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The prevalence of cirrhosis has risen significantly over recent decades and is predicted to rise further. Widespread use of non-invasive testing means cirrhosis is increasingly diagnosed at an earlier stage. Despite this, there are significant variations in outcomes in patients with cirrhosis across the UK, and patients in areas with higher levels of deprivation are more likely to die from their liver disease. This three-part best practice guidance aims to address outpatient management of cirrhosis, in order to standardise care and to reduce the risk of progression, decompensation and mortality from liver disease. Part 1 addresses outpatient management of compensated cirrhosis: screening for hepatocellular cancer, varices and osteoporosis, vaccination and lifestyle measures. Part 2 concentrates on outpatient management of decompensated disease including management of ascites, encephalopathy, varices, nutrition as well as liver transplantation and palliative care. In this, the third part of the guidance, we focus on special circumstances encountered in managing people with cirrhosis, namely surgery, pregnancy, travel, managing bleeding risk for invasive procedures and portal vein thrombosis., Competing Interests: Competing interests: DM has received consultancy fees from Falk Pharma; SMcPherson has received personal fees outside the submitted work from Gilead, Intercept and Novonordisk and Norgine Pharmaceuticals; SMasson has received speakers fees from Dr Falk, Norgine Pharmaceuticals, Sandoz; JAL has received speakers fees from Advanz; DLS has undertaken consultancy for Norgine Pharmaceuticals, EnteroBiotix, Mallinckrodt Pharmaceuticals and ONO Pharma UK and has delivered paid lectures for Norgine Pharmaceuticals, Falk Pharma and Aska Pharmaceutical., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

The prevalence of cirrhosis has risen significantly over recent decades and is predicted to rise further. Widespread use of non-invasive testing means cirrhosis is increasingly diagnosed at an earlier stage. Despite this, there are significant variations in outcomes in patients with cirrhosis across the UK, and patients in areas with higher levels of deprivation are more likely to die from their liver disease. This three-part best practice guidance aims to address outpatient management of cirrhosis, in order to standardise care and to reduce the risk of progression, decompensation and mortality from liver disease. Here, in part one, we focus on outpatient management of compensated cirrhosis, encompassing hepatocellular cancer surveillance, screening for varices and osteoporosis, vaccination and lifestyle measures. We also introduce a compensated cirrhosis care bundle for use in the outpatient setting. Part two concentrates on outpatient management of decompensated disease including management of ascites, encephalopathy, varices, nutrition as well as liver transplantation and palliative care. The third part of the guidance covers special circumstances encountered in managing people with cirrhosis: surgery, pregnancy, travel, managing bleeding risk for invasive procedures and portal vein thrombosis., Competing Interests: Competing interests: DM has received consultancy fees from Falk Pharma. SMc has received personal fees outside the submitted work from Gilead, Intercept and Novonordisk and Norgine Pharmaceuticals. SM has received speakers' fees from Dr Falk, Norgine Pharmaceuticals and Sandoz. JAL has received speakers' fees from Advanz. DLS has undertaken consultancy for Norgine Pharmaceuticals, EnteroBiotix, Mallinckrodt Pharmaceuticals and ONO Pharma UK, and has delivered paid lectures for Norgine Pharmaceuticals, Falk Pharma and Aska Pharmaceutical Co., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

There are two distinct phases in the natural history of cirrhosis: compensated disease (corresponding to Child Pugh A and early Child Pugh B disease), where the patient may be largely asymptomatic, progressing with increasing portal hypertension and liver dysfunction to decompensated disease (corresponding to Child Pugh late B-C), characterised by the development of overt clinical signs, including jaundice, hepatic encephalopathy (HE), ascites, renal dysfunction and variceal bleeding. The transition from compensated cirrhosis to decompensated cirrhosis (DC) heralds a watershed in the nature and prognosis of the disease. DC is a systemic disease, characterised by multiorgan/system dysfunction, including haemodynamic and immune dysfunction. In this second part of our three-part series on the outpatient management of cirrhosis, we address outpatient management of DC, including management of varices, ascites, HE, nutrition, liver transplantation and palliative care. We also introduce an outpatient DC care bundle. For recommendations on screening for osteoporosis, hepatocellular carcinoma surveillance and vaccination see part one of the guidance. Part 3 of the guidance focusses on special circumstances encountered in patients with cirrhosis, including surgery, pregnancy, travel, management of bleeding risk for invasive procedures and portal vein thrombosis., Competing Interests: Competing interests: DM has received consultancy fees from Falk Pharma; SMcPherson has received personal fees outside the submitted work from Gilead, Intercept and Novonordisk and Norgine Pharmaceuticals; SMasson has received speakers fees from Dr Falk, Norgine Pharmaceuticals, Sandoz; JAL has received speakers fees from Advanz; DLS has undertaken consultancy for Norgine Pharmaceuticals, EnteroBiotix, Mallinckrodt Pharmaceuticals and ONO Pharma UK and has delivered paid lectures for Norgine Pharmaceuticals Ltd, Falk Pharma and Aska Pharmaceutical., (© Author(s) (or their employer(s)) 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Liver transplantation (LT) remains integral to the management of end-stage chronic liver disease (CLD). However, referral thresholds and assessment pathways remain poorly defined. Distance from LT centre has been demonstrated to impact negatively on patient outcomes resulting in the development of satellite LT centres (SLTCs). We aimed to evaluate the impact of SLTCs on LT assessment in patients with CLD and hepatocellular carcinoma (HCC)., Methods: A retrospective cohort study was undertaken including all patients with CLD or HCC assessed for LT at King's College Hospital (KCH) between October 2014 and October 2019. Referral location, social, demographic, clinical and laboratory data were collected. Univariable and multivariable analyses (MVA) were performed to assess the impact of SLTCs on patients being accepted as LT candidates and contraindications being identified., Results: 1102 and 240 LT assessments were included for patients with CLD and HCC, respectively. MVA demonstrated significant associations with; patients living greater than 60 min from KCH/SLTCs and LT candidacy acceptance in CLD, and less deprived patients and LT candidacy acceptance in HCC. However, neither variable was associated with identification of LT contraindications. MVA demonstrated that referrals from SLTCs were more likely to result in acceptance of LT candidacy and less likely to result in a contraindication being identified in CLD. However, such associations were not demonstrated in HCC., Conclusion: SLTCs improve LT assessment outcomes in CLD but not HCC reflecting the standardised HCC referral pathway. Developing a formal regional LT assessment pathway across the UK would improve equity of access to transplantation., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Prepregnancy counselling (PPC) is an important aspect of care for women with chronic liver disease (CLD) and liver transplantation (LT), yet its impact has not been well described. This study aims to assess the experience of women attending a joint obstetric-hepatology PPC clinic in a single-centre unit., Design/methods: A retrospective questionnaire-based study in a tertiary unit within the UK where patients who attended the PPC clinic between March 2016 and July 2021 were invited to participate by filling in a questionnaire. Descriptive data and free-text content were subsequently analysed., Results: 108 women attended the PPC clinic over a 5-year period. Overall, 58/108 (54%) completed the questionnaire. Principal concerns regarding pregnancy included fears around deterioration in health (66%), maternal death (24%), pregnancy loss (66%), medication effects (60%) and disease transmission (36%). 17/58 (14%) patients felt the presence of multiple doctors was intimidating, however, perceptions improved by the end of the consultation.Overall, 44/58 (76%) respondents felt the clinic helped them reach a decision about pursuing pregnancy. Almost all respondents would recommend the clinic to others. There were no major differences in pregnancy outcomes between those that received PPC and those that did not., Conclusion: The PPC clinic facilitates a personalised approach to care and is well received by patients with CLD/LT. It is difficult to elucidate whether attendance alone impacts on pregnancy outcomes; registry data may be better placed at addressing this important question., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Competing interests: None declared.

