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1.

001-es BibID:BIBFORM132792
035-os BibID:(Scopus)105019565415 (WoS)001598867700001
Első szerző:Gerussi, Alessio
Cím:Trends in Primary Biliary Cholangitis : prospective Cohort Study from the European Reference Network Registry (R-LIVER) / A. Gerussi, E. Nofit, D. P. Bernasconi, C. Kroll, F. Groß, I. Schregel, A. Marini, L. Cristoferi, F. Malinverno, M. Papp, G. Dalekos, E. Rigopoulou, M. K. Janik, P. Milkiewitz, H. Ytting, A. Leburgue, F. Braun, C. Casar, M. Sebode, A. Lohse, B. Hansen, M. Carbone, C. Schramm, P. Invernizzi
Dátum:2025
ISSN:2050-6406 2050-6414
Megjegyzések:Background and Aims: The European Reference Network on Hepatological Diseases (ERN RARE LIVER) is a Europe-wide network for centers of excellence in the management of rare liver diseases. We aimed to evaluate the current diagnostic and therapeutic trends of primary biliary cholangitis (PBC). Methods: Prospective data of PBC cases diagnosed from 2017 to March 2024 were extracted from the R-LIVER registry of ERN-RARE LIVER. Cases without two follow-ups within 24 months were excluded from the treatment analysis. Biochemical response according to Toronto criteria and normalization of alkaline phosphatase (ALP) values after 12 months of Ursodeoxycholic Acid (UDCA) were evaluated. Results: This study included 327 incident cases from six centers. Median age was 56 years, 89.3% were female. At the time of diagnosis, median values of ALP were 1.37 x ULN, and median bilirubin was 0.49 x ULN. Transient elastography (TE) was performed in 230 patients (70.3%) at baseline, median liver stiffness was 6.2 kPa. Out of 316 subjects, treatment with UDCA was started in 312 patients (98.7%); 246 (85.1%) achieved ALP values < 1.67 x ULN at 12 months. Normalization of ALP values occurred in 143 subjects (49.5%) at 12 months. Among 43 patients with inadequate response, 18 (41.9%) were treated with second-line therapies, and had worse liver biochemistry at baseline. Conclusion: In the current era, patients with PBC are diagnosed at an early stage using non-invasive methods and are almost all treated with UDCA. The biochemical response rate is 85.1%, but use of second-line therapies for inadequate responders remains suboptimal.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
liver
autoimmunity
epidemiology
UDCA
cholestasis
Megjelenés:United European Gastroenterology Journal. - 13 : 10 (2025), p. 1955-1963. -
További szerzők:Nofit, E. Bernasconi, D. P. Kroll, Claudia Groß, F. Schregel, Ida Marini, A. Cristoferi, Laura Malinverno, F. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Dalekos, George N. Rigopoulou, E. Janik, Maciej K. Milkiewicz, Piotr Ytting, Henriette Leburgue, Angela Braun, F. Casar, Christian Sebode, Marcial Lohse, Ansgar W. Hansen, Barbara C. Carbone, Marco Schramm, Christoph Invernizzi, Pietro
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2.

