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Background: A National Registry for Wilson disease (WD) was recently started by the Spanish Association for Study of the Liver (AEEH). We evaluated the genetic data of the patients and the differences between regions and hospitals., Method: Multicentric observational study from the WD Registry after the first year., Results: Patients from 30 hospitals, in 13/17 Spanish regions (covering 80% of population) were included. Genetic data were available for 260/320 patients. More than 130 mutations in the ATP7B gene were registered, the majority in less than 4 alleles, being most prevalent p.Met645Arg (20% alleles, mainland and the Canary Islands), p.Leu708Pro (16.5%, Canary Islands) and p.His1069Gln (8.3%, mainland). Only 15 mutations occurred in homozygosis, 3 in more than 5 patients: p.Leu708Pro (24 patients), p.Met645Arg and p.His1069Gln (6 patients each). Genetic data availability ranged from 0-100% among regions; similarly, the difference among hospitals within a region was larger than 50%. Without a genetic test up to 45% of patients would not have reached Leipzig score >3. In screening cases, genetics was used in 45/58 (78%); without genetics, two thirds of them would not have reached a Leipzig score >3 (33/49)., Conclusion: Here we present the first genetic map of WD in Spain. The use of genetic test was highly heterogeneous, being higher in screening cases. Although a large variability of mutations was found, regional characteristics indicated that screening for a limited number of exons (6, 8, 10, 14 or 17) would detect more than 50% of alleles in a given region, thus enabling the design of better diagnosis/screening tailored strategies.

Spain is worldwide leader in deceased donation rates per million habitants and count on a strong network of twenty-five liver transplant institutions. Although the access to liver transplantation is higher than in other countries, approximately 10% of patients qualifying for liver transplantation in Spain will die in the waiting list or would be excluded due to clinical deterioration. A robust waiting list prioritization system is paramount to grant the sickest patients with the first positions in the waiting list for an earlier access to transplant. In addition, the allocation policy may not create or perpetuate inequities, particularly in a public and universal healthcare system. Hitherto, Spain lacks a unique national allocation system for elective liver transplantation. Most institutions establish their own rules for liver allocation and only two autonomous regions, namely Andalucía and Cataluña, share part of their waiting list within their territory to provide regional priority to patients requiring more urgent transplantation. This heterogeneity is further aggravated by the recently described sex-based disparities for accessing liver transplantation in Spain, and by the expansion of liver transplant indications, mainly for oncological indications, in absence of clear guidance on the optimal prioritization policy. The present document contains the recommendations from the first consensus of waiting list prioritization for liver transplantation issued by the Spanish Society of Liver Transplantation (SETH). The document was supported by all liver transplant institutions in Spain and by the Organización Nacional de Trasplantes (ONT). Its implementation will allow to homogenize practices and to improve equity and outcomes among patients with end-stage liver disease.

Background and Aims: large clinical trials and small real-world studies show that a 1-L polyethylene glycol and ascorbic acid solution (1-L PEG-ASC) is an effective and safe bowel preparation for colonoscopy. Here, the effectiveness and safety of 1-L PEG-ASC was evaluated in a large cohort of patients in routine clinical practice in Spain., Methods: a sub-analysis was performed in an observational, multicenter, retrospective study assessing the effectiveness and safety of 1-L PEG-ASC in adult patients undergoing a colonoscopy at ten centers in Spain. Cleansing quality was assessed with the Boston Bowel Preparation Scale, scores ≥ 6 with all segmental scores ≥ 2 were considered as adequate colon cleansing, and high-quality was considered as cleansing ≥ 8 or = 3 in the right colon. Polyp and adenoma detection rates, and adverse events were also assessed., Results: data were collected from 7,160 patients; 48.3 % were males, mean age was 58.0 and 33.6 % were ≥ 65 years old. Adequate overall bowel cleansing was achieved in 95.6 % of patients (95 % CI: 95.1-96.0 %), high quality cleansing in 74.4 % (95 % CI: 73.4-75.4 %) and high-quality right colon cleansing in 66.0 % (95 % CI: 64.9-67.1). The adequate overall cleansing rate was 97.0 % with a split-dose and 94.0 % with same-day regimen (p < 0.0001), and high-quality right colon cleansing was 69.0 % and 62.5 % (p < 0.0001), respectively. Colonoscopy was completed in 97.2 % of cases. A multivariate regression analysis revealed that an overnight split-dose regimen and age < 65 years were independent predictors of adequate bowel cleansing of the overall colon, age < 65 years and female gender were independent predictors of high quality (HQ) cleansing of the overall colon, and the three covariates were independent predictors of HQ cleansing of the right colon. At least one adverse event was experienced by 3.3 % of participants, with nausea (1.5 %) and vomiting (1.2 %) being the most frequent., Conclusion: this sub-analysis confirmed 1-L PEG-ASC to be an effective and safe bowel cleansing preparation in a real world setting in Spain.

