دورية أكاديمية
أعرض في EDS

Factors Associated With Outcomes of Patients With Primary Sclerosing Cholangitis and Development and Validation of a Risk Scoring System.

التفاصيل البيبلوغرافية
العنوان: Factors Associated With Outcomes of Patients With Primary Sclerosing Cholangitis and Development and Validation of a Risk Scoring System.
المؤلفون: Goode EC; Norfolk and Norwich University Hospital, Norwich, United Kingdom.; Academic Department of Medical Genetics, Addenbrooke's Hospital, University of Cambridge, Cambridge, United Kingdom.; Wellcome Trust Sanger Institute, Hinxton, Cambridge, United Kingdom.; Norwich Medical School, University of East Anglia, Norwich, United Kingdom.; Cambridge Transplant Centre, Addenbrooke's Hospital, Cambridge, United Kingdom., Clark AB; Norwich Medical School, University of East Anglia, Norwich, United Kingdom., Mells GF; Academic Department of Medical Genetics, Addenbrooke's Hospital, University of Cambridge, Cambridge, United Kingdom., Srivastava B; Academic Department of Medical Genetics, Addenbrooke's Hospital, University of Cambridge, Cambridge, United Kingdom., Spiess K; Academic Department of Medical Genetics, Addenbrooke's Hospital, University of Cambridge, Cambridge, United Kingdom., Gelson WTH; Cambridge Transplant Centre, Addenbrooke's Hospital, Cambridge, United Kingdom., Trivedi PJ; National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre, Birmingham, United Kingdom.; Institute of Immunology & Immunotherapy, University of Birmingham, Birmingham, United Kingdom.; Centre for Rare Diseases, Institute of Translational Medicine, University Hospitals Birmingham, Birmingham, United Kingdom., Lynch KD; Translational Gastroenterology Unit, John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom., Castren E; Norfolk and Norwich University Hospital, Norwich, United Kingdom., Vesterhus MN; Norwegian PSC Research Center, Department of Transplantation Medicine, Oslo University Hospital Rikshospitalet, Oslo, Norway.; Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway., Karlsen TH; Norwegian PSC Research Center, Department of Transplantation Medicine, Oslo University Hospital Rikshospitalet, Oslo, Norway.; Institute of Clinical Medicine, Faculty of Medicine, University of Oslo, Oslo, Norway., Ji SG; Wellcome Trust Sanger Institute, Hinxton, Cambridge, United Kingdom., Anderson CA; Wellcome Trust Sanger Institute, Hinxton, Cambridge, United Kingdom., Thorburn D; Sheila Sherlock Liver Centre, Royal Free Hospital, London, United Kingdom., Hudson M; Liver Medicine and Transplantation Service, Freeman Hospital, Newcastle, United Kingdom., Heneghan MA; Institute of Liver Studies, Kings College Hospital, London, United Kingdom., Aldersley MA; Department of Hepatology, Leeds Teaching Hospital, Leeds, United Kingdom., Bathgate A; Scottish Liver Transplant Unit, Royal Infirmary of Edinburgh, Edinburgh, United Kingdom., Sandford RN; Academic Department of Medical Genetics, Addenbrooke's Hospital, University of Cambridge, Cambridge, United Kingdom., Alexander GJ; Cambridge Transplant Centre, Addenbrooke's Hospital, Cambridge, United Kingdom.; Sheila Sherlock Liver Centre, Royal Free Hospital, London, United Kingdom., Chapman RW; Translational Gastroenterology Unit, John Radcliffe Hospital, and Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom., Walmsley M; PSC Support, Oxfordshire, United Kingdom., Hirschfield GM; National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre, Birmingham, United Kingdom.; Institute of Immunology & Immunotherapy, University of Birmingham, Birmingham, United Kingdom.; Centre for Rare Diseases, Institute of Translational Medicine, University Hospitals Birmingham, Birmingham, United Kingdom.; Toronto Centre for Liver Disease, University Health Network and University of Toronto, Toronto, Canada., Rushbrook SM; Norfolk and Norwich University Hospital, Norwich, United Kingdom.; Norwich Medical School, University of East Anglia, Norwich, United Kingdom.
مؤلفون مشاركون: UK-PSC Consortium
المصدر: Hepatology (Baltimore, Md.) [Hepatology] 2019 May; Vol. 69 (5), pp. 2120-2135. Date of Electronic Publication: 2019 Mar 04.
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't; Validation Study
اللغة: English
بيانات الدورية: Publisher: Wolters Kluwer Health, Inc Country of Publication: United States NLM ID: 8302946 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1527-3350 (Electronic) Linking ISSN: 02709139 NLM ISO Abbreviation: Hepatology Subsets: MEDLINE
أسماء مطبوعة: Publication: 2023- : [Philadelphia] : Wolters Kluwer Health, Inc.
