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The introduction of Cystic Fibrosis Trans Regulatory modulator (CFTRm) drugs has seen a transformation in Cystic Fibrosis (CF) treatment. This has led to a significant improvement in lung function and quality of life with the potential for a real impact on life expectancy. Transient mild to moderate hepatic transaminitis is a well-known side effect of CFTRm drugs, which often improves on cessation and may not recur following the re-institution of the drug. We describe a case of transaminitis developing nine months after the initiation of Kaftrio, which progressed to liver necrosis despite stopping Kaftrio and took many months to resolve. The patient had experienced significant improvement in lung function and overall health while on Kaftrio and deteriorated when it was stopped. He was keen to restart; however, Kaftrio was not reinstated due to the potential risk of acute liver failure.

Introduction Non-alcoholic fatty liver disease (NAFLD) is a significant public health concern. Rates are increasing due to increasing levels of obesity. Early identification of patients in primary could prevent progression to end stage liver failure. The aim of this project is to pilot introduction of a screen for NAFLD and liver fibrosis into the existing NHS Health Check. Simple fibrosis scores have been extensively evaluated in a secondary care setting, however their utility in primary care has not been established. Methods Five GP practices took part in the pilot. The NHS Health Check is offered by GPs to any patient aged 40–74 years who is not already on a disease register. Patients who attended for this were screened to determine if they met the inclusion criteria. Patients were included if they had an increased BMI (≥30 or≥27.5 in high risk ethnicities) or waist circumference (>94 cm men or >90 cm men high risk ethnicities and >80 cm women) and low alcohol intake ( Results Out of the 82 included patients, 64 (78%) had at least one increased fibrosis score. Twenty-six patients with raised fibrosis score/s were reviewed in outpatient clinic and were offered further investigations. Of 15 patients who had an US scan 10 (66%) had fatty liver and the rest were normal. Twenty-one patients had liver elastography and 1 was found to have possible fibrosis with a fibrosis score of 8.1 kPa. Subsequent liver histology showed fibrosis, however it was thought to be drug induced. Conclusions Informal feedback from the staff performing the NHS Health Check indicated that incorporating the liver screen was not burdensome. However, based on these Results simple fibrosis markers are not a useful screening test for use in primary care, as the high false positive rate Resulted in too many unnecessary referrals to secondary care to exclude fibrosis. It remains unclear how patients with liver fibrosis can be easily identified in primary care.

Introduction Severe Alcoholic Hepatitis (AH) (defined as a Maddray score >32) is a life-threatening condition with a recently reported 1 month mortality of 14% and 1 year mortality of 57%. The current mainstay of treatment is corticosteroid therapy, but previous studies suggest adding N-acetylcysteine (NAC) improves short-term mortality. We assess the effect on mortality of NAC as additional treatment for severe AH. Methods We collected data using a standard proforma for patients admitted and diagnosed with severe AH (Maddray score of >32). Patients were treated with prednisolone (40 mg/day) and 5 days of IV NAC (at a dose of 150, 50, and 100 mg per kilogram of body weight in 250, 500, and 1000 ml of 5% glucose solution over a period of 30 mins, 4 hours, and 16 hours, respectively) and on days 2 through 5 (100 mg per kilogram per day in 1000 ml of 5% glucose solution). Patients were collected between 1 st of May and 30th of October. We calculated the Lille score on day 7 of treatment and continued prednisolone in responders. Analysis was on a per-protocol basis. Mortality was assessed at 1, 3 and 6 months. Results 10 patients were included. Mortality is show in table 1 and compared to previous trials. The mean age of patients included was 51.6±11.06. Participants baseline characteristics were consistent with previous publications Maddray score 61±27.9, Bilirubin 233.6±119.3, Prothrombin time (PT) 21.1+/-5.7. Over the six months, there were 4 episodes of infection (40%) and 0 episodes of hepatorenal syndrome. After treatment the mean Lille score was 0.433±0.31. Of those patients that died there was a significant difference compared to patients who survived in initial Maddray score at 30 days (Alive: 53.4±23.2, Dead: 95.5±16.3 (p=0.041)) and at 6 months (Alive 38.6±16.1, Dead 83.4±15.4 (p=0.002)). There was no significant difference in Lille scores in patients that died at 1 or 6 months. Conclusions Treatment with combination of NAC and corticosteroids demonstrated slightly worse outcomes compared with recent trials although our numbers are too small to be certain. This study suggests significantly elevated Maddray scores are associated with an increased risk of mortality in severe AH. We feel a larger study to validate previous data and fully assess the effect of NAC in the treatment of AH on short term mortality is needed.