Esophageal squamous cell carcinoma (ESCC) remains the most prevalent subtype of esophageal cancer worldwide. Major risk factors include chronic alcohol consumption and heavy smoking. We present the case of a 65-year-old male with chronic alcoholism and a 75 pack-year smoking history, admitted for his first hydropic decompensation of alcoholic cirrhosis. The patient presented with abdominal distension and discomfort. Physical examination confirmed ascites, and further evaluation indicated decompensated cirrhosis. An upper gastrointestinal endoscopy revealed a 15 mm flat mucosal lesion located 35 cm from the dental arcade, classified as IIb according to the Paris Classification. The lesion had a denuded surface, and narrow band imaging (NBI) showed a brownish appearance with abnormal intrapapillary capillary loops (IPCL), suggesting a degenerative squamous lesion of the esophagus. A computed tomography (CT) scan showed no signs of distant metastasis. Histopathological examination of biopsies confirmed high-grade dysplasia (carcinoma in situ).

Endoscopic findings in primary or secondary intestinal lymphoma are non-specific, which can lead to finding a polyp, an ulcer or a completely normal mucosa. As a matter of fact, this makes having a high clinical suspicion with only the endoscopic technique, quite difficult. Due to this reason, we believe that either taking random biopsies or biopsies from visible lesions of affected sections in another imaging tests, should be indicated in order to increase the diagnostic capacity of the endoscopic technique.

A 15-year-old boy was admitted to the hospital due to ataxia, drowsiness and bradypsychia. He was known to have a short bowel syndrome Initial venous blood gases revealed a metabolic acidosis with a high anion gap of 24 mmol/L and normal L-lactate. He improved with fasting and fluids and was discharged with oral metronidazole. 2 weeks later he was admitted again with similar symptoms. A specific study of D-Lactic acidosis was carried out, confirming the diagnosis. D-lactic acidosis is an uncommon complication of short bowel syndrome. It occurs as a consequence of the metabolism of unabsorbed carbohydrates. The symptoms are mainly neurological. Limiting the dietary carbohydrates is useful to avoid recurrences. Poorly absorbable antibiotics are used but with varying results. Surgery may be an option if medical treatment fails. Probiotics might be useful to avoid symthoms recurrence.

Introduction: Metabolic syndrome (MS) and cardiovascular risk factors are common in liver transplant (LT) candidates and recipients. Cardiovascular events and de novo tumors are increasingly common causes of mortality in liver transplant recipients. The aim of this study were (i) assess the prevalence of MS in LT recipients and its growth over the years and (ii) determine if the presence of MS pre-LT is associated with a higher risk of post-LT cardiovascular events (CVE), de novo tumors or early and late survival., Patients and Methods: A retrospective study was performed that included LT recipients from January 2012 to December 2017. Baseline features (MS before LT and at 1year post-LT) and outcomes (CVE, de novo tumors and survival) were recorded., Results: 483 recipients were included, MS was present in 20% of pre-LT with an increasing prevalence over time, from 16% in 2012 to 34% in 2017 (p=0.025). One-year post-LT, an additional 12% had developed de novo MS. At a median of 56-months follow-up, 13% developed a CVE and 9% a de novo tumor. One and 5-yr survival rates were 91% and 83% in those with pre-LT MS and 93% and 85% in those without (p=0.94).The presence of MS before LT was independently associated with a higher risk of post-LT CVE (HR: 2.66 IC (95%): 1.6-4.4 p< 0.001), but not with de novo tumors (p=0.94) nor early and late survival (p=0.58 and p=0.87)., Conclusion: Pre-LT MS is increasing among LT candidates and is associated with a higher risk of post-LT morbidity CVE yet without affecting mortality.  .

