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001-es BibID:BIBFORM090567
035-os BibID:(WoS)000654740300009 (Scopus)85102488004
Első szerző:Marjot, Thomas
Cím:SARS-CoV-2 infection in patients with autoimmune hepatitis / Thomas Marjot, Gustav Buescher, Marcial Sebode, Eleanor Barnes, Alfred S. Barritt IV, Matthew J. Armstrong, Luke Baldelli, James Kennedy, Carolyn Mercer, Ann-Kathrin Ozga, Christian Casar, Christoph Schramm, Andrew M. Moon, Gwilym J. Webb, Ansgar W. Lohse, Collaborators of ERN RARE-LIVER / COVID-Hep / SECURE-Cirrhosis
Dátum:2021
ISSN:0168-8278
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
autoimmune liver disease
autoimmune hepatitis
SARS-CoV-2
COVID-19
immunosuppression
Megjelenés:Journal Of Hepatology. - 74 : 6 (2021), p. 1335-1343. -
További szerzők:Buescher, Gustav Sebode, Marcial Barnes, Eleanor Barritt, Alfred S. IV Armstrong, Matthew J. Baldelli, Luke Kennedy, James Mercer, Carolyn Ozga, Ann-Kathrin Casar, Christian Schramm, Christoph Moon, Andrew M. Webb, Gwilym J. Lohse, Ansgar W. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Balogh Boglárka (1993-) (belgyógyász) Collaborators of ERN RARE-LIVER / COVID-Hep / SECURE-Cirrhosis
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2.

001-es BibID:BIBFORM085824
Első szerző:Pape, Simon
Cím:High discontinuation rate of azathioprine in autoimmune hepatitis, independent of time of treatment initiation / Pape Simon, Gevers Tom J. G., Vrolijk Jan Maarten, van Hoek Bart, Bouma Gerd, van Nieuwkerk Carin M. J., Taubert Richard, Jaeckel Elmar, Manns Michael P., Papp Maria, Sipeki Nora, Stickel Felix, Efe Cumali, Ozaslan Ersan, Purnak Tugrul, Nevens Frederik, Kessener Dominik J. N., Kahraman Alisan, Wedemeyer Heiner, Hartl Johannes, Schramm Christoph, Lohse Ansgar W., Heneghan Michael A., Drenth Joost P. H.
Dátum:2020
ISSN:1478-3223
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:Liver International. - 40 : 9 (2020), p. 2164-2171. -
További szerzők:Gevers, Tom J. G. Vrolijk, Jan Maarten van Hoek, Bart Bouma, Gerd van Nieuwkerk, Carin M. J. Taubert, Richard Jaeckel, Elmar Manns, Michael P. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Sipeki Nóra (1987-) (általános orvos) Stickel, Felix Efe, Cumali Ozaslan, Ersan Purnak, Tugrul Nevens, Frederik Kessener, Dominik J. N. Kahraman, Alisan Wedemeyer, Heiner Hartl, Johannes Schramm, Christoph Lohse, Ansgar W. Heneghan, Michael A. Drenth, Joost P. H.
Pályázati támogatás:ÚNKP-19-4
Egyéb
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3.

