bluuebunny/crossref_metadata_2025_split huggingface dataset:
A205 IS REPEAT ERCP REQUIRED AFTER INITIAL ENDOSCOPIC MANAGEMENT OF POST-SURGICAL BILE LEAKS? MULTI-CENTER VALIDATION OF THE CALGARY BILE LEAK RULE
Authors: Xiao Y , Salim M , Meng Z , Khan U , Kohansal A R , Forbes N , Heitman S , James P D 
Published: February 2022

Abstract

Background
The Calgary Bile Leak Rule was developed to identify patients in whom biliary stent removal via gastroscopy could be safely performed in lieu of ERCP for post-surgical bile leaks.

Aims
This study aimed to evaluate a Modified Calgary Bile Leak Rule (MCBLR) for a cohort of patients who underwent laparoscopic cholecystectomy complicated by bile leak.

Methods
This retrospective cohort study included patients who underwent ERCP for management of laparoscopic cholecystectomy-induced bile leaks between 2005 and 2017. The primary outcome was defined as the absence of persisting bile leak or other pathology on follow-up ERCP. The MCBLR includes a) normal post-surgical serum alkaline phosphatase, b) small or absent leak with no other biliary pathology on initial ERCP, and c) time between initial and follow-up ERCP was 4–8 weeks. Test performance of the prediction rule was analyzed by calculating sensitivity, specificity, positive predictive value and negative predictive value.

Results
124 cases met inclusion criteria, of which 116 (94%) of bile leak cases had no leak identified during the follow-up ERCP. 8 (6.4%) had a persisting bile leak on follow-up ERCP. Bivariate analysis found no factors significantly associated with the primary outcome. The MCBLR demonstrated a sensitivity of 100% (95% CI 63% - 100%), a specificity of 35% (95% CI 26% - 44%), a positive predictive value of 10% (95% CI 4% - 18%), and a negative predictive value of 100.0% (91% to 100%).

Conclusions
The MCBLR demonstrated high sensitivity and negative predictive value for determining the need for repeat ERCP following endoscopic management of laparoscopic cholecystectomy-induced bile leaks.

Funding Agencies
None
A208 ASSESSING FEASIBILITY AND ACCEPTABILITY OF AN ONLINE MIND-BODY WELLNESS PROGRAM FOR PRIMARY BILIARY CHOLANGITIS
Authors: Watt M , Hyde A , Wright G M , Vander Well S , Spence J C , Mason A , McLeod M , Johnson E 
Published: February 2022

Abstract

Background
Persons with primary biliary cholangitis (PBC) experience significantly higher rates of fatigue, stress, anxiety, depression, and impaired health related quality of life (HRQOL) as compared to the general population. While online wellness programming has been shown to be effective in decreasing fatigue and improving mental wellness in a variety of chronic disease populations, limited data is available for PBC.

Aims
This pilot study aimed to assess the hypothesis that a 12-week, online, mind-body wellness program would be feasible (assessed through adherence and retention) and acceptable in people with PBC. We also aimed to explore indicators of impact on measures of wellbeing.

Methods
Persons with PBC were recruited across Alberta and British Columbia in January 2021. The program included a 20–30 minute video containing low intensity mindful movement, meditation, and breathwork (goal 2–3 times/week) as well as a weekly behaviour change tip, PBC tip from a physician, and PBC nutrition tip. The online programming was accompanied by brief (10-minute) once weekly phone check-ins from a member of the study team, and optional once monthly zoom group sessions hosted by the Canadian PBC Society. Satisfaction and adherence were assessed at the end of the study using a survey. The pre-post exploratory efficacy assessment included: fatigue (Modified Fatigue Impact Scale), perceived stress (Perceived Stress Scale), anxiety and depression (Hospital Anxiety and Depression Scale), and HRQOL (PBC-40). Using a qualitative descriptive approach, we conducted semi-structured interviews at the end of the study to explore experiences with the intervention, and gather feedback for improvement.