Background and Aim: reduction in calcineurin inhibitor levels is considered crucial to decrease the incidence of kidney dysfunction in liver transplant (LT) recipients. The aim of this study was to evaluate the safety and impact of everolimus plus reduced tacrolimus (EVR + rTAC) vs. mycophenolate mofetil plus tacrolimus (MMF + TAC) on kidney function in LT recipients from Spain., Methods: the REDUCE study was a 52-week, multicenter, randomized, controlled, open-label, phase 3b study in de novo LT recipients. Eligible patients were randomized (1:1) 28 days post-transplantation to receive EVR + rTAC (TAC levels ≤ 5 ng/mL) or to continue with MMF + TAC (TAC levels = 6-10 ng/mL). Mean estimated glomerular filtration rate (eGFR), clinical benefit in renal function, and safety were evaluated., Results: in the EVR + rTAC group (n = 105), eGFR increased from randomization to week 52 (82.2 [28.5] mL/min/1.73 m2 to 86.1 [27.9] mL/min/1.73 m2) whereas it decreased in the MMF + TAC (n = 106) group (88.4 [34.3] mL/min/1.73 m2 to 83.2 [25.2] mL/min/1.73 m2), with significant (p < 0.05) differences in eGFR throughout the study. However, both groups had a similar clinical benefit regarding renal function (improvement in 18.6 % vs. 19.1 %, and stabilization in 81.4 % vs. 80.9 % of patients in the EVR + rTAC vs. MMF + TAC groups, respectively). There were no significant differences in the incidence of acute rejection (5.7 % vs. 3.8 %), deaths (5.7 % vs. 2.8 %), and serious adverse events (51.9 % vs. 44.0 %) between the 2 groups., Conclusion: EVR + rTAC allows a safe reduction in tacrolimus exposure in de novo liver transplant recipients, with a significant improvement in eGFR but without significant differences in renal clinical benefit 1 year after liver transplantation.

Background and Aim: fifty to 70 percent of pancreatic neuroendocrine tumors are diagnosed incidentally. The objective of this study was to compare the phenotype and oncological outcomes of incidental versus symptomatic pancreatic neuroendocrine tumors., Methods: a retrospective study was performed, identifying all incidental and symptomatic tumors resected between 2000 and 2019. Baseline characteristics, symptoms, operative variables and pathological stage were all recorded. Patterns of recurrence and overall and disease-free survival were analyzed in both groups., Results: fifty-one incidental and 45 symptomatic pancreatic tumor resections were performed. Symptomatic tumors were more frequent in females (29 vs 17; p = 0.005) and younger patients (median years; 50 vs 58; p = 0.012) and were detected at a more advanced stage (p = 0.027). There were no differences in location and most resections (n = 49; 51 %) were performed laparoscopically. There were no operative mortalities and 17 (17.7 %) severe complications (≥ IIIb on the Clavien-Dindo classification) were recorded with no differences between the two groups. With a median follow-up of 64.4 months (range 13.5-90), overall survival at five and ten years was 89.7 % and 72.8 % for the non-incidental tumors and 80.9 % and 54.6 % for the incidental tumors (p = ns), respectively. Disease-free survival in both groups (excluding M1a) was 71.2 % and 47.5 %, and 93.7 % and 78.1 %, respectively (p = ns)., Conclusions: symptomatic tumors are more frequent in females and present at more advanced pathological stages. There were no significant differences in overall and disease-free survival between the two groups. Resection of incidental tumors ≥ 1.5-2 cm seems advisable, although each case should be assessed on an individual basis.

We report the case of a 61-year-old woman having corticoid treatment with corticosteroids for polyarthralgia, who underwent a post-polypectomy surveillance colonoscopy, identifying a 5-mm diameter, flat-elevated polyp in the proximal transverse colon (Paris 0-IIa).

