Introduction

Primary sclerosing cholangitis (PSC) is an immune-mediated cholestatic liver disease characterized by progressive inflammation, fibrosis, and stricturing of the intra- and extrahepatic medium-large bile ducts. Due to its progressive nature, PSC can lead to life-threatening complications, including recurrent cholangitis, cirrhosis, hepatocellular carcinoma, and cholangiocarcinoma. Liver transplantation (LT) remains the only definitive treatment for patients with advanced disease. However, recurrence of PSC (rPSC) after LT remains a significant clinical challenge. Understanding recipient and donor factors associated with rPSC is crucial to optimizing patient care.

Aims & Methods

This systematic review and meta-analysis aimed to identify risk and protective factors associated with PSC recurrence following LT. The study followed PRISMA guidelines. Comprehensive searches were conducted in PubMed/MEDLINE, Embase, and Cochrane CENTRAL. Eligible studies included full-text articles reporting on LT for PSC with recurrence data. Conference abstracts and studies lacking data on recurrence predictors were excluded. Pooled estimates were calculated using a random-effects model, and heterogeneity was assessed with the I² statistic. Statistical analyses were performed using R Studio.

Results

Twenty-eight studies were included, comprising 4,161 patients who underwent LT for PSC (62.8% male). Most data originated from Europe (46.5%) and North America (31.9%), with additional cohorts from Asia (15.4%), South America (3.2%), and Oceania (3%). The reported rPSC rate ranged from 0% to 36.5%, with a pooled recurrence proportion of 18.5% (95% CI: 14.9-22.1%). Significant protective factors for rPSC included older recipient age (mean difference [MD] = 5.22 years, 95% CI: 3.62-6.82), male sex (risk ratio [RR] = 0.91, 95% CI: 0.86-0.97), younger donor age (MD = - 3.78 years, 95% CI: [-6.16; - 1.39]), and post-LT cyclosporine use (RR = 0.60, 95% CI: 0.40-0.91), while acute rejection episodes were associated with an increased risk of recurrence (RR = 1.61, 95% CI: 1.10-2.36). No significant associations were found with donor sex, donor-recipient sex mismatch, pre-LT inflammatory bowel disease diagnosis, pre/post-LT colectomy, pre-LT MELD score, recipient cytomegalovirus status, type of graft (deceased or living donor), hepaticojejunostomy, cold and warm ischemia times, or post-LT tacrolimus use.

Conclusion

This updated meta-analysis, encompassing 4,161 patients across four continents, estimates a pooled rPSC rate of 18.5%. Older recipient age, male sex, younger donor age, and cyclosporine use post-LT were associated with a lower risk of rPSC, while acute rejection episodes were associated with a higher risk of recurrence. These findings may inform clinical decision-making and support future research into strategies to reduce PSC recurrence following liver transplantation. Additional multicenter prospective studies are warranted to validate these results.

Disclosure

Rodrigo V. Motta, MD has received a research grant from PSC Support. Guilherme G. L. Cançado, MD, PhD has received a research grant from IPSEN. This study was conducted without funding support.

Introduction

Primary biliary cholangitis (PBC) is often associated with extrahepatic autoimmune diseases (EAIDs) such as Sjögren’s syndrome, autoimmune thyroiditis, and connective tissue disorders. While this association suggests a shared immunological predisposition, its effect on disease prognosis remains unclear.

Aims & Methods

This retrospective study included 69 patients diagnosed with PBC, excluding those with overlap syndromes involving autoimmune hepatitis. Patients were divided into two groups: isolated PBC (n = 35) and PBC associated with EAIDs (n = 34). The two groups were compared based on clinical, immunological, elastometric characteristics, disease progression, and prognostic scores (Paris I, Paris II, Rotterdam, Barcelona, Rochester, GLOBE, UK-PBC). Statistical analysis was conducted using appropriate tests, with a significance threshold set at p < 0.05.

Results

The average age at diagnosis was 55.6 years. The disease was initially suspected based on functional symptoms in 39% of cases (fatigue, pruritus, arthralgia), biological cholestasis in 33.3%, and decompensated cirrhosis in 20.3%. The most common associated autoimmune diseases were Sjögren’s syndrome (17.4%), thyroid disorders (13%), and systemic lupus erythematosus (10.1%). All patients received treatment with ursodeoxycholic acid at 13-15 mg/kg/day. No significant differences were found between the two groups regarding functional symptoms, biological markers (bilirubin, albumin, prothrombin time), autoantibodies (AMA, Anti-GP210, anti-SP100, anti-PML), non-invasive fibrosis scores (Fib4, Fibroindex, Fibroscan, S-index), or hepatic complications (HDH, cirrhosis, hepatic encephalopathy, liver-related death). Prognostic scores were also comparable between both groups: Paris I (p = 0.468), Paris II (p = 0.519), GLOBE score (p = 0.6), UK-PBC (p = 0.49), Rotterdam (p = 0.897), Barcelona (p = 0.181), Rochester (p = 0.727).