Results
Participants (N = 32) completed baseline surveys and 29 (91%) were retained to end-of-study. Twenty-five (86%) adhered to the program goal of carrying out the mind-body practice at least 2–3 days per week. Comparing baseline to end-of-study, significant reductions were observed in fatigue (13%, p=0.004), anxiety (30%, p=0.005), and depression (28%, p=0.022), and significant improvements were observed in the PBC-40 itch (22%, 0.043), fatigue (13%, 0.005), cognitive (17%, 0.006), and emotional (18%, 0.001) domains. Eleven individuals participated in qualitative interviews, reporting an increase in energy, a more positive outlook, and increased knowledge of PBC. Feedback supported acceptability (satisfaction score of 90%), with fatigue cited as the primary barrier to increased program participation.

Conclusions
These findings suggest that a 12-week online mind-body intervention is feasible and acceptable to persons with PBC and has promising impact on efficacy. Recognizing the limitations of a single-arm study with a small sample size, a future RCT will be designed using this feedback.

Funding Agencies
MITACS Accelerate, Canadian PBC Society
A210 HEPATOLITHIASIS IS A FREQUENT AND PROGNOSTIC FINDING IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS
Authors: Jubran B , Ismail M , Stein M , Little D H , Hansen B , Gulamhusein A , Hirschfield G 
Published: February 2022

Abstract

Aims
Intrahepatic biliary stones (hepatolithiasis) are not well characterised in patients with primary sclerosing cholangitis (PSC).

Methods
Chart reviews were conducted on 302 patients with a histologic or radiographic diagnosis of PSC followed at the Toronto Centre for Liver Disease. Radiographic data were collected for patients between the years 2008–2018. Depending on frequency of testing, magnetic resonance imaging (MRI) and ultrasound (US) data was reviewed every 3–5 years. We assessed factors associated with hepatolithiasis based on sex, race, age and phenotype of PSC and inflammatory bowel disease (IBD). Qualitative radiographic findings on image report review, episodes of cholangitis, endoscopic retrograde cholangiopancreatography (ERCP) and occurrence of cholangiocarcinoma (CCA), death and transplant were documented. Data are reported with median and IQR and analysed using χ 2 and Mann-Whitney U tests.

Results
302 patients were reviewed. The median time to follow-up, defined as from date of diagnosis to last clinic visit or to transplantation date, was 98 months (IQR = 87). The mean age at diagnosis was 38 (SD = 15.1) years; 54% of patients were male. A total of 224 patients had IBD (74%). Of the 302 patients, 80 patients (26%) had evidence of hepatolithiasis on US or MRI. Patients with hepatolithiasis were more likely to be younger (37.4 vs 39.1, p = 0.025), male (65% vs. 50%, p = 0.021), and have large duct disease (99% vs. 88%, p = 0.004). Imaging report review revealed patients with hepatolithiasis were more likely to have intrahepatic biliary thickening (76% vs. 45%, p &lt; 0.001), extrahepatic biliary thickening (69% vs. 50%, p = 0.003), focal biliary dilation (96% vs. 78%, p &lt; 0.001) and disease characterised by more reported strictures on qualitative imaging report review (89% vs 69%, p &lt; 0.001). Concomitant presence of cholelithiasis was greater in the hepatolithiasis vs. the non-hepatolithiasis group (45% vs. 19%, p &lt; 0.001). There was no significant difference in the prevalence of hepatic or portal venous thrombosis in both groups. Patients with hepatolithiasis more likely have experienced acute ascending cholangitis (50% vs. 20%, p &lt; 0.001) and need for ERCP (50% vs. 35%, p = 0.020). CCA was numerically higher in the hepatolithiasis group (8.75% vs. 4%, p = 0.1). Patients with hepatolithiasis received transplant more frequently (26.3% vs 12.2%, p &lt; 0.001) with no significant difference in mortality.