The portal venous aneurysm(PVA) is a very rare entity, which can be congenital or acquired (if portal hypertension, cirrhosis, pancreatitis, surgical history). Although the discussion is still ongoing, treatment would be indicated in symptomatic PVA, over 30mm or progressive growth. In order to provide an update on the subject due to the low incidence of this finding, we present the case of a 51-year-old woman who comes to the ER for painful obstructive jaundice; the ultrasound shows the presence of cholelitiasis and choledicolitiasis and identifies a nodular image in continuity with the right portal branch of 25x22x21 mm. This dilation is confirmed by MRI. After committee evaluation, follow-up was decided and to reserve the possibility of embolization if growth or symptomatology appeared.

Liver failure (LF) is a severe entity, which represents a diagnostic challenge for clinicians. Knowing the etiology of liver aggression is key for its management. We present a rare case of cholestatic LF secondary to primary AL (kappa-light chain) amyloidosis as a presentation associated to multiple myeloma.

Background: chronic liver disease (CLD) patients often present thrombocytopenia (TCP) and when severe, it may prevent them from undergoing necessary invasive procedures due to an increased bleeding risk. The lack of scientific evidence makes it impossible to determine key aspects of the current management and associated healthcare burden of these patients in Spain., Purpose: to gain insight into the current situation of patients with CLD-associated severe TCP undergoing invasive procedures in Spain, based on the experience of clinical experts., Methods: national Delphi study involving 32 medical experts., Results: the estimated prevalence of CLD-associated severe TCP is approximately 5,967, with an annual incidence of 1,148 new patients. Patients undergo a median of 1 (0-3) invasive procedures/year. Platelet transfusions (PTs) are the standard option to raise platelet counts and are associated with significant burden. The achievement of target platelet levels (≥ 50 x 109/l) after a transfusion is not routinely measured. The lack of effectiveness and short life span of transfused platelets can lead to procedure cancellations and bleeding events, which potentially affect patient outcomes. Adverse events occur in 1-25 % of patients, including mild (febrile and allergic reactions) and severe events (e.g., transfusion-related acute lung injury). Between 5-15 % of patients are unfit to receive PTs and approximately 3 % are treated off-label with thrombopoietin receptor agonists., Conclusions: this study provides a snapshot of the current situation in Spain, highlighting that the current management is poorly standardized and suboptimal in some cases. The results suggest the benefit of developing a consensus document to address some of these shortcomings and to advance in the search for alternatives to PTs.

We present the case of a 76-year-old male who underwent arthrodesis due to a C7 fracture, which was complicated by an esophageal perforation and required primary surgical closure.

Most extrahepatic cholangiocarcinomas are unresectable at the time of diagnosis and even in case of a resectable cancer, surgery is not an option for the elderly or patients with comorbidities (1). Current treatment alternatives in these scenarios are very limited. Biliary stenting with self-expanding metal stents (SEMS) is the mainstay palliative treatment for biliary obstruction (2). However, emerging experience with endoscopic RF ablation (RFA) in this setting has been reported in the literature.

Giant inflammatory pseudopolyps (> 15 mm) are an uncommon complication of inflammatory bowel disease (IBD) and a differential diagnosis with adenomas and carcinomas is challenging. Although usually asymptomatic, they may result in intestinal obstruction or intussusception due to their size. The standard management involves lesion biopsies and endoscopic excision for selected cases; surgery is usually reserved for size-associated complications or an uncertain pathology. We report the case of a 43-year-old female patient with Crohn's disease (CD) in clinical remission, with no specific treatment at the time. A giant pseudopolyp of 40-mm was found during a screening colonoscopy. Therapy was initiated with infliximab and azathioprine in an attempt to reduce the size of the polyp and allow an endoscopic resection. Additional colonoscopies were performed following induction doses at weeks 0, 2, and 6, which revealed a reduced lesion size. Mucosal resection was attempted but failed due to severe fibrosis, which prevented base injections from lifting up the polyp. However, a follow-up colonoscopy three months later showed that the lesion had completely disappeared. The evidence in the literature regarding giant pseudopolyp management is scarce, but reports indicate that they rarely disappear with medical therapy alone and usually require surgery or endoscopic resection.