Conclusion

The presence of extrahepatic autoimmune diseases in PBC does not appear to affect the clinical, biological, or prognostic profiles of patients. Despite their frequency, these comorbidities do not seem to influence the progression of PBC.

Disclosure

None.

Clinical Case Summary

A 58 year-old woman was admitted for an elective rectosigmoidectomy after two previous uncomplicated diverticulitis episodes. On the third postoperative day, her clinical status deteriorated, and an abdominal CT scan revealed an anastomotic leak, leading to a new surgery and confection of a protective ileostomy. Her condition improved progressively, and she was discharged 10 days after. However, the patient came back to the emergency one week later with abdominal pain, fatigue, and refractory nausea and vomiting, in addition to acute renal failure with severe hyponatremia and hyperkalemia. Because her daily ileostomy’s output reached about 2 liters/day, she was started on high dose opioids and loperamide to decrease the stoma’s output, as well as replacement fluid therapy, improving quickly within 24 to 48 hours. She was discharged in 5 days.
Despite the adequate control of the ileostomy’s output, her second readmission occurred 12 days later, showing the same clinical and laboratorial patterns. Considering she had a typical profile for adrenal insufficiency, fludrocortisone was associated with the antimotility agents, showing a significant improvement within hours. Nonetheless, she discontinued fludrocortisone on her own, and a third readmission occurred in 10. One more time, clinical and laboratorial characteristics were the same, with rapid improvement in the short term. An extensive investigation was conducted, revealing an aldosterone level more than 10 times higher than upper limit of normal, with normal renin levels and an adrenocorticotropic hormone in the lower limit of normal. These paradoxical findings were reinforced with a low urine and high ileostomy sodium dosages, and a diagnosis of a type 3 pseudohypoaldosteronism was made. Definitive treatment with intestinal transit’s reconstruction was performed, with complete resolution.

References

1. Carl C, Dinkelbach L, Mohr J, Perez R, Vera Lopez T, Fricke-Otto S, Niehues T. Case report: Life threatening hyponatremia in infants with urinary tract infections: two cases of type III pseudohypoaldosteronism and review of the literature. Front Pediatr. 2024 Jan 5;11:1233205. doi: 10.3389/fped.2023.1233205. PMID: 38250595; PMCID: PMC10797885.
2. Amir Babiker, Haya Aldabas, Shahad Alanazi, Bashayer Alahmadi, Waleed Tamimi, Mohsen Al Atawi, Fahad Aljuraibah, Angham Almutair. Pseudohypoaldosteronism: A challenging diagnosis with management pitfalls - Case series. Journal of Clinical and Translational Endocrinology: Case Reports, Volume 32, 2024, 100172. ISSN 2214-6245
3. Stojanović, V., Spasojević, S., Radovanović, T., & Doronjski, A. (2017). Pseudohypoaldosteronism: report of three cases. Journal of Endocrinology, Metabolism and Diabetes of South Africa, 22(2), 17–20. https://doi.org/10.1080/16089677.2017.1335532
4. Nakasone, Ruka et al. Three cases of pseudohypoaldosteronism following ileostomy in preterm infants. Pediatrics & Neonatology, Volume 62, Issue 1, 119 - 121

Introduction

Eosinophilic oesophagitis [EoE] is a chronic, antigen-driven, immune-mediated oesophageal disease characterised by eosinophil-predominant inflammation. It typically presents with dysphagia, food bolus impaction, and, in children, feeding difficulties and failure to thrive [1]. Histologically, it is defined by ≥15 eosinophils per high-power field on oesophageal biopsy in the absence of other causes of eosinophilia [2]. The incidence and prevalence of EoE have increased markedly over recent decades, particularly in Western countries, likely due to increased recognition, changing environmental exposures, and altered microbiota [3,4]. Though EoE is increasingly reported worldwide, data specific to Aotearoa New Zealand, and especially regional patterns by ethnicity, remain limited.

Aims & Methods

This study aimed to describe the incidence, demographic distribution, and clinical characteristics of EoE in the Canterbury region over an 11-year period.
We conducted a retrospective analysis of electronic medical records from 2013 to 2023, identifying EoE cases using diagnostic codes and histological confirmation. Demographic data, clinical presentations, and laboratory findings were collected. Crude incidence rates were calculated using average population data for the Canterbury region, stratified by ethnicity (European vs. non-European). Clinical features including food bolus obstruction, oesophageal strictures, and peripheral eosinophilia were assessed. Incidence rates are reported per 100,000 population with 95% confidence intervals (CI).

