Arndtz Katherine, Hirschfield Gideon M
Centre for Liver Research, NIHR Birmingham Biomedical Research Centre, University of Birmingham, Birmingham, UK.
Centre for Rare Diseases, Institute of Translational Medicine, Birmingham Health Partners, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
Frontline Gastroenterol. 2017 Oct;8(4):260-266. doi: 10.1136/flgastro-2017-100815. Epub 2017 Jul 25.
Primary sclerosing cholangitis (PSC) is a rare chronic autoimmune disease with no effective therapy and a large unmet need for new treatments. Patients require significant healthcare resources over their lifetime with high rates of hospital admission, malignancy, liver transplantation and death. As a rare disease, expertise in management can be limited to large referral liver transplant programmes, and even then there is frequently variation in practice. In this case-based review, we aim to discuss common clinical dilemmas encountered by clinicians managing patients with PSC and address related competencies in the 2010 Gastroenterology Curriculum.
原发性硬化性胆管炎（PSC）是一种罕见的慢性自身免疫性疾病，目前尚无有效治疗方法，对新治疗方法的需求远未满足。患者一生中需要大量医疗资源，住院率、恶性肿瘤发生率、肝移植率和死亡率都很高。作为一种罕见疾病，管理方面的专业知识可能仅限于大型转诊肝移植项目，即便如此，实践中也常常存在差异。在本基于病例的综述中，我们旨在讨论管理PSC患者的临床医生遇到的常见临床困境，并探讨2010年胃肠病学课程中的相关能力要求。
