamide samples' characterization. The hydrogels' response to temperature changes was determined spectrophotometrically and to pH changes gravimetrically. The polyamide samples' response to pH and temperature changes was determined gravimetrically and expressed as the samples' water uptake/loss or moisture regain/loss. The aim of this work was to develop stimuli-responsive polyamide which could serve for controlled substance release and increased wear comfort with a variety of applications in protection clothing and sportswear.

Published
2009

50. Predictors of hepatic steatosis in living liver donors

Author

Karasu, Z., primary, Tokat, Y., additional, Nart, D., additional, Celebi, A., additional, Arikan, C., additional, Kilic, M., additional, Akarca, U.S., additional, Sebnem, O., additional, Demirbas, T., additional, Gurgen, U., additional, Gunsar, F., additional, Ersoz, G., additional, and Batur, Y., additional

Published
2003
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