Conclusions
Hepatolithiasis is common in PSC and associated with an increased clinical and radiologic disease burden.

Funding Agencies
None
A212 LIVER TRANSPLANTATION FOR PRIMARY SCLEROSING CHOLANGITIS (PSC): EXPERIENCE FROM THE LAST THREE DECADES
Authors: Zhu J , Lim D R , James L J , Yoshida E M , Peltekian K M 
Published: February 2022

Abstract

Background
In liver transplantation (LT) for PSC, pre-transplant colectomy may lower the risk of PSC recurrence (rPSC), and well-controlled or lack of IBD may protect against rPSC, however, these findings are not consistent across all studies. It is unknown whether recipient/donor sex plays a role in rPSC.

Aims
To study factors associated with PSC recurrence in the post-transplant population.

Methods
This is a retrospective study on adults who received an LT for PSC in Halifax NS from 1985 to 2020. Graft and patient outcomes are analyzed by logistic regression models.

Results
A total of 92 patients underwent deceased donor LT for PSC with a mean follow-up time of 9.2 yrs (SD 7.1). Fifty-three remain active in the program, 2 were lost to follow up and 37 died. The mean age at transplant was 43 (SD13, range 18.9–67.8). Seven patients had a second transplant. The five- and 10-year patient survival rate of the entire cohort was 78.7% and 66% respectively. In the active cohort, the prevalence of rPSC was 23.5% (12/51). Retransplantation for rPSC occurred in 8.3% (1/12). The prevalence of IBD was 82.4% (42/51), consisted of ulcerative pancolitis (64.7%, 33/51), ulcerative proctitis (3.9%), left-sided UC (2%) and Crohn’s disease (9.8%). Males are much more likely than females to undergo a colectomy at any time (OR 5.5, 95% CI 1.07–28.22, p 0.041). Refractory IBD was the predominant indication for a colectomy (10/16), followed by dysplasia or colon cancer (6/16). In the post-transplant period, 69% had stable IBD without therapy escalation, 11.9% were escalated to a biologic, 19% underwent a colectomy for active IBD symptoms. Pre-transplant colectomy negatively predicted rPSC, in this subgroup 0% (0/6) developed rPSC. Neither recipient sex (OR 1.14, 95% CI 0.26–5.0, p 0.86) or recipient age predicted the likelihood of rPSC. There was no association between donor sex on rPSC (OR 1.25, 95% CI 0.30–5.27, p 0.76). A trend towards increased rPSC was observed in male donors to female recipients versus female-to-female transplants (OR 6, 95% CI 0.33–107.42, p 0.224). Overall, having a post-transplant colectomy, subtotal or total, irrespective of timing, did not significantly impact rPSC (OR 0.389, 95% CI 0.09–1.66, p 0.20). Diagnosis of IBD was not associated with an increased risk of rPSC (OR 1.21, p 0.83).

Conclusions
Several factors were associated with rPSC after liver transplant in patients with PSC and IBD, pre-transplant colectomy was found to be protective, male donor to female recipient was a potential risk factor. It is important to study these factors in multi-centered cohorts to understand the pathogenesis of PSC. Pre-transplant total colectomy may be beneficial for several reasons, reducing rPSC, controlling IBD activities, and lowering dysplasia and colon cancer rates in the post-transplant population.

Funding Agencies
None
A213 ERCP-RELATED ADVERSE EVENTS IN PRIMARY SCLEROSING CHOLANGITIS: A SYSTEMATIC REVIEW & META-ANALYSIS
Authors: Natt N , Michael F , Michael H , Dubois S , Al Mazrou’i A 
Published: February 2022

Abstract

Background
Endoscopic retrograde cholangiopancreatography (ERCP) is a valuable diagnostic and therapeutic tool in primary sclerosing cholangitis (PSC). Although the complications of ERCP are well known in the general population, there is conflicting data regarding complications in patients with PSC. Factors that predict ERCP-related adverse events in PSC are also unclear.

