Achieving Improving Quality in Liver Services (IQILS) accreditation - lessons learned from one unit's experience.

In 2017 the Royal College of Physicians launched a voluntary accreditation process supported by British Association for the Study of the Liver (BASL) and the British Society of Gastroenterologists (BSG) to improve the quality and consistency of liver services across the UK and Ireland. This article describes the approach that we took and the challenges that we met on the way to achieving accreditation.

Improving compliance with the duty of candour: 5-year experience within an endoscopy department.

BACKGROUND AND AIMS: Duty of candour (DoC) is the requirement for timely and transparent disclosure after significant healthcare-related harm. We describe the experience of DoC following patient safety incidents (PSI) related to endoscopy, and offer reflections on improving compliance across other areas of clinical medicine. METHODS: PSI notified on an electronic reporting system (DATIX) from January 2015 to June 2021 were identified. Details of the procedure, level of harm and evidence of both verbal and written DoC were collected and analysed. RESULTS: 33 PSI were notified on DATIX. A verbal apology was documented in 23 cases (70%) and a written notification was offered or sent to in 20 (61%). Verbal apologies were timely, while written DoC was delayed. PSI reporting and verbal DoC increased over this period. Patients or families were invited to present questions for investigation in all 20 with written DoC. There were two claims for compensation during this period. CONCLUSION: DoC remains challenging for clinicians and patient safety teams 8 years after its inception. Improved compliance requires promotion by clinical leaders and high levels of awareness among clinical and nursing staff, a culture of openness and importantly, sustained administrative support to ensure that downstream actions are not overlooked.

Regular feedback to individual endoscopists is associated with improved adenoma detection rate and other key performance indicators for colonoscopy.

Objective: Monitoring of key performance indicators (KPIs) is a vital element of endoscopy quality improvement. Adenoma detection rate (ADR) is considered the best marker for colonoscopic quality as it inversely correlates with subsequent colonic cancer incidence and mortality, while polyp detection rate (PDR) is an easier-to-calculate surrogate for ADR. This study assessed whether regular feedback to individual endoscopists about their KPIs improved departmental performance. Methods: Individual KPIs were calculated for a period of 8 years (January 2012-December 2019) and fed back to all endoscopists at 6 monthly intervals, alongside anonymised indicators for other endoscopists, aggregate departmental performance data and benchmarks. An automated natural language processing software (EndoMineR) was used to identify adenomas in pathology reports and calculate ADR. Linear regressions were calculated for departmental ADR, PDR and other KPIs at 6 monthly intervals. Results: 39 359 colonoscopies (average 2460 in every 6-month period, range 1799-3059) were performed by an average of 42 (range 34-50) endoscopists. A continuous improvement in collective performance including ADR (12.7%-21.0%, R2 0.92, p<0.001) and PDR (19.0%-29.6%, R2 0.77, p<0.001) was observed throughout the study. Other KPIs showed similar improvement. The detection of non-neoplastic polyps did not increase. When analysed separately, ADR and PDR appeared to improve for gastroenterologists and nurse endoscopists but not for surgeons. Conclusion: Regular feedback with individual and departmental KPIs was associated with improved ADR and overall performance throughout the 8-year study period. Concomitant monitoring of ADR and PDR may prevent 'gaming' behaviour and ensure that genuine improvement is achieved.

Hepatopulmonary syndrome as the first and only manifestation of cirrhosis in a patient with hypopituitarism.

Hepatopulmonary syndrome (HPS) is characterised by the development of intrapulmonary arteriovenous blood shunts and vascular dilatation with consequent hypoxaemia, usually in the context of end-stage liver disease (ESLD). The estimated incidence of HPS in ESLD has been reported to be 13%-47%. Chronic liver disease has been described in patients with hypothalamic-pituitary dysfunction, mainly in the form of non-alcoholic fatty liver disease due to metabolic syndrome, with occasional progression to cirrhosis. We report a challenging case of a 27-year-old man with a background of hypopituitarism with no known liver disease who presented with progressive dyspnoea and hypoxaemia and was eventually diagnosed with severe HPS.

A three-tiered approach to investigating patient safety incidents in endoscopy: 4-year experience in a teaching hospital.