001-es BibID:BIBFORM081815
035-os BibID:(WoS)000552446400014 (Scopus)85084968025
Első szerző:Pape, Simon
Cím:Rapid Response to Treatment of Autoimmune Hepatitis Associated with Remission at 6 and 12 Months / Simon Pape, Tom J. G. Gevers, Jan Maarten Vrolijk, Bart van Hoek, Gerd Bouma, Carin M. J. van Nieuwkerk, Richard Taubert, Elmar Jaeckel, Michael P. Manns, Maria Papp, Nora Sipeki, Felix Stickel, Cumali Efe, Ersan Ozaslan, Tugrul Purnak, Frederik Nevens, Dominik J. N. Kessener, Alisan Kahraman, Heiner Wedemeyer, Johannes Hartl, Christoph Schramm, Ansgar W. Lohse, Joost P. H. Drenth, Michael A. Heneghan
Dátum:2020
ISSN:1542-3565 1542-7714
Megjegyzések:Background & Aims: Changes in serum levels of transaminases immediately after initiation of treatment for autoimmune hepatitis (AIH) might be associated with biochemical markers of remission and liver-related events. We assessed the outcomes of patients with vs without rapid responses to treatment of AIH in a large international cohort. Methods: We performed a retrospective cohort study, collecting data from 2 independent cohorts of adults with AIH from 12 centers in 7 countries in Europe. We collected information on patient demographics; serologic, histologic, and biochemical analyses; and treatment. We used a receiver operating characteristic curve and Youden index to calculate the optimal percentage decrease in level of aspartate aminotransferase (AST) after 8 weeks of treatment that associated with normalization of transaminase levels after 26 weeks of treatment with predniso(lo)ne (primary outcome) in the first (discovery) cohort (n = 370). We evaluated the results in the second (validation) cohort (n = 370). Secondary outcomes were liver-related death or transplantation. We performed univariate and multivariable logistic and Cox regression with correction for confounders. Results: A significant decrease in level of AST after 8 weeks of treatment was significantly associated with normalization of transaminase levels at 26 and 52 weeks ( P <.001); a decrease of more than 80% in level of AST was associated with optimal normalization. In both cohorts, rapid responders (?80% decrease in level of AST after 8 weeks) were more likely to achieve normalization of transaminases at 26 and 52 weeks when compared to non-rapid responders. Rapid responders in the discovery cohort had lower risk of liver-related death or transplantation (adjusted hazard ratio 0.18; 95% CI 0.05?0.63; P =.007), although this was not confirmed in the validation cohort. Results from measurement of alanine aminotransferase did not differ significantly from those of AST for the primary outcome. Slow responders (without normalization of transaminases after 1 year) had the highest risk of liver transplantation or liver-related death. Conclusions: In a retrospective study of patients with AIH, we found that a rapid response to treatment, based on level of AST after 8 weeks, associates with normalization of transaminase levels in the following year. Patients with a rapid response also have a lower risk of liver-related death or transplantation than patients without this rapid response.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Induction therapy,
prognostic factor,
liver enzyme,
Megjelenés:Clinical Gastroenterology and Hepatology. - 18 : 7 (2020), p. 1609-1617. -
További szerzők:Gevers, Tom J. G. Maarten Vrolijk, Jan Hoek, Bart van Bouma, Gerd Nieuwkerk, Carin M. J. van Taubert, Richard Jaeckel, Elmar Manns, Michael P. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Sipeki Nóra (1987-) (általános orvos) Stickel, Felix Efe, Cumali Ozaslan, Ersan Purnak, Tugrul Nevens, Frederik Kessener, Dominik J. N. Kahraman, Alisan Wedemeyer, Heiner Hartl, Johannes Schramm, Christoph Lohse, Ansgar W. Drenth, Joost P. H. Heneghan, Michael A.
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4.

001-es BibID:BIBFORM077050
035-os BibID:(WoS)000482217300030 (Scopus)85065040027
Első szerző:Pape, Simon
Cím:Predniso(lo)ne Dosage and Chance of Remission in Patients With Autoimmune Hepatitis / Simon Pape, Tom J. G. Gevers, Michail Belias, Ilyas F. Mustafajev, Jan Maarten Vrolijk, Bart van Hoek, Gerd Bouma, Carin M. J. van Nieuwkerk, Johannes Hartl, Christoph Schramm, Ansgar W. Lohse, Richard Taubert, Elmar Jaeckel, Michael P. Manns, Maria Papp, Felix Stickel, Michael A. Heneghan, Joost P. H. Drenth