Aims
To conduct a systematic review and meta-analyses to 1. compare ERCP-related adverse events in patients with and without PSC and 2. determine risk factors associated with ERCP-related adverse events in PSC.

Methods
A systematic search was conducted in Embase, PubMed and CENTRAL for studies published from January 1, 2000 to May 12, 2021. Eligible studies included adults with PSC undergoing ERCP and reported at least one ERCP-related adverse event (bleeding, perforation, pancreatitis, cholangitis) or risk factor associated with complications. Raw event rates for adverse events and risk factors were used to calculate odds ratios (ORs) which were then pooled using random-effects models.

Results
Four studies contributed to the first meta-analysis. There was a significant three-fold increase in the 30-day odds of cholangitis in PSC compared to those without PSC (4.3% vs. 2.0%; OR 3.26, 95% CI 1.08–9.90; p=0.037; I2=73.0%) (Figure 1). However, there were no significant differences in 30-day pancreatitis (4.2% vs. 3.4%; OR 0.89, 95% CI 0.26–3.07; p=0.851; I2=87.9%), bleeding (0.3% vs. 1.1%; OR 0.36, 95% CI 0.06–2.21; p=0.272; I2=50.3%), or perforation (0.7% vs. 0.5%; OR 1.19, 95% CI 0.40–3.51; p=0.752; I2=28.5%).
In a second meta-analysis, risk factors contributing to post-ERCP pancreatitis (PEP) in PSC were pooled from five studies. While female sex was not associated with PEP, accidental passage of wire into the pancreatic duct (OR 7.44, 95% CI 3.33–16.65; p&lt;0.001; I2=65.0%) and biliary sphincterotomy (OR 4.80, 95% CI 1.92–12.03; p=0.001; I2=73.1%) were associated with higher PEP odds.

Conclusions
In the context of limited comparative data and study heterogeneity, PSC patients have higher odds of post-ERCP cholangitis despite the majority receiving antibiotics. Odds of bleeding, pancreatitis, and perforation were similar between groups. Accidental wire passage and biliary sphincterotomy increased odds of PEP, which helps identify higher-risk groups. Future studies should elucidate ERCP-related risks in PSC and guide preventive strategies.

Figure 1: 30-Day ERCP Complications

Funding Agencies
None
A215 CAUSE-SPECIFIC MORTALITY IN PATIENTS WITH CIRRHOSIS: A POPULATION-BASED COHORT STUDY
Authors: Wang P , Djerboua M , Flemming J 
Published: February 2022

Abstract

Background
Cirrhosis is a major global health concern with increasing mortality secondary to cirrhosis and chronic liver diseases observed both in the United States and Canada. However, population-level causes of death among patients with cirrhosis in North America have not been reported.

Aims
The aim of this study was to describe cause-specific mortality in patients with cirrhosis stratified by cirrhosis etiology.

Methods
Retrospective cohort study using linked administrative healthcare data from Ontario, Canada. Adult patients with cirrhosis 2000/01/01-2017/12/31 were identified and etiology of liver disease was assigned as hepatitis C (HCV), hepatitis B (HBV), alcohol-associated liver disease (ALD), non-alcoholic fatty liver disease (NAFLD), or autoimmune liver disease (AI)/other using validated algorithms for cirrhosis (sensitivity [sn] 99%, specificity [sp] 79%) and cirrhosis etiology (sn 75%-97%, sp 95%-100%). Patients were followed until death, liver transplant, or end of study. The primary outcome of cause of death was defined based on the top 10 causes of death reported by Statistics Canada; however, hepatocellular carcinoma and cholangiocarcinoma were included in liver-related deaths as opposed to malignant neoplasms. The proportion of deaths by each cause was described, stratified by cirrhosis etiology. The cumulative incidence of death with liver transplant and hepatocellular carcinoma as competing risks were calculated at 1, 5 and 10 years.