Results

A total of 219 cases of EoE were identified between 2013 and 2023. The average annual incidence was 3.23 per 100,000 population (95% CI: 1.82–4.65), based on a regional population of 618,409. The mean number of cases per year was 20 (IQR 16–22). Among European individuals (average population 395,690), the mean annual incidence was 4.42 per 100,000 (95% CI: 2.35–6.49), with 17.5 average cases per year (IQR 14–21). Among non-European individuals (average population 222,627), the mean incidence was 1.08 per 100,000 (95% CI: -0.29–2.44), with 2.4 average cases per year (IQR 1–4).
Of the 219 cases, 70 patients (31.9%) presented with oesophageal food bolus impaction. The annual number of such presentations ranged from 3 to 12 per year (median 6, IQR 4–9). Twenty patients (9.1%) had documented stricturing disease on endoscopy, and 60 patients (27.4%) exhibited peripheral eosinophilia at diagnosis.

Conclusion

In our cohort, eosinophilic oesophagitis presents commonly with oesophageal food bolus impaction. There was a higher incidence of EoE observed in individuals of European descent. Just under one-third of patients in our cohort presented with oesophageal food bolus impaction and about 9% had stricturing disease. The frequent presentation with food bolus obstruction and the presence of complications such as strictures highlight the importance of timely recognition and management. Peripheral eosinophilia was found commonly in our cohort but the prevalence of this finding is not adequate to be used as a diagnostic test in EoE, although more studies are needed.

References

1. Dellon ES, Liacouras CA, Molina-Infante J, et al. Updated International Consensus Diagnostic Criteria for Eosinophilic Esophagitis: Proceedings of the AGREE Conference. Gastroenterology. 2018;155(4):1022-1033.e10.
2. Lucendo AJ, Molina-Infante J, Arias Á, et al. Guidelines on eosinophilic esophagitis: evidence-based statements and recommendations for diagnosis and management in children and adults. United European Gastroenterol J. 2017;5(3):335-358.
3. Hruz P, Straumann A, Bussmann C, et al. Escalating incidence of eosinophilic esophagitis: A 20-year prospective, population-based study in Olten County, Switzerland. J Allergy Clin Immunol. 2011;128(6):1349-1350.e5.
4. Arias Á, Lucendo AJ. Epidemiology and risk factors for eosinophilic esophagitis: Lessons for clinicians. Expert Rev Gastroenterol Hepatol. 2020;14(2):103–115.

Disclosure

None

Introduction

Endoscopic ultrasound-guided hepaticogastrostomy (EUS-HGS) is an effective treatment for patients in whom transpapillary biliary drainage fails. However, EUS-HGS is associated with a high incidence of early procedure-relatedadverse events (PRAEs) due to bile leakage through a biliogastrointestinal fistula.

Aims & Methods

This multicenter study retrospectively evaluated the clinical outcomes of EUS-HGS. This study included 222 patients (120 men, 102 women; median age, 73 years) who underwent initial EUS-HGS between November 2014 and August 2024. Technical and clinical success rates, PRAEs, and stent dysfunction rates were analyzed. PRAEs were defined as adverse events occurring within 2 weeks according to the assessment of the American Society for Gastrointestinal Endoscopy.

Results

Benign and malignant tumors were observed in 18 (8.1%) and 204 (91.9%) patients, respectively. The median procedure time for EUS-HGS was 41 min (range, 11–173 minutes). Plastic and metal stents were deployed in 51.4% and 48.6% of the patients, respectively. Technical and clinical success rates and PRAEs were 97.8%, 85.1%, and 22.1%, respectively. The PRAEs were classified into mild (16.7%), moderate (4.1%), or severe (1.4%). Recurrent biliary obstruction (RBO) occurred in 51 (23.1%) patients. The median time to RBO in the metal stent group was significantly longer than that in the plastic stent group (313 vs. 162 days, P=0.02). Multivariate analysis identified the use of any dilation device (P=0.01; odds ratio [OR]: 3.55; 95% confidence interval [CI]: 1.35–9.36) and concomitant antegrade stenting (P=0.03; OR: 2.62; 95% CI: 1.12–6.12) as risk factors for PRAEs.

Conclusion

EUS-HGS is feasible; however, careful follow-up is required for patients undergoing fistula dilation and concomitant antegrade stenting.

Introduction

Current colonoscopy quality indicators recommend a minimum withdrawal time (WT) of 6 minutes. However, this standard may reduce colonoscopy efficiency and does not account for endoscopists' experience or bowel preparation quality. This study aims to investigate whether the 6-minute WT remains necessary for experienced endoscopists with excellent bowel preparation (BBPS≥8) by comparing the impact of 5-minute versus 6-minute WT on adenoma detection rate (ADR) and adenoma miss rate (AMR).