Introduction: Nasobiliary drains (NBDs) have been successfully used to manage intrahepatic cholestasis, bile leaks and obstructive cholangitis. It allows external drainage of bile, bypassing the ileum where bile salts are reabsorbed. We assessed the utility of placement with effect on markers of cholestasis and patient symptoms., Methods: Consecutive patients undergoing NBD over 12 years for the management of pruritus were retrospectively analysed. Recorded variables included patient demographics, procedural characteristics and response to therapy., Results: Twenty-three patients (14, 61% male) underwent 30 episodes of NBD. The median age was 26 years old (range 2-67 years old). A single procedure was carried out in 20. One patient each had two, three and five episodes of NBD. The most common aetiologies were hereditary cholestatic disease (n=17, 74%) and drug-induced cholestasis (n=5, 22%),NBD remained in situ for a median of 8 days (range 1-45 days). Significant improvement in bilirubin was seen at 7 days post-NBD (p=0.0324), maintained at day 30 (335 μmol/L vs 302 µmol/L vs 167 µmol/L). There was symptomatic improvement in pruritus in 20 (67%, p=0.0494) episodes. One patient underwent NBD during the first trimester of pregnancy after medical therapy failure with a good symptomatic response. The catheters were well tolerated in 27 (90%) of cases. Mild pancreatitis occurred in 4 (13%) cases., Conclusion: NBD can be used to provide symptomatic improvement to patients with pruritus associated with cholestasis. It is well tolerated by patients. They can be used in pregnancy where medical management has failed., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Delivering excellent healthcare depends on accurate communication between professionals who may be in different locations. Frequently, the first point of contact with the liver unit at King's College Hospital (KCH) is through a telephone call to a specialist registrar or liver fellow, for whom no case notes are available in which to record information. The aim of this study was to improve the clinical governance of telephone referrals and to generate contemporaneous records that could be easily retrieved and audited., Design: An electronic database for telephone referrals and advice was designed and made securely available to registrars in our unit., Setting: Service development in a tertiary liver centre that receives referrals from across the UK and Europe., Main Outcome Measures: Demographic and clinical data were recorded prospectively and analysed retrospectively., Results: Data from 350 calls were entered during 5 months. The information included the nature and origin of the call (200 from 75 different institutions), disease burden and severity of disease among the patients discussed with KCH, and outcome of the call. The majority of cases were discussed with consultants or arrangements were made for formal review at KCH., Conclusions: A telephone referrals and advice database provides clinical governance, serves as a quality indicator and forms a contemporaneous record at the referral centre. Activity data and knowledge of disease burden help to tailor services to the needs of referrers and commissioners. We recommend implementation of similar models in other centres that give extramural verbal advice.

Autoimmune hepatitis (AIH) is a progressive necroinflammatory liver disease associated with significant morbidity and mortality. Mainly affecting females, AIH has a varied clinical presentation from minor symptomatology to acute liver failure. The diagnosis should be considered in anyone with abnormal liver function tests. Diagnostic features include biochemical evidence of transaminitis, elevated IgG and positive autoantibodies. Liver biopsy may show interface hepatitis with portal-based plasma cell infiltrates. A clinical and pathological spectrum of disease exists with other autoimmune liver disease in rare cases. AIH responds promptly to immunosuppression therapy, including corticosteroids (prednis(ol)one or budesonide) with azathioprine. Treatment failure can be addressed with several second-line immunosuppressive agents. Liver transplantation remains a successful salvage therapy for acute autoimmune liver failure or treatment failure in chronic AIH complicated by synthetic dysfunction, portal hypertension or hepatocellular carcinoma.