Background and Objectives: endoscopic septotomy of the cricopharyngeal muscle (ESCM) is a technique used for the treatment of Zenker's diverticulum (ZD). The experience with computerized vascular sealing systems (LigaSure® type) is limited. The objective of this study was to evaluate the efficacy and safety of ESCM using LigaSure®., Methods: this was a long-term prospective study of 18 patients with ZD, who were referred to our hospital due to ESCM between 2010 and 2016. The severity of the symptoms was determined using the Dakkak-Bennett validated scale for dysphagia and the rest with numerical scales. The rates of relapse and retreatment were evaluated., Results: ESCM with LigaSure® was performed in 17 cases, one case was excluded due to technical difficulties. The median age was 72 years and regurgitation, dysphagia and respiratory symptoms were found in 100%, 89% and 56% of cases, respectively. The median size of the diverticulum was 28 mm (20-60 mm). The median time of the procedure was 35 minutes (25-45 minutes). There were four complications, two hemorrhages and two perforations. The median follow-up was 13 months (range: 12-82 months). Clinical improvements were observed for all symptoms and were maintained 12 months after treatment (p < 0.05). There was no relapse during follow-up in 13 patients. A complete section was not achieved and clinical relapse occurred after a median time of seven months that required retreatment in the remaining patients., Conclusions: ESCM with LigaSure® may be a safe and effective technique in long-term follow-up situations, with low rates of relapse.

Digestive endoscopy is the most effective tool available for the diagnosis of multiple gastrointestinal (GI) tract conditions, and it represents a key aspect in the training of gastroenterology residents according to the Spanish MIR (médico interno residente) program. The Sociedad Española de Endoscopia Digestiva (SEED), aware of all the technical advances that have emerged during the past few years, deems it necessary to define a program of the skills specialists-in-training in gastroenterology should acquire during their residency. This paper describes the goals of endoscopy training, the techniques that should be mastered, and the diagnostic and therapeutic skills this specialty requires. Finally, a model is suggested for the assessment of competence.

Introduction: primary biliary cholangitis (PBC) is a rare disease with limited data regarding its epidemiology and standard clinical management in Spain., Objective: to gain insight into the epidemiology, patient flow, diagnosis, follow-up and treatment of PBC in Spain., Methods: a review of the literature and Delphi study involving 28 specialists in two rounds of consultations and an in-person results validation workshop., Results: there are approximately 9,400 patients with PBC in Spain, with an annual incidence of 0.51-3.86 cases/100,000 population. Albeit, a high error margin may be presumed due to the scarcity of relevant studies on this subject. Several months may elapse from suspicion to a confirmed diagnosis, usually by a gastroenterologist or hepatologist. The role of the liver biopsy for diagnosis and follow-up is heterogeneous. Overall, 95% of patients are treated with ursodeoxycholic acid (UDCA) and response is primarily monitored using the Barcelona criteria. Follow-up is performed every six months, with a heterogeneous use of the various available techniques. No recommendations or second-line commercial drugs are available in the case of no response, inadequate response or intolerance to UDCA., Conclusions: while epidemiology may be estimated based on expert opinions, national registries are needed to provide accurate, up-to-date information on epidemiological parameters, disease stage and response to treatment in patients with PBC. Furthermore, novel therapies are required for selected patient groups.

This document summarizes the contents of the Clinical Guidelines for the Endoscopic Mucosal Resection of Non-Pedunculated Colorectal Lesions that was developed by the working group of the Spanish Society of Digestive Endoscopy (GSEED of Endoscopic Resection). This document presents recommendations for the endoscopic management of superficial colorectal neoplastic lesions.

We report the case of a 35-year-old homosexual man with previous history of HIV, with primary chacre in the rectum. We believe this paper is significant because the diagnosis of rectal ulcer is more common in recent years, however rectal syphilis is a poorly recognized entity, especially with primary chancre formation.