Dátum:2019
ISSN:1542-3565 1542-7714
Megjegyzések:BACKGROUND & AIMS: Patients with autoimmune hepatitis (AIH) commonly receive induction therapy with predniso(lo)ne followed by maintenance therapy with azathioprine. European Association for Study of the Liver clinical practice guidelines advise a predniso(lo)ne dose range of 0.50-1 mg/kg/day, which leaves room for practice variation. We performed a multicenter study to determine the efficacy of different dose ranges of predniso(lo)ne induction therapy in a large European cohort of patients with AIH. METHODS: We performed a retrospective cohort study using a comparative effectiveness design. We collected data from 451 adults with AIH who began treatment from 1978 through 2017 at 9 centers in 5 European countries. We assigned patients to a high-dose group (initial predniso(lo)ne dose ?0.50 mg/kg/day; n=281) or a low-dose group (<0.50 mg/kg/day; n=170). Logistic regression was performed to determine difference in outcomes between the groups. The primary outcome was normal serum levels of transaminases at 6 months after initiation of therapy. RESULTS: There was no significant difference in rates of normalization of transaminases between the high-dose predniso(lo)ne group and the low-dose group (70.5% vs 64.7%; P=.20). After multivariable logistic regression with correction for confounders, there was no difference in the likelihood of normalization of transaminases between the groups (odds ratio, 1.21; 95% CI, 0.78 - 1.87; P=.38). Patients given an initial high dose of predniso(lo)ne received more predniso(lo)ne over time than patients started on a lower dose (median doses over 6 months: 3780 mg vs 2573 mg) (P<.01). CONCLUSIONS: In a retrospective study of patients with AIH in Europe, we found that the dose of predniso(lo)ne to induce remission in patients with AIH is less relevant than assumed. An initial predniso(lo)ne dose below 0.50 mg/kg/day substantially decreases unnecessary exposure to predniso(lo)ne in patients with AIH.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
autoimmune hepatitis
EASL guidelines
ALT
AST
IgG
corticosteroid
induction therapy
cirrhosis
prednison
prednisolon
Megjelenés:Clinical Gastroenterology and Hepatology. - 17 : 10 (2019), p. 2068-2075.e2. -
További szerzők:Gevers, Tom J. G. Belias, Michail Mustafajev, Ilyas F. Vrolijk, Jan Maarten van Hoek, Bart Bouma, Gerd van Nieuwkerk, Carin M. J. Hartl, Johannes Schramm, Christoph Lohse, Ansgar W. Taubert, Richard Jaeckel, Elmar Manns, Michael P. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Stickel, Felix Heneghan, Michael A. Drenth, Joost P. H.
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5.

001-es BibID:BIBFORM074575
035-os BibID:(cikkazonosító)1959 (WoS)000442936400001 (Scopus)85068120394
Első szerző:Sowa, Mandy
Cím:Mucosal autoimmunity to cell-bound GP2 isoforms is a sensitive marker in PSC and associated with the clinical phenotype / Mandy Sowa, Rafał Kolenda, Daniel Baumgart, Johann Pratschke, Maria Papp, Tamas Tornai, Jaroslaw Suchanski, Dimitrios Bogdanos, Maria Mytilinaiou, Jutta Hammermann, Martin Laass, Karsten Conrad, Christoph Schramm, Andre Franke, Dirk Roggenbuck, Peter Schierack
Dátum:2018
ISSN:1664-3224
Megjegyzések:Introduction: Zymogen granule glycoprotein 2 (GP2) was demonstrated as first autoimmune mucosal target in primary sclerosing cholangitis (PSC) associated with disease severity. Autoantibodies to four GP2 isoforms (aGP21-4) were found in patients with inflammatory bowel diseases but reactivity against specific GP2 epitopes has not been investigated in PSC yet. Hence, the prevalence of aGP21-4 and their association with the PSC phenotype for risk prediction were examined.Methods: GP2 isoforms were stably expressed as glycosylphosphatidylinositol- anchored molecules in the membrane of HEp-2-cells and used as autoantigenic targets in indirect immunofluorescence assay (IFA). aGP21-4 IgA and IgG were detected by IFA in 212 PSC patients of four European university hospitals and 145 controls comprising 95 patients with cystic fibrosis and 50 healthy subjects.Results: Combined aGP21 and aGP24 IgA testing with a sensitivity of 66.0% and a specificity of 