Critical shortfalls in the management of PBC: Results of a UK-wide, population-based evaluation of care delivery.

Background & Aims: Guidelines for the management of primary biliary cholangitis (PBC) were published by the British Society of Gastroenterology in 2018. In this study, we assessed adherence to these guidelines in the UK National Health Service (NHS). Methods: All NHS acute trusts were invited to contribute data between 1 January 2021 and 31 March 2022, assessing clinical care delivered to patients with PBC in the UK. Results: We obtained data for 8,968 patients with PBC and identified substantial gaps in care across all guideline domains. Ursodeoxycholic acid (UDCA) was used as first-line treatment in 88% of patients (n = 7,864) but was under-dosed in one-third (n = 1,964). Twenty percent of patients who were UDCA-untreated (202/998) and 50% of patients with inadequate UDCA response (1,074/2,102) received second-line treatment. More than one-third of patients were not assessed for fatigue (43%; n = 3,885) or pruritus (38%; n = 3,415) in the previous 2 years. Fifty percent of all patients with evidence of hepatic decompensation were discussed with a liver transplant centre (222/443). Appropriate use of second-line treatment and referral for liver transplantation was significantly better in specialist PBC treatment centres compared with non-specialist centres (p <0.001). Conclusions: Poor adherence to guidelines exists across all domains of PBC care in the NHS. Although specialist PBC treatment centres had greater adherence to guidelines, no single centre met all quality standards. Nationwide improvement in the delivery of PBC-related healthcare is required. Impact and implications: This population-based evaluation of primary biliary cholangitis, spanning four nations of the UK, highlights critical shortfalls in care delivery when measured across all guideline domains. These include the use of liver biopsy in diagnosis; referral practice for second-line treatment and/or liver transplant assessment; and the evaluation of symptoms, extrahepatic manifestations, and complications of cirrhosis. The authors therefore propose implementation of a dedicated primary biliary cholangitis care bundle that aims to minimise heterogeneity in clinical practice and maximise adherence to key guideline standards.

The use of tacrolimus in the management of checkpoint inhibitor immunotherapy-induced hepatitis.

Immune-mediated hepatitis is recognised as a frequently occurring complication with the use of checkpoint inhibitor immunotherapy drugs. The mainstay of treatment involves the use of immunosuppressive agents, such as corticosteroids. Mycophenolate mofetil is added when steroid resistant. However, there are limited data to guide further management when the hepatitis remains refractory to these therapies. We present two patients who developed severe immunotherapy-induced hepatitis and required prolonged immunosuppressive therapy with three agents in combination before resolution was achieved. We demonstrate that the addition of tacrolimus can be successful when other drugs fail. We also illustrate how a subsequent wean off immunosuppression can be safely performed and highlight the use of concordant antimicrobial prophylaxis to mitigate against opportunistic infections.

The National Audit of Primary Biliary Cholangitis (PBC) in the United Kingdom: Defining the Audit Dataset and Data Collection System.

Primary biliary cholangitis (PBC) is a debilitating chronic liver disease that progresses to cirrhosis with attendant complications in a substantial proportion of patients. It is a major cause of liver-related morbidity and mortality in the United Kingdom (UK). The British Society of Gastroenterology (BSG) published guidelines on PBC management, which included key audit standards. Therefore, we propose the first UK-wide audit of the management of PBC, sanctioned by the BSG and the British Association for Study of the Liver (BASL), to benchmark NHS trusts and health boards against these audit standards as a guide to targeted improvement in the delivery of PBC-related health care.

Fatal donor-derived Kaposi sarcoma following liver transplantation.

Human herpesvirus-8 (HHV8) is a recognised precursor for a number of neoplastic and non-neoplastic processes. Immunosuppressed recipients of both solid organ and haematopoietic stem cell transplants are at risk of life-threatening lytic reactivations of HHV8-infected B-lymphocytes, primary infections after receiving grafts from HHV8-seropositive donors and more rarely by the direct transplantation of malignant Kaposi sarcoma cells seeded within graft tissue. We describe the case of an HHV8-seronegative patient with confirmed, post-orthotopic liver transplant transmission of HHV8 from a seropositive donor with quantitative evidence of viraemia and subsequent development of disseminated visceral and cutaneous Kaposi sarcoma with a rapidly fatal outcome.

Assistantship improves medical students' perception of their preparedness for starting work.

BACKGROUND: The GMC has recommended introducing student assistantships during which final year students, under supervision, undertake most of the responsibilities of a FY1 doctor. The Medical School at Queen's University Belfast in 2011/12 introduced an assistantship programme. We have evaluated the impact of the assistantship on students' perception of their preparedness for starting work. METHODS: Students were asked to complete a questionnaire at the beginning of the assistantship. It assessed the students' perception of their preparedness in five areas: clinical and practical skills, communications skills, teaching and learning, understanding the work environment and team working. After the assistantship they again completed the questionnaire. Comparison of the results allowed an assessment of the impact of the assistantship. RESULTS: There was a statistically significant improvement in the students' perception of their preparation for 49 of 56 tasks contained within the questionnaire. After the assistantship 81.2% of students felt well prepared for starting work compared with 38.9% before the assistantship. 93.9% agreed that the assistantship had improved their preparedness for starting work. CONCLUSIONS: The assistantship at Queen's University improves medical students' perception of their preparedness for starting work. The majority of medical students feel well prepared for starting work after completing the assistantship.