001-es BibID:BIBFORM083946
Első szerző:Lopens, Steffi
Cím:The search for the Holy Grail : autoantigenic targets in primary sclerosing cholangitis associated with disease phenotype and neoplasia / Steffi Lopens, Marcin Krawczyk, Maria Papp, Piotr Milkiewicz, Peter Schierack, Yudong Liu, Ewa Wunsch, Karsten Conrad, Dirk Roggenbuck
Dátum:2020
ISSN:2038-0305 2038-3274
Megjegyzések:Unlike in other autoimmune liver diseases such as autoimmune hepatitis and primary biliary cholangitis, the role and nature of autoantigenic targets in primary sclerosing cholangitis (PSC), a progressive chronic immune-mediated, life threatening, genetically predisposed, cholestatic liver illness, is poorly elucidated. Although anti-neutrophil cytoplasmic antibodies (ANCA) have been associated with the occurrence of PSC, their corresponding target(s) have not yet been identified entirely. Genome wide association studies recently revealed a significant number of immune-related and even disease-modifying susceptibility loci for PSC. However, they did not allow to discern a clear autoimmune pattern nor do therapy options and male gender preponderance in PSC support a pathogenic role of autoimmune responses. Yet, there is growing evidence that PSC is closely linked to the co-occurrence of inflammatory bowel disease (IBD) phenotypes. The identification of novel autoantigenic targets interacting with the gut microbiota in IBD such as the major zymogen granule membrane glycoprotein 2 (GP2) or the detection of high-sensitive proteinase 3 (PR3) autoantibodies have refocused the interest on a putative association of loss of tolerance with the IBD phenotype. Not surprisingly, the report of GP2 IgA to be associated with fibrosis in PSC and disease severity gave new impetus to autoantibody research for autoimmune liver illnesses and might usher in a new era of serological research in this field. The mucosal loss of tolerance against the microbiota-sensing GP2 modulating innate and adaptive intestinal immunity and its putative role in the pathogenesis of PSC will be discussed along with other potential PSC-related autoantigenic targets such as the neutrophil PR3 in this review. GP2 IgA may represent a group of new pathogenic antibodies which share characteristics of both type 2 and 3 of antibody-mediated hypersensitive reactions according to Coombs and Gell.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
primary sclerosing cholangitis
microbiota
ulcerative colitis
Crohn's disease
inflammatory bowel disease
cirrhosis
cholangiocarcinoma
immunoglobulin A
Megjelenés:Autoimmunity Highlights. - 11 : 1 (2020), p. 1-14. -
További szerzők:Krawczyk, Marcin Papp Mária (1975-) (belgyógyász, gasztroenterológus) Milkiewicz, Piotr Schierack, Peter Liu, Yudong Wunsch, Ewa Conrad, Karsten Roggenbuck, Dirk
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3.

001-es BibID:BIBFORM121373
035-os BibID:(WoS)001273414600001 (Scopus)85199139220
Első szerző:Schregel, Ida
Cím:Unmet needs in autoimmune hepatitis : results of the prospective multicentre European Reference Network Registry (R-LIVER) / Schregel Ida, Papp Maria, Sipeki Nora, Kovats Patricia J., Taubert Richard, Engel Bastian, Campos-Murguia Alejandro, Dalekos George N., Gatselis Nikolaos, Zachou Kalliopi, Milkiewicz Piotr, Janik Maciej K., Raszeja-Wyszomirska Joanna, Ytting Henriette, Braun Felix, Casar Christian, Sebode Marcial, Lohse Ansgar W., Schramm Christoph, European Reference Network (ERN) RARE-LIVER
Dátum:2024
ISSN:1478-3223
Megjegyzések:The European Reference Network on Hepatological Diseases (ERN RARE-LIVER) launched the prospective, multicentre, quality-controlled R-LIVER registry on rare liver diseases. The aim of this study was to assess the presentation and outcome of autoimmune hepatitis (AIH) after one year of treatment. Methods Data was prospectively collected at the time of diagnosis, and after 6- and 12-months follow-up. Complete biochemical response (CBR) was defined as normalization of alanine aminotransferase (ALT) and immunoglobulin G (IgG) serum levels. Results 231 patients from six European centres were included in the analysis. After six months of treatment 50% (106/212), and after 12 months 63% (131/210) of patients reached CBR with only 27% (56/211) achieving a steroid-free CBR within the first year. Overall, 16 different treatment regimens were administered. Change of treatment, mostly due to intolerance, occurred in 30.4% within the first six months. In multivariate analysis, younger age at diagnosis (OR = 1.03 [95%CI 1.01 ? 1.05]; p = .007), severe fibrosis (OR 0.38 [95% 0.16 ? 0.89], p = .026) and change of treatment within the first six months (OR 0.40 [95% CI 0.2 ? 0.86]; p = .018) were associated with a lesser chance of ALT normalization at 12 month-follow-up. The landscape of AIH-treatment in Europe is highly heterogenous, even between expert centres. The results from this first European multicentre prospective registry reveal several unmet needs, highlighted by the overall low rates of CBR and the frequent failure to withdraw corticosteroids.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Autoimmune Hepatitis
treatment regime
complete biochemical response
immunosuppression
remission
Megjelenés:Liver International. - 44 : 10 (2024), p. 2687-2699. -
További szerzők:Papp Mária (1975-) (belgyógyász, gasztroenterológus) Sipeki Nóra (1987-) (általános orvos) Kováts Patrícia (1995-) Taubert, Richard Engel, Bastian Campos-Murguia, Alejandro Dalekos, George N. Gatselis, Nikolaos K. Zachou, Kalliopi Milkiewicz, Piotr Janik, Maciej K. Raszeja-Wyszomirska, Joanna Ytting, Henriette Braun, Felix Casar, Christian Sebode, Marcial Lohse, Ansgar W. Schramm, Christoph European Reference Network (ERN) RARE-LIVER
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4.

