Diagnosis and management of Abernethy syndrome.

Abernethy syndrome (AS or extrahepatic portosystemic shunt) is an uncommon congenital malformation consisting of agenesis or hypoplasia of the portal vein (PV) in such a way that splanchnic venous blood drains directly into the systemic circulation through aberrant communications, resulting in a portosystemic shunt that bypasses the liver AS is an underdiagnosed condition with unknown incidence and complication rate given that symptoms are usually absent. AS identification is increasingly common because of improved imaging techniques, hence prognostic implications and clinical management need be understood. This editorial reviews the natural history of AS and its diagnostic-therapeutic implications, illustrating the process with a series of cases from our institution.

Endoscopic findings unrelated to portal hypertension in patients with liver cirrhosis undergoing a varicose vein screening programme. / Hallazgos endoscópicos no relacionados con hipertensión portal en pacientes con cirrosis hepática sometidos a un programa de cribado de varