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Summary: Background: High‐output intestinal fistulas and small bowel enterostomies are associated with morbidity and mortality. Current standard treatment for output reduction consists of fluid and dietary restrictions and medical therapy. There is conflicting evidence regarding the use of somatostatin analogues for output reduction. Aim: The aim of this study is to investigate whether lanreotide, added to current standard treatment, further reduces intestinal output in patients with high‐output fistulas and enterostomies. Methods: This was an open‐label, multicentre, randomised controlled trial. Adult patients with a high‐output intestinal fistula (>500 mL/24 h) or small bowel enterostomy (>1500 mL/24 h) more than 4 weeks post‐surgery and receiving standard medical treatment (dietary‐ and fluid restriction, PPI, loperamide and codeine) for at least 2 weeks were eligible for inclusion. We randomised patients 1:1 between continuing standard treatment (control), and subcutaneous lanreotide 120 mg every 4 weeks with standard treatment. The primary outcome was the number of responders, with response defined as an output reduction of ≥25%, 8 weeks after randomisation. We also investigated the proportional change in output. Results: We randomised 40 patients; 17 had a fistula and 23 a small bowel enterostomy. There were 9/20 responders in the intervention group and 2/20 in the control group (p = 0.013). The proportional output reduction was −26% (IQR −4 to −38) in the intervention group, compared to an increase of 4% (IQR 20 to −13) in the control group (p = 0.004). Conclusions: In patients with a high‐output fistula or small bowel enterostomy, addition of lanreotide to current standard treatment can provide a clinically relevant output reduction. Trial registration: EudraCT: 2013‐003998‐10. [ABSTRACT FROM AUTHOR]

In conclusion, anti-IFC antibodies are capable of inducing an acute extravascular haemolytic transfusion reaction and IFC-compatible blood should be strongly considered for elective blood transfusion in patients with CHAPLE syndrome. CHAPLE syndrome is an extremely rare autosomal recessive condition hallmarked by a complete loss of the CD55 protein, hyperactivation of complement, angiopathic thrombosis and protein-losing enteropathy based on primary lymphangiectasia.[1] The mechanism behind this syndrome is believed to reside in the fact that CD55, also known as the decay accelerating factor, mitigates complement activation and thus modulates the immune response.[[1]] Importantly, the CD55 protein harbours the entire Cromer blood group system and patients with CHAPLE syndrome characteristically produce antibodies against these antigens when exposed to red blood cells (RBCs) from CD55 SP + sp individuals.[[3], [5]] The clinical characteristics of these antibodies have not been well documented and are thus largely unclear. It is possible that anti-IFC antibodies vary between patients in their potential of causing a haemolytic transfusion reaction. [Extracted from the article]

Background: Single-operator peroral cholangioscopy (sPOCS) is considered a valuable diagnostic modality for indeterminate biliary strictures. Nevertheless, studies show large variation in its characteristics and measures of diagnostic accuracy. Our aim was to estimate the diagnostic accuracy of sPOCS visual assessment and targeted biopsies for indeterminate biliary strictures. Additional aims were: estimation of the clinical impact of sPOCS and comparison of diagnostic accuracy with brush cytology.Methods: A retrospective single-center study of adult patients who underwent sPOCS for indeterminate biliary strictures was performed. Diagnostic accuracy was defined as sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV). The clinical impact of sPOCS was assessed by review of medical records, and classified according to its influence on patient management.Results: 80 patients were included, with 40 % having primary sclerosing cholangitis (PSC). Prior ERCP was performed in 88 %, with removal of a biliary stent prior to sPOCS in 55 %. The sensitivity, specificity, PPV, and NPV for sPOCS visual impression and targeted biopsies were 64 %, 62 %, 41 %, and 84 %, and 15 %, 65 %, 75 %, and 69 %, respectively. The clinical impact of sPOCS was limited; outcome changed management in 17 % of patients. Sequential brush cytology sensitivity, specificity, PPV, and NPV were 47 %, 95 %, 80 %, and 83 %.Conclusions: The diagnostic accuracy of sPOCS for indeterminate biliary strictures was found to be inferior to brush cytology, with a low impact on patient management. These findings are obtained from a select patient population with a high prevalence of PSC and plastic stents in situ prior to sPOCS. [ABSTRACT FROM AUTHOR]

Liver transplantation for primary sclerosing cholangitis (PSC) can be complicated by recurrence of PSC (rPSC). This may compromise graft survival but the effect on patient survival is less clear. We investigated the effect of post-transplant rPSC on graft and patient survival in a large European cohort. Registry data from the European Liver Transplant Registry regarding all first transplants for PSC between 1980 and 2015 were supplemented with detailed data on rPSC from 48 out of 138 contributing transplant centres, involving 1,549 patients. Bayesian proportional hazards models were used to investigate the impact of rPSC and other covariates on patient and graft survival. Recurrence of PSC was diagnosed in 259 patients (16.7%) after a median follow-up of 5.0 years (quantile 2.5%-97.5%: 0.4-18.5), with a significant negative impact on both graft (HR 6.7; 95% CI 4.9-9.1) and patient survival (HR 2.3; 95% CI 1.5-3.3). Patients with rPSC underwent significantly more re-transplants than those without rPSC (OR 3.6, 95% CI 2.7-4.8). PSC recurrence has a negative impact on both graft and patient survival, independent of transplant-related covariates. Recurrence of PSC leads to higher number of re-transplantations and a 33% decrease in 10-year graft survival., (© 2021 The Authors. Transplant International published by John Wiley & Sons Ltd on behalf of Steunstichting ESOT.)