Background and study aims Patient safety incidents (PSIs) in endoscopy, although infrequent, can lead to significant morbidity or mortality. There is no commonly agreed strategy to investigate PSIs. We describe a three-tiered approach to investigation to facilitate appropriate action, shared learning, and timely disclosure to patients as mandated in the UK health system by the Duty of Candor (DoC). Methods PSIs were identified prospectively over a 3-year, 7-month period in a large teaching hospital. Level of investigation was agreed by a group of three senior clinicians. Levels of investigation comprised: 1) rapid desktop review; 2) departmental "mini-root cause analysis" (mini-RCA, developed internally); and 3) hospital-level RCA or mortality review. Results Of 63006 procedures there were 73 reported cases of significant harm. Eleven resulted in death. Thirty PSIs were related to hepatobiliary endoscopy, 17 to lower gastrointestinal endoscopy, and 26 to upper gastrointestinal endoscopy. Hospital-level RCA was performed in six cases, mini-RCA/mortality review in 14, and 53 were examined by the endoscopy lead. Findings were presented in an endoscopy user group (EUG) meeting. There was learning in relation to informed consent, pre-procedural radiology reviews, pre-procedural treatment, escalation planning, teamwork and communication, preparation of equipment, and recognition of delayed complications. Open and honest communication with patients and relatives was facilitated. Conclusions The introduction of an endoscopy-tailored investigation tool, the mini-RCA, as part of a three-tiered approach, facilitated investigation, appropriate action, learning, and disclosure after PSIs.

IgG4-related disease: long-term natural history and management of a relapsing multisystem disease entity.

Although awareness of IgG4-related disease has grown over the past decade, with earlier diagnosis and treatment, understanding of its natural history over the long term and the optimal management remains unclear. We report the case of a 48-year-old man who presented with a pancreatic pseudotumour causing bile duct obstruction with coexisting autoimmune hepatitis and multisystem involvement. His symptoms settled on steroids and maintenance with azathioprine was commenced, however periodic relapses occurred involving multiple organs. A timeline-relating IgG4 levels, clinical features and immunosuppressive therapy are presented. The protean and relapsing-remitting nature of this condition is emphasised, and a brief review of long-term therapeutic options is provided.

Pitfalls in histoacryl glue injection therapy for oesophageal, gastric and ectopic varices: A review.

Histoacryl glue is used increasingly for the treatment of gastric and ectopic varices, and there is experience in its use for oesophageal varices. It is an effective treatment, yet numerous reports of complications have accumulated. This review of the literature describes the technique, explores circulatory and vascular consideration unique to portal hypertension and categorises the complications into: "Embolisation", "local venous thrombosis", "fistulisation and extravascular injection", "ulceration, erosion and extrusion", and "nidus of infection". A case is then made for standardisation of the technique and the consent process.

Clinical and Pathophysiological Characteristics of Cirrhotic Patients with Grade 1 and Minimal Hepatic Encephalopathy.

BACKGROUND AND AIMS: EASL/AASLD hepatic encephalopathy (HE) guidelines proposed the alternative use of the term 'Covert HE' combining minimal HE (mHE) and Grade 1 HE into a single entity. However, longitudinal data to indicate that these are indeed a single entity are lacking. The aims of this study were to determine whether the occurrence of complications of cirrhosis requiring hospital admission and mortality were similar in these sub-groups of patients. METHODS: Clinically-stable cirrhotic patients (n = 106) with no previous history of 'Overt HE' were included over a 2-year period and classified as having no HE (n = 23), mHE (n = 39) or Grade 1 HE (n = 44). Standard biochemistry, venous ammonia, bacterial DNA and neutrophil function were measured at inclusion and the patients were followed for a mean of 230±95 days. RESULTS: Patients with Grade 1 HE had significantly more complications requiring hospitalisation (infection 9/18/34%; HE 4/8/18%; other 13/10/11%; P = 0.02) and significantly greater mortality (4/5/20%; P = 0.04) compared to patients with no HE or mHE respectively. Patients with mHE and grade 1 HE had similar ammonia levels, but higher than the no HE group (P<0.001). MELD score was similar between groups but Grade 1 HE patients had increased frequency of bacterial translocation (P = 0.06) and neutrophil spontaneous respiratory burst (P = 0.02) compared to patients with mHE. CONCLUSIONS: The results of this study show for the first time that 'Covert HE' is a heterogeneous entity with significantly greater hospitalisations and mortality in the Grade 1 HE patients compared with mHE. Further prospective longer-term studies are required before EASL/AASLD guidance is fully implemented.

Gastric variceal bleeding from segmentary idiopathic splenic vein stenosis with left-sided portal hypertension: An unappreciated complication of pregnancy.

Segmentary idiopathic splenic vein stenosis is a very rare condition. We report a unique case of acute gastric variceal bleeding in a 31-year-old pregnant woman with left-sided portal hypertension from segmentary idiopathic splenic vein stenosis. Hemorrhage was controlled by endoscopic acrylate glue injection and urgent cesarean section allowed successful delivery. The patient declined subsequent intervention and has been on beta-blockers with no bleeding recurrence since then. This condition, its pathophysiological implications and management are discussed.

Liver replacement therapy.