001-es BibID:BIBFORM126381
035-os BibID:(Scopus)85218769201 (WoS)001510104200001
Első szerző:Snijders, Romee
Cím:Health-related quality of life is impaired in people with autoimmune hepatitis : results of a multicentre cross sectional study within the European Reference Network / Romée J. A. L. M. Snijders, Maciej K. Janik, Meike Mund, Natalie Uhlenbusch, Joanna Raszeja-Wyszomirska, Alessio Gerussi, Francesca Bolis, Laura Cristoferi, Pietro Invernizzi, Patricia Kovats, Mária Papp, Lisbet Grønbæk, Henning Grønbæk, Eric T. T. L. Tjwa, Luise Aamann, Henriette Ytting, Vincenzo Ronca, Katheryn Olsen, Ye H. Oo, Adriaan J. van der Meer, João Madaleno, Bernardo Canhão, Bastian Engel, Alejandro Campos-Murguia, Richard Taubert, Özgür M-Koc, Matthijs Kramer, José A. Willemse, Bernd Löwe, Ansgar W. Lohse, Joost P. H. Drenth, Christoph Schramm, Piotr Milkiewicz, Tom J. Gevers
Dátum:2025
ISSN:0270-9139
Megjegyzések:Abstract Background: Impaired health-related quality of life (HRQoL) contributes to the overall disease burden in autoimmune hepatitis (AIH). This study aimed to evaluate HRQoL in people with AIH and to identify potentially modifiable factors associated with impaired HRQoL using validated patient-reported outcome measures. Methods: Adult AIH patients diagnosed at 12 European centers were enrolled in this prospective, cross-sectional study from July 2020-June 2023. HRQoL was assessed using the Physical Component Score (PCS) and Mental Component Score (MCS) of the 12-item Short Form Health Survey (SF-12), and the European Quality-of-life 5-Dimension 5-Level (EQ-5D-5L) utility index (UI) score. Mixed-model regression analyses identified factors associated with HRQoL and somatic symptom severity. Controls were recruited from the general population in five European countries. Results: A total of 882 patients with AIH (mean age: 51.0 years [SD 17.0]; 76.4% female) and 178 controls were included. Physical but not mental HRQoL was impaired in the AIH group compared with the control group (PCS: 46.3 vs. 51.9, p<0.001; EQ-5D UI: 0.87 vs. 0.95, p<0.001). HRQoL was associated with severe somatic symptoms (PCS ? = -4.26, p<0.001), fatigue (PCS ? = -0.25, p<0.001; MCS ? = -0.25, p<0.001), and depression/ anxiety (PCS ? = 3.37, p<0.001; MCS ? = -6.79, p<0.001). A complete biochemical responseCBR was associated with a lower somatic symptom severity (odds ratio 0.69, p<0.05). Conclusions: People with AIH had significantly impaired HRQoL compared with controls, particularly in terms of physical well-being. HRQoL scores are associated with symptom burden, encompassing both somatic and psychosocial dimensions.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
mental health
physical health
depression
complete biochemical response
symptoms
Megjelenés:Hepatology. - 82 : 5 (2025), p. 1058-1072. -
További szerzők:Janik, Maciej K. Mund, Meike Uhlenbusch, Natalie Raszeja-Wyszomirska, Joanna Gerussi, Alessio Bolis, Francesca Cristoferi, Laura Invernizzi, Pietro Kováts Patrícia (1995-) Papp Mária (1975-) (belgyógyász, gasztroenterológus) Groenbaek, Lisbet Groenbaek, Henning Tjwa, Eric Aamann, Luise Ytting, Henriette Ronca, Vincenzo Olsen, Katheryn Oo, Ye Htun Meer, Adriaan J. van der Madaleno, João Canhão, Bernardo Engel, Bastian Campos-Murguia, Alejandro Taubert, Richard M-Koc, Özgür Kramer, Matthijs Willemse, José A. Löwe, Bernd Lohse, Ansgar W. Drenth, Joost P. H. Schramm, Christoph Milkiewicz, Piotr Gevers, Tom J. G.