Results
202,022 patients with cirrhosis were identified (60% male sex, median age 56 years [IQR 46–67], 52% NAFLD, 26% ALD, 11% HCV, 5% HBV, 6% AI/Other). Overall, 81,428 (40%) patients died after a median follow-up of 5 years (IQR 2–12) and 3,024 (2%) patients received liver transplant. The overall leading cause of death was liver-related (32%) but varied substantially by cirrhosis etiology. Liver-related deaths were highest among those with viral hepatitis (HBV 56%, HCV 52%), and lowest in NAFLD (20%). In NAFLD cirrhosis, the most common causes of death were non-hepatic malignancy (26%), followed by a composite of cardiovascular disease, cerebrovascular disease, or diabetes (22%).

Conclusions
Although the overall leading cause of death in patients with cirrhosis is liver-related, the most common causes of mortality in patients with NAFLD cirrhosis is non-hepatic malignancy, cerebrovascular disease, and diabetes. This supports the involvement of multidisciplinary teams and healthcare providers for patients with NAFLD cirrhosis to optimize appropriate cancer screening and management of co-morbid cardiovascular and metabolic conditions.

Funding Agencies
None
A220 THE IMPACT OF DIABETES AND AGE ON PERFORMANCE OF NON-INVASIVE SERUM-BASED TESTS FOR PREDICTION OF ADVANCED FIBROSIS IN BIOPSY-PROVEN NAFLD
Authors: Kosick H M , Shengir M , Adeyi O , Sebastiani G , Patel K 
Published: February 2022

Abstract

Background
Non-alcoholic fatty liver disease (NAFLD) is now a leading cause of end-stage liver disease. Advanced stage F3-4 fibrosis predicts liver-related mortality in NAFLD patients. Simple non-invasive serum-based tests (NIT) for F3-4 are limited by indeterminate scores, necessitating secondary tests or liver biopsy. Diagnostic NIT cut-offs may vary in NAFLD patients with diabetes mellitus (DM) and the elderly. Identifying appropriate thresholds in populations in which these tests can be applied will reduce indeterminates and facilitate their broader use.

Aims
The aim of this study was to assess the impact of DM status and age on the performance of NIT for prediction of advanced fibrosis in patients with biopsy-proven NAFLD.

Methods
Patients presenting to two Canadian tertiary care centers between 2010–2018 for liver biopsy to diagnose NAFLD were included in this study. NIT including NFS, FIB4, BARD, AST-to-platelet ratio index (APRI), and AST to ALT ratio (AST/ALT) were calculated for each patient using validated cut-offs.

Results
457 patients were included in this study. Mean age was 48.8±12.9 years, 56% male, mean BMI 32.3 ± 6.7kg/m2, 69% with DM, and F3-4 prevalence 48%. Indeterminate rates for NIT were generally higher for older patients, with or without DM (27–49% and 37–52%, vs. 33–42% and 20–37%, respectively). FIB-4 and NFS both had high specificity &gt;0.9 in DM patients &lt;60 years (Table 1). There were no differences in AUROC for individual NITs between patients with and without DM, and those &lt; 60 vs. ≥ 60, nor between individual NIT within these groups.

Conclusions
DM status and age, ≥ 60 vs. &lt; 60, do not appear to have a significant impact on diagnostic performance of serum-based NIT in our cohort. Older patients had higher indeterminate results and reduced specificity, but T2DM status and age did not appear to have an impact on rate of misclassified patients. Serum-based NIT thresholds need to be optimized for older patients to reduce indeterminates and improve specificity.

Funding Agencies
None
A221 CONGENITAL PORTOSYSTEMIC SHUNTS: FROM PRENATAL DIAGNOSIS TO LONG- TERM POSTNATAL OUTCOME
Authors: Steg Saban O , Weissbach T , Achiron R , Pekar Zlotin M , Haberman Y , Kassif E , Weiss B 
Published: February 2022

Abstract

Background
Congenital portosystemic shunts (CPSS) are rare vascular malformations that result in blood bypassing the liver and re-entering the systemic circulation unfiltered. The outcomes of CPSS diagnosed prenatally are unknown. Previously published data is related to patients diagnosed after symptom appearance, including hepatic encephalopathy, liver tumors and portal hypertension.