Original Publication: Baltimore, MD : Williams & Wilkins, [c1981]-
مواضيع طبية MeSH: Cholangitis, Sclerosing/*mortality, Alkaline Phosphatase/blood ; Cholangitis, Sclerosing/blood ; Cholangitis, Sclerosing/genetics ; Cholangitis, Sclerosing/surgery ; Female ; HLA Antigens/genetics ; Humans ; Liver Transplantation ; Male ; Middle Aged ; Risk Assessment ; United Kingdom/epidemiology
مستخلص: We sought to identify factors that are predictive of liver transplantation or death in patients with primary sclerosing cholangitis (PSC), and to develop and validate a contemporaneous risk score for use in a real-world clinical setting. Analyzing data from 1,001 patients recruited to the UK-PSC research cohort, we evaluated clinical variables for their association with 2-year and 10-year outcome through Cox-proportional hazards and C-statistic analyses. We generated risk scores for short-term and long-term outcome prediction, validating their use in two independent cohorts totaling 451 patients. Thirty-six percent of the derivation cohort were transplanted or died over a cumulative follow-up of 7,904 years. Serum alkaline phosphatase of at least 2.4 × upper limit of normal at 1 year after diagnosis was predictive of 10-year outcome (hazard ratio [HR] = 3.05; C = 0.63; median transplant-free survival 63 versus 108 months; P < 0.0001), as was the presence of extrahepatic biliary disease (HR = 1.45; P = 0.01). We developed two risk scoring systems based on age, values of bilirubin, alkaline phosphatase, albumin, platelets, presence of extrahepatic biliary disease, and variceal hemorrhage, which predicted 2-year and 10-year outcomes with good discrimination (C statistic = 0.81 and 0.80, respectively). Both UK-PSC risk scores were well-validated in our external cohort and outperformed the Mayo Clinic and aspartate aminotransferase-to-platelet ratio index (APRI) scores (C statistic = 0.75 and 0.63, respectively). Although heterozygosity for the previously validated human leukocyte antigen (HLA)-DR*03:01 risk allele predicted increased risk of adverse outcome (HR = 1.33; P = 0.001), its addition did not improve the predictive accuracy of the UK-PSC risk scores. Conclusion: Our analyses, based on a detailed clinical evaluation of a large representative cohort of participants with PSC, furthers our understanding of clinical risk markers and reports the development and validation of a real-world scoring system to identify those patients most likely to die or require liver transplantation.
(© 2018 The Authors. Hepatology published by Wiley Periodicals, Inc. on behalf of American Association for the Study of Liver Diseases.)
التعليقات: Comment in: Hepatology. 2020 Jan;71(1):398-399. (PMID: 31544247)
Comment in: Hepatology. 2020 Jan;71(1):399-400. (PMID: 31550385)
Comment in: Eur J Gastroenterol Hepatol. 2023 Apr 1;35(4):480-487. (PMID: 36719819)
References: Mayo Clin Proc. 2000 Jul;75(7):688-94. (PMID: 10907383)
J Hepatol. 2002 Mar;36(3):321-7. (PMID: 11867174)
Gut. 2002 Nov;51(5):731-5. (PMID: 12377815)
Gastroenterology. 1992 Dec;103(6):1893-901. (PMID: 1451982)
Am J Epidemiol. 2007 Mar 15;165(6):710-8. (PMID: 17182981)
Gastroenterology. 1991 Jun;100(6):1710-7. (PMID: 1850376)
J Hepatol. 2009 Jan;50(1):158-64. (PMID: 19012991)
Gastroenterology. 2010 Mar;138(3):1102-11. (PMID: 19944697)
Hepatology. 2010 Feb;51(2):660-78. (PMID: 20101749)
Am J Ther. 2011 May;18(3):198-205. (PMID: 20228674)
BMC Med. 2010 Mar 30;8:20. (PMID: 20353578)
J Hepatobiliary Pancreat Sci. 2011 Mar;18(2):154-61. (PMID: 20740366)
Dig Liver Dis. 2011 Apr;43(4):309-13. (PMID: 21251891)
Hepatology. 2011 Nov;54(5):1842-52. (PMID: 21793028)
J Hepatol. 2013 Feb;58(2):329-34. (PMID: 23085647)
Clin Gastroenterol Hepatol. 2013 Jul;11(7):841-6. (PMID: 23353641)
BMC Med Res Methodol. 2013 Mar 06;13:33. (PMID: 23496923)
Nat Genet. 2013 Jun;45(6):670-5. (PMID: 23603763)
Hepatology. 2013 Dec;58(6):2045-55. (PMID: 23775876)
Gastroenterology. 2014 Apr;146(4):970-9; quiz e15-6. (PMID: 24389304)
Ann Hepatol. 2014 Jul-Aug;13(4):316-26. (PMID: 24927602)
Gastroenterology. 2014 Dec;147(6):1338-49.e5; quiz e15. (PMID: 25160979)
Hepatology. 2015 Jul;62(1):188-97. (PMID: 25833813)
Ann Intern Med. 2015 May 19;162(10):735-6. (PMID: 25984857)
Hepatology. 2016 Mar;63(3):930-50. (PMID: 26223498)
Hepatology. 2016 Feb;63(2):644-59. (PMID: 26290473)
Hepatology. 1989 Oct;10(4):430-6. (PMID: 2777204)
Nat Genet. 2017 Feb;49(2):269-273. (PMID: 27992413)
Gastroenterology. 2017 Jun;152(8):1975-1984.e8. (PMID: 28274849)
Gut. 2018 Oct;67(10):1864-1869. (PMID: 28739581)
Clin Gastroenterol Hepatol. 2018 Feb;16(2):278-287.e7. (PMID: 28993258)
Hepatology. 1995 Nov;22(5):1404-8. (PMID: 7590655)
Gut. 1996 Apr;38(4):610-5. (PMID: 8707097)
معلومات مُعتمدة: International Norwegian PSC Research Center; International Addenbrooke's Charitable Trust, Cambridge University Hospitals; International National Institute of Health Research; International Isaac Newton Trust; International Health Research; United Kingdom DH_ Department of Health
المشرفين على المادة: 0 (HLA Antigens)
EC 3.1.3.1 (Alkaline Phosphatase)
تواريخ الأحداث: Date Created: 20181220 Date Completed: 20200615 Latest Revision: 20230423
رمز التحديث: 20240628
مُعرف محوري في PubMed: PMC6519245
DOI: 10.1002/hep.30479
PMID: 30566748
قاعدة البيانات: MEDLINE
الوصف
تدمد:1527-3350
DOI:10.1002/hep.30479