Xanthogranulomatous pancreatitis (XGP) is an extremely rare entity. In most cases, XGP is preoperatively misdiagnosed as a pancreatic neoplasm. The pathogenesis is not well established. To our knowledge, only 15 cases have been reported in the English language literature. Surgical resection was performed before the histologic diagnosis in all published cases. We report the first case of XGP described in Spain, diagnosed by CT-guided biopsy, without requiring surgical resection, in a patient initially diagnosed as having a pancreatic neoplasm.

Due to the rising prevalence of coronary heart disease, endoscopists are more frequently performing a polypectomy in patients on antiplatelet therapy (APT) and dual antiplatelet therapy (DATP). Despite the availability of several guidelines with regard to the management of antiplatelet drugs during the periprocedure period, there is still variability in the current clinical practice. This may be influenced by the low quality of the evidence supporting recommendations, because most of the studies dealing with APT and polypectomy are observational and retrospective, and include mainly small (< 10 mm) polyps. However, some recommendations can still be made. An estimation of the bleeding and thrombotic risk of the patient should be made in advance. In the case of DAPT the procedure should be postponed, at least until clopidogrel can be safely withheld. If possible, non-aspirin antiplatelet drugs should be withheld 5-7 days before the procedure. Polyp size is the main factor related with post-polypectomy bleeding and it is the factor that should drive clinical decisions regarding the resection method and the use of endoscopic prophylactic measures. Non-aspirin antiplatelet agents can be reintroduced 24-48 hours after the procedure. In conclusion, there is little data with regard to the management of DAPT in patients with a scheduled polypectomy. Large randomized controlled trials are needed to support clinical recommendations.

Olmesartan is one of the various angiotensin II antagonists currently used for the management of high blood pressure. A sprue-like enteropathy was first described in 2012 in association with this antihypertensive drug. An observational, descriptive study was carried out on a series of 12 patients who met the clinical, histopathological, and outcome criteria for olmesartan-related sprue-like enteropathy from May 2013 to December 2015. All patients had watery diarrhea, weight loss, and negative celiac serology. They all were admitted with severe illness including dehydration with prerenal kidney failure, metabolic acidosis, water-electrolyte imbalance, and malnutrition parameters. Most common laboratory abnormalities included anemia and hypoalbuminemia. Duodenal biopsy histology revealed villous atrophy in all 12 patients. They all responded well to drug discontinuation, and 100% of individuals with follow-up biopsy showed histological recovery. Olmesartan should therefore be considered a potential cause of severe diarrhea, particularly in patients with duodenal villous atrophy and negative celiac serology.

Ischemic colitis (IC) is an uncommon adverse event associated with antipsychotic agents, more commonly found with phenothiazine drugs and atypical neuroleptics such as clozapine. The risk of developing ischemic colitis increases when anticholinergic drugs are associated. We report the case of a 38-year-old woman with a history of schizoaffective disorder who had been on chronic quetiapine for 3 years, and presented to the ER because of diarrhea for 5 days. Four months previously, olanzapine had been added to her psychiatric drug regimen. Physical examination revealed abdominal distension with abdominal tympanic sounds and tenderness. Emergency laboratory tests were notable for increased acute phase reagents. Tomography revealed a concentric thickening of the colonic wall in the transverse, descending and sigmoid segments, with no signs of intestinal perforation. Colonoscopy demonstrated severe mucosal involvement from the sigmoid to the hepatic flexure, with ulcerations and fibrinoid exudate. Biopsies confirmed the diagnosis of ischemic colitis. The only relevant finding in her history was the newly added drug to her baseline regimen. An adverse effect was suspected because of its anticholinergic action at the intestinal level, and the drug was withdrawn. After 6 months of follow-up clinical, laboratory and endoscopic recovery was achieved. Therefore, antipsychotic medication should be considered as a potential cause of ischemic colitis, particularly atypical antipsychotics such as clozapine and olanzapine; despite being uncommon, this adverse event may result in high morbidity and mortality.

CE soon became an effective tool in the pediatric age, where the invasiveness of conventional endoscopy always represented a real handicap. In this issue of our Journal an interesting consensus paper is reported under the leadership of F. Argüelles where a group of national CE experts shed light on its use in this population.