Aims
To describe a cohort of prenatally diagnosed children with CPSS and report on the natural history and outcomes.

Methods
The study was conducted between 2006 and 2019. Prenatal diagnosis was performed during routine prenatal ultrasound. Patients were followed by the pediatric gastroenterologists’ unit.

Results
32 patients were identified; 28 patients with intrahepatic shunts and four patients with extrahepatic shunts. During follow up, Failure to Thrive (FTT) was observed in three of the patients with one shunt (16.7%), and five of the patients with two shunts or more (55.6%). The difference is significant (p- value=0.037). 24 patients with intrahepatic shunts had their shunts closed spontaneously. Median time for closure of the shunt was seven months (IQR 2–14 months, range 0–35 months). No predicting factor was detected for closure of the shunt before the age of one year. All extrahepatic shunts required surgical closure.

Conclusions
Congenital intrahepatic shunts usually close spontaneously and do not need intervention. All patients in our prenatally diagnosed cohort survived with limited to no sequelae.

Funding Agencies
None
A222 BILIARY ATRESIA IN BRITISH COLUMBIA: THE ROLE OF REFERRAL AGE AND DIAGNOSITIC EVALUATION ON OUTCOME
Authors: Smyth M , Baird R , Schreiber R 
Published: February 2022

Abstract

Background
Biliary atresia (BA), a newborn liver disease, is the leading cause of cirrhosis and liver-related death in children and the most common indication for pediatric liver transplantation (LT). The current standard of BA care is sequential surgery with an initial Kasai hepatoportoenterostomy (KP), followed by LT for those who progress to liver failure. Survival with native liver (SNL) correlates to infant age at KP with best outcome at early KP at &lt;30 days of age. Novel screening tests and diagnostic algorithms have been proposed, however the variability of clinical presentation and lack of a diagnostic test challenge early diagnosis and timely KP.

Aims
To assess age at BA presentation and subsequent investigations to the timing of KP and outcome.

Methods
A retrospective study of all BA cases referred to BC Children’s Hospital January 1, 2000-December 31, 2018. Data collection included age at referral, clinical presentation, laboratory and imaging studies, age at KP and LT. SNL and overall survival rates were determined. Descriptive statistics and data analyses using SSPS were applied.

Results
In this 19-year study, there were 48 cases (58% female) of BA in BC (1:17,000 live births). KP was performed in 41 patients and 7 had primary LT. Following the initial KP, 23 cases had LT. Median age at presentation decreased from 55 to 42 days after introduction of the BC BA stool card screening program in 2014. The Median (IQR) age at KP was 62 days (48–87). Median delay from the age at first encounter to the KP was 10 days (4–21); early referral (youngest 3rd of cohort) had a mean delay to KP of 25 days (15–40) compared with the late group (oldest 3rd of cohort) with median delay to KP of 5 days (1–8). There were 2.4 and 1.4 investigations/patient before undergoing KP in the youngest and oldest age at presentation cohorts. HIDA scan was done in 41% and 19% of the youngest and oldest presentation cohorts respectively. Median LT age was 9.6 months (8-13months). Median age at KP for patients who received LT was 77 days (53–92), compared to 52 days (41–79) in those without LT (p=0.08). All KPs were completed by 7 surgeons, each completing 1- 10 KPs. Overall patient survival and SNL were 98% and 37.5%.

Conclusions
In this cohort, SNL rates were below SNL rates in other national studies. SNL rates were higher in patients who underwent KP at a younger age, and early findings from a provincial screening program show a shift in age at presentation, potentially owing in part to increased community awareness of BA. A diagnostic algorithm that accounts for age at presentation is needed achieve timely KP.

Funding Agencies
None