The realization of a support device able to effectively replace liver function in patients with hepatic failure has thus far been an elusive goal. The complexity of liver metabolic, synthetic, detoxifying, and excretory functions make artificial hepatic support extremely challenging. Currently, no specific treatment is available to reverse acute or acute-on-chronic liver failure, and morbidity and mortality of these syndromes are still high. Present management strategies are supportive, while waiting for spontaneous liver regeneration or liver transplant. Because of the scarcity of donor organs, liver support strategies are needed for patients with inadequate liver function until an appropriate organ becomes available for transplantation or until their liver recovers from injury. Currently available liver support systems comprise nonbiological systems (e.g., hemodiafiltration, albumin dialysis, and plasma exchange) and bioartificial systems utilizing viable liver cells. The role for these novel systems and their impact on survival or other clinically important outcomes are controversial. Development and use of bioartificial systems are limited by the inherent cost.

Evidence of neutrophil functional defect despite inflammation in stable cirrhosis.

BACKGROUND & AIMS: Deranged neutrophil function in alcoholic hepatitis has been shown to be transmissible to normal neutrophils by patient plasma. The aims of this study were (i) to evaluate whether patients with stable cirrhosis have a similar transmissible neutrophil defect and (ii) to explore the possible mechanisms. METHODS: Plasma samples from 108 stable cirrhotic patients (Child A or B: 58; Child C: 50) and matched controls were incubated with normal neutrophils. Neutrophil resting respiratory burst, phagocytosis, and toll-like receptors 2, 4, and 9 expressions as well as plasma endotoxin, bacterial DNA, and cytokines were measured. In a separate study, eight patients and five controls were studied using a novel 'skin-window' technique to evaluate neutrophil function in an area of induced sterile inflammation. RESULTS: Patient plasma induced neutrophil phagocytic dysfunction was greater in patients with more severe disease and was associated with increased expression of toll-like receptors 2 and 4. An increased resting respiratory burst was observed in a subset of patients, showing higher levels of inflammatory cytokines and more pronounced phagocytic impairment. No correlation was found with endotoxemia or bacterial DNA. In patients with compensated cirrhosis and apparently normal neutrophil function, the 'skin-window' study disclosed a severe phagocytic defect at the site of inflammation. Significantly higher levels of neutrophil elastase and IL-8 were found in the blister fluid. CONCLUSIONS: Stable cirrhosis is characterized by neutrophil phagocytic dysfunction which may be subtle and only revealed in inflamed peripheral tissues where excessive inflammatory mediators continue to be released.

Preserved liver function, portal thrombosis and absence of oesophageal varices are risk factors for metastasis of hepatocellular carcinoma.

BACKGROUND: Extrahepatic metastases represent a major obstacle for further improving prognosis of hepatocellular carcinoma. AIM: To assess clinical predictors of extrahepatic metastases in a large cohort followed in a single centre. METHODS: We evaluated clinical files of 520 consecutive patients with hepatocellular carcinoma admitted from 1994 to 2002 to our Liver Unit. The following risk factors were assessed: age, gender, hepatitis viruses, alcohol, diabetes, size, number and differentiation of hepatocellular carcinoma, percutaneous biopsy, portal thrombosis, alpha-fetoprotein, Child-Pugh, Cancer Liver Italian Program and Model for End-stage Liver Disease scores, Barcelona Clinic Liver Cancer classification, varices, hepatocellular carcinoma treatment. RESULTS: Extrahepatic metastases were detected in 55/520 patients (10.5%) after 0-72 months (median 15, CI 3-20) from initial evaluation. Lower bilirubin, INR, Child-Pugh and Model for End-stage Liver Disease scores, higher alpha-feto protein levels, portal thrombosis and absence of oesophageal varices were all associated with distant metastases at univariate analysis. Absence of oesophageal varices and portal thrombosis resulted as independent predictors (P = 0.0003 and P = 0.004, respectively) on multivariate logistic regression. Patients with metastases showed poorer survival (3 months) than total hepatocellular carcinoma population (26 months). CONCLUSIONS: Extrahepatic metastases of hepatocellular carcinoma are rare but significantly impair prognosis. Extrahepatic metastases were more frequent in patients with well preserved liver function. Absence of oesophageal varices and presence of portal thrombosis were the strongest risk factors.

Effect of alcohol, cigarette smoking, and diabetes on occurrence of hepatocellular carcinoma in patients with transfusion-acquired hepatitis C virus infection who develop cirrhosis.