Aims & Methods

This study based experienced endoscopists and excellent bowel preparation (Boston Bowel Preparation Scale score, BBPS≥8) is aimed to compare the effect of 5-minute WT versus 6-minute WT on adenoma detection rate (ADR) and adenoma miss rate (AMR).
This multicenter, randomized controlled trial involving 423 patients was conducted from June 2023 to March 2025 in 5 centers, including Wuxi People's Hospital, Jiangsu Province People's hospital, Xuzhou First People's Hospital, The Second Affiliated Hospital of Soochow University, Taizhou People's Hospital. Colonoscope was inserted and withdrew twice on each patient and all procedures for the same patient were done by the same endoscopist. Participants were randomly divided into the 5-minute WT group (the first WT-5-minute and the second WT-6-minute, n =214) and the 6-minute WT group (the first WT-6-minute and the second WT-6-minute, n=209), and a timer was used to adjust the withdrawal speed. The primary outcome was ADR and AMR.

Results

The baseline characteristics of the two groups were similar (Table 1) and colonoscopy characteristics of the patients in the two groups regarding quality of bowel preparation, cecal intubation rate, cecal intubation time, cecal intubation distance, number of abdominal compression, number of position change were comparable (Table 2). Overall ADR (39.7% vs 43.5%, P = 0.425), ADR in the first (36.0% vs 36.4%, P = 0.935) and second withdrawal (10.7% vs 13.9%; P = 0.327) were not significantly increased in the 6-minute WT group compared with the 5-minute WT group. Likewise, the overall advanced adenoma detection (AADR) (4.7% vs 5.3%, P = 0.780), AADR in the first (4.7% vs 5.3%, P = 0.780) and second withdrawal (0.5% vs 0.5%, P = 0.745) did not differ statistically. Further analysis showed that AMR (10.7% vs 13.9%, P = 0.372) was lower in the 5-minute WT group than in the 6-minute WT group, less adenomas were missed in the 5-minute WT group, although not statistically significant (Table 3).
Table 3. Lesion-level detection and miss rate in the two groups
PDR, polyp detection rate; ADR, adenoma detection rate; AADR, advanced adenoma detection rate; AMR, adenoma miss rate; AAMR, advanced adenoma miss rate; PMR, polyp miss rate.
Table 3. Lesion-level detection and miss rate in the two groups

Conclusion

Shortening WT from 6 to 5 minutes didn't reduce ADR based experienced endoscopists and excellent BBPS, a 5-minute WT benchmark was acceptable with experienced endoscopists and excellent bowel preparation quality of colonoscopy.

Introduction

Lynch syndrome (LS), the most common hereditary colorectal cancer (CRC) syndrome, is associated with an increased risk of small bowel malignancies. Given the feasibility of ileal intubation during colonoscopy, this study assesses the prevalence of ileal cancer in a large LS cohort and evaluates ileal intubation as a screening tool.

Aims & Methods

An analysis was performed on Lynch syndrome (LS) patients with ileal cancer enrolled in the prospectively maintained database of the German Consortium for Familial Intestinal Cancer (GCFIC). Demographic characteristics and cancer history were extracted. High-risk pathogenic germline variants (PGVs) were defined as MLH1, MSH2, and EPCAM, while MSH6 and PMS2 were considered low risk.
Additionally, data from 236 LS patients who underwent colonoscopy at a tertiary care center between 2018 and 2023 were retrospectively evaluated. A total of 596 complete colonoscopies were reviewed.

Results

Eight ileal cancers (0.28%) were identified, exclusively in high-risk PGV carriers (MLH1: 62.5%, MSH2: 37.5%), predominantly male (87.5%), with a median age of 56 years. Most cancers (75%) were symptom-detected; only one case was identified through screening. In the tertiary care cohort, ileal intubation was achieved in 96.6% of colonoscopies, leading to the identification of five ileal neoplasms (four adenomas, one carcinoma), all in patients with a history of right hemicolectomy or colectomy. The prevalence of ileal neoplasia in patients without an ileocecal valve was statistically significant at 7.7% (χ²(1, N = 236) = 11.8, p < .001).

Table 1: Cohort characteristics of the German Consortium for Familial Intestinal Cancer

Conclusion

Ileal intubation is a valuable extension of standard colonoscopy, especially for male high-risk PGV carriers with prior CRC and ileocecal valve removal. The strong link between ileal neoplasia and loss of the ileocecal valve suggests a role for colonic reflux, inflammation, and microbiome changes in ileal carcinogenesis.

Disclosure

Robert Hüneburg was funded by Deutsche Krebshilfe (DECADE), and Wilhelm-Sander-Stiftung.
Jacob Nattermann was funded by Wilhelm-Sander-Stiftung.