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5.

001-es BibID:BIBFORM123332
035-os BibID:(scopus)85202766719
Első szerző:Stern, Louisa
Cím:Efficacy and safety of palliative treatment in patients with autoimmune liver disease-associated hepatocellular carcinoma / Stern Louisa, Schmidt Constantin, Kocheise Lorenz, Joerg Vincent, Casar Christian, Walter Aurélie, Drenth Joost P. H., Papp Maria, Gatselis Nikolaos K., Zachou Kalliopi, Pinter Matthias, Scheiner Bernhard, Vogel Arndt, Kirstein Martha M., Finkelmeier Fabian, Waidmann Oliver, Weinmann Arndt, Milkiewicz Piotr, Thorburn Douglas, Halliday Neil, Lleo Ana, Huber Samuel, Dalekos George N., Lohse Ansgar W., Wege Henning, von Felden Johann, Schulze Kornelius
Dátum:2024
ISSN:1665-2681
Megjegyzések:Introduction and Objectives Autoimmune liver diseases (AILD) are rare causes hepatocellular carcinoma (HCC). and data on the efficacy and tolerability of anti-tumour therapies are scarce. This pan-European study aimed to assess outcomes in AILD-HCC patients treated with tyrosine kinase inhibitors (TKIs) or transarterial chemoembolization (TACE) compared with patients with more common HCC etiologies, including viral, alcoholic or non-alcoholic fatty liver disease. Materials and Methods 107 patients with HCC-AILD (AIH:55; PBC:52) treated at 13 European centres between 1996 and 2020 were included. 65 received TACE and 28 received TKI therapy. 43 (66%) were female (median age 73 years) with HCC tumour stage BCLC A (34%), B (46%), C (9%) or D (11%). For each treatment type, propensity score matching was used to match AILD to non-AILD-HCC on a 1:1 basis, yielding in a final cohort of 130 TACE and 56 TKI patients for comparative analyses of median overall survival (mOS) and treatment tolerability. Results HCC-AILD patients showed comparable mOS to controls for both TACE (19.5 vs 22.1 months, p=0.9) and TKI (15.4 vs 15.1 months, p=0.5). Adverse events were less frequent in AILD-HCC patients than controls (33% vs 62%, p=0.003). For TKIs, there were no significant differences in adverse events (73% vs. 86%, p=0.2) or interruption rates (44% vs. 36%, p=0.7). Conclusions In summary, this study demonstrates comparable mOS for AILD-HCC patients undergoing local and systemic treatments, with better tolerability than HCC of other causes. TKIs remain important therapeutic options for AILD-HCC patients, particularly given their exclusion from recent immunotherapy trials.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
TACE
AILD
TKI
HCC
AIH
PBC
Megjelenés:Annals of Hepatology. - 29 : 6 (2024), p. 1-6. -
További szerzők:Schmidt, Constantin Kocheise, Lorenz Joerg, Vincent Casar, Christian Walter, Aurélie Drenth, Joost P. H. Papp Mária (1975-) (belgyógyász, gasztroenterológus) Gatselis, Nikolaos K. Zachou, Kalliopi Pinter, Matthias Scheiner, Bernhard Vogel, Arndt Kirstein, Martha M. Finkelmeier, Fabian Waidmann, Oliver Weinmann, Arndt Milkiewicz, Piotr Thorburn, Douglas Halliday, Neil Lleo, Ana Huber, Samuel Dalekos, George N. Lohse, Ansgar W. Wege, Henning von Felden, Johann Schulze, Kornelius
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6.