97.9% resulted in the best diagnostic performance (Youden index: 0.64) regarding all aGP2 and combinations thereof. aGP24 IgA positivity is significantly associated with the presence of cirrhosis in PSC (p=0.0056). Logistic regression revealed the occurrence of aGP21 IgA (odds ratio [OR] 1.38, 95% confidence interval [CI]: 1.03-1.86) and aGP24 IgA (OR 1.52, 95%CI: 1.07-2.15) along with male gender (OR 0.51, 95%CI: 0.27-0.97) and older age (OR 1.03 95%CI: 1.01-1.05) as significant risks for the concomitant presence of cirrhosis in PSC.Conclusions: Combined aGP21 and aGP24 IgA analysis is preferred to single aGP2 isoform analysis for sensitive PSC autoantibody testing. Positivity for aGP21 and aGP24 IgA is associated with cirrhosis in PSC and could be used for risk stratification.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
zymogen granule glycoprotein 2
primary sclerosing cholangitis
cirrhosis
cholangiocarcinoma
immunoglobulin A
Megjelenés:Frontiers in Immunology. - 9 (2018), p. 1-10. -
További szerzők:Kolenda, Rafał Baumgart, Daniel Pratschke, Johann Papp Mária (1975-) (belgyógyász, gasztroenterológus) Tornai Tamás István (1984-) (belgyógyász) Suchanski, Jaroslaw Bogdanos, Dimitrios P. Mytilinaiou, Maria Hammermann, Jutta Laass, Martin Conrad, Karsten Schramm, Christoph Franke, Andre Roggenbuck, Dirk Schierack, Peter
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6.

001-es BibID:BIBFORM109814
035-os BibID:(cikkazonosító)193 (scopus)85150952445 (wos)000959451600002
Első szerző:Uhlenbusch, Natalie
Cím:Improving quality of life in patients with rare autoimmune liver diseases by structured peer-delivered support (Q.RARE.LI) : study protocol for a transnational effectiveness-implementation hybrid trial / Natalie Uhlenbusch, Arpinder Bal, Boglárka Balogh, Annika Braun, Anja Geerts, Gideon Hirschfield, Maciej K. Janik, Ansgar W. Lohse, Piotr Milkiewicz, Mária Papp, Carine Poppe, Christoph Schramm, Löwe Bernd
Dátum:2023
ISSN:1471-244X
Megjegyzések:Aims: Psychosocial support is a crucial component of adequate rare disease care, but to date psychosocial support needs of this patient population are insufficiently met. We strive to evaluate the effectiveness of a peer-delivered psychosocial support intervention in patients with rare autoimmune liver diseases in five countries and prepare its implementation. Methods: The psychosocial support program combines structured, manual-based self-help and peer-support. Participant complete a self-help book from home over the course of six weeks, which is based on Acceptance and Commitment Therapy. In addition, they receive weekly telephone-based support sessions with a trained and supervised peer-counsellor. In a first efficacy trial (Depping et al., 2021, JAMA Psychiatry), the program led to improved quality of life, disease acceptance, self-management abilities, social support and reduced helplessness. Within Q.RARE.LI, we will conduct an effectiveness-implementation hybrid trial. To assess effectiveness, we will include N=240 patients with rare autoimmune liver diseases into a two-armed RCT, comparing the support program in addition to care-as-usual (CAU) with CAU alone. Our primary effectiveness outcome is mental health-related quality of life. To assess implementability, we will conduct a mixed-methods process evaluation, examining perceived acceptability and feasibility of the program and explore its implementability with patients, peer-counselors, and healthcare providers. Based on these results, we will derive country-specific implementation strategies. Discussion: The trans-diagnostic and location-independent program has the potential to address the unmet psychosocial support needs of patients with rare diseases. By preparing the implementation in five countries, we hope to improve comprehensive healthcare for an often neglected group.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Megjelenés:BMC Psychiatry. - 23 : 1 (2023), p. 1-11. -
További szerzők:Bal, Arpinder Balogh Boglárka (1993-) (belgyógyász) Braun, Annika Geerts, Anja Hirschfield, Gideon M. Janik, Maciej K. Lohse, Ansgar W. Milkiewicz, Piotr Papp Mária (1975-) (belgyógyász, gasztroenterológus) Poppe, Carine Schramm, Christoph Bernd, Löwe
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