AIM: Alcohol drinking, cigarette smoking, and diabetes have been claimed as risk factors for hepatocellular carcinoma in case-control studies. The aim of this study was to define the impact of these risk factors on the development of hepatocellular carcinoma in hepatitis C virus-related liver cirrhosis. METHODS: A historical cohort of 138 patients with posttransfusion hepatitis C virus-related cirrhosis was selected by reviewing all files of patients referred to our liver unit. Sixty-three of them (46%) developed hepatocellular carcinoma. RESULTS: At univariate analysis, risk factors for hepatocellular carcinoma were observed in patients aged above 59 years [P=0.004; relative risk (RR): 2.08, 95% confidence interval (CI): 1.19-3.68], male sex (P<0.001; RR: 2.48, 95% CI: 1.59-3.87), habit of alcohol drinking (P=0.001; RR: 1.89, 95% CI: 1.24-2.88), and duration of alcohol consumption of more than 30 years (P=0.02; RR: 2.08, 95% CI: 0.98-4.40). At Cox regression analysis, only male sex was an independent predictive factor (beta=0.86; P=0.002; hazard ratio=2.4, 95% CI: 1.3-4.1). CONCLUSION: Diabetes, smoking, and alcohol drinking were not independently related to the risk of developing hepatocellular carcinoma in hepatitis C virus-related cirrhosis.

Fatal ischemic acute pancreatitis complicating trans-catheter arterial embolization of small hepatocellular carcinoma: do the risks outweigh the benefits?

Hepatocellular carcinoma (HCC) is an accepted indication for liver transplantation (LT). Pre-LT adjuvant ablation treatments to prevent tumour progression and drop out from the waiting list have been increasingly adopted at most transplant centers. Trans-catheter arterial chemo-embolization (TACE) is frequently used, but the procedure can be difficult and severe complications may arise. Among them, acute ischemic pancreatitis occasionally occurs and may clinically mimic a post-embolization syndrome. Fatal outcomes of this complication have been reported exceptionally but never in patients awaiting LT. The present case raises concern about the widespread application of TACE and highlights the need for a critical evaluation of the risks and benefits to patients with monofocal small HCC who are scheduled for LT. Superselective embolization of the tumour-feeding artery and systematic monitoring of serum pancreatic enzymes after this radiological procedure are recommended.

Sustained virological response to antiviral therapy reduces mortality in HCV reinfection after liver transplantation.

BACKGROUND/AIMS: HCV infection recurs almost in all HCV-positive patients receiving liver transplantation and carries a poor prognosis. Aim of this study was to analyze efficacy and effect on survival of antiviral therapy in this clinical setting. METHODS: Pegylated-interferon alpha-2b and ribavirin were administered at a dose of 1 microg/kg of bwt weekly and 600-800 mg/day. Planned duration of treatment was 24 or 48 weeks according to HCV genotype. Patients who failed to respond at week 24 were considered as non-responders. RESULTS: 61 patients were enrolled. According to intention-to-treat analysis, 44 (72%) patients were considered as treatment failure (31 non-responders, 4 relapsers, 9 dropout). Sustained virological response was achieved in 17 cases (28%). Genotype 2, higher doses of antivirals and absence of histological cirrhosis were predictors of sustained virological response. In the follow up, patients with sustained virological response had a significantly lower mortality compared to patients with treatment failure (chi2=6.9; P<0.01). CONCLUSIONS: Response rate to antiviral therapy in HCV reinfection after liver transplantation is higher if a full dose of antiviral drugs is administered and if treatment starts before histological cirrhosis has developed. Sustained virological response improves patient survival.

Prognostic value of quantitative liver function tests in viral cirrhosis: a prospective study.

BACKGROUND AND AIMS: Widespread application of quantitative liver function tests as a prognostic tool is controversial. In this study we assessed the predictivity of serial evaluations of galactose elimination capacity (GEC) and the monoethylglycinexylidide (MEGX) test on survival in viral cirrhosis, and secondarily we compared these tests with Child-Turcotte-Pugh (CTP) and Model for End Stage Liver Disease (MELD) scores. METHODS: In a cohort of 35 patients with viral cirrhosis, GEC and MEGX were evaluated every 6 months for 24 months and compared with CTP and MELD scores at the same time intervals. The end points were patient death or liver transplantation. RESULTS: Statistically significant differences between dead/transplanted patients and survivors were found for basal values of GEC, MEGX, CTP and MELD. Receiver-operating characteristics curves of CTP and MELD scores showed a higher prognostic accuracy than GEC and MEGX. On multivariate analysis, neither GEC nor MEGX were independent predictors of survival. Repeated-measures analysis of GEC and MEGX did not increase the prognostic accuracy of these tests and did not add useful prognostic information on patient outcome during the following 6 months. CONCLUSIONS: Our data suggest that neither single nor repeated determinations of GEC and MEGX are superior to CTP and MELD scores in predicting prognosis of patients with viral cirrhosis.