001-es BibID:BIBFORM134841
Első szerző:Uhlenbusch, Natalie
Cím:Quality of life in patients with variant syndromes of autoimmune liver diseases : a cross-sectional multicenter study / Natalie Uhlenbusch, Romée J. A. L. M. Snijder, Meike Mund, Maciej K. Janik, Piotr Milkiewicz, Bernd Löwe, Claudia Kroll, Joanna Raszeja-Wyszomirska, Alessio Gerussi, Francesca Bolis, Laura Cristoferi, Pietro Invernizzi, Patricia Kovats, Mária Papp, Lisbet Grnbk, Henning Grnbk, Eric T. T. L. Tjwa, Luise Aamann, Henriette Ytting, Vincenzo Ronca, Kathryn Olsen, Ye H. Oo, Adriaan J. van der Meer, Joao Madaleno, Bernardo Canhao, Bastian Engel, Alejandro Campos-Murguia, Richard Taubert, Özgür M. Koc, Matthijs Kramer, José A Willemse, Ansgar W. Lohse, Joost P. H. Drenth, Tom J. G. Gevers, Christoph Schramm
Dátum:2026
ISSN:1478-3223
Megjegyzések:Background & Aims Primary biliary cholangitis (PBC), primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) go along with reduced health-related quality of life (HRQOL). Variant syndromes, i.e. conditions with features of both PBC/PSC and AIH, are associated with higher clinical complexity and worse prognosis. Studies on HRQOL in patients with variant syndromes are lacking. We aimed to provide large-scale evidence addressing this gap. Methods We included adult patients with clinical diagnoses of autoimmune liver diseases across nine countries in a cross-sectional study. We descriptively compared demographical, clinical and patient-reported outcomes between the conditions and investigated whether additional AIH contributes to reduced HRQOL compared to the cholestatic liver disease alone. Further, we explored the role of fatigue, cirrhosis and depression severity regarding HRQOL. Results N=1275 patients were included (PBC: n=342, PBC-AIH: n=160, PSC: n=305, PSC-AIH: n=121, AIH: n=347). Patients with variant syndromes showed high rates of cirrhosis and increased depressive or anxiety symptoms. Additional AIH was associated with further reduction in physical and mental HRQOL in people with PSC (physical: ?R2=.012, p=.041; mental: ?R2=.016, p=.020), but not with PBC (physical: ?R2=.008, p=.081; mental: ?R2=.001, p=.609). Physical HRQOL was associated with higher age and fatigue, while mental HRQOL was associated with lower age, fatigue and depression severity. Conclusions Patients with variant syndromes of autoimmune liver diseases show high physical and mental burden, with fatigue as the main contributor. Particularly PSC-AIH goes along with more severely reduced HRQOL compared to the cholestatic liver disease alone, which is attributable to higher symptom burden.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
autoimmune liver diseases
cholestatic liver diseases
autoimmune hepatitis
variant syndrome
overlap syndrome
quality of life
Megjelenés:Liver International. - "Accepted by Publisher" (2026). -
További szerzők:Snijders, Romee Mund, Meike Janik, Maciej K. Milkiewicz, Piotr Löwe, Bernd Kroll, Claudia Raszeja-Wyszomirska, Joanna Gerussi, Alessio Bolis, Francesca Cristoferi, Laura Invernizzi, Pietro Kováts Patrícia (1995-) Papp Mária (1975-) (belgyógyász, gasztroenterológus) Grnbk, Lisbet Grnbk, Henning Tjwa, Eric Aamann, Luise Ytting, Henriette Ronca, Vincenzo Olsen, Katheryn Oo, Ye Htun van der Meer, Adriaan J. Madaleno, João Canhão, Bernardo Engel, Bastian Campos-Murguia, Alejandro Taubert, Richard Koc, Özgür M. Kramer, Matthijs Willemse, José A. Lohse, Ansgar W. Drenth, Joost P. H. Gevers, Tom J. G. Schramm, Christoph
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7.

001-es BibID:BIBFORM109814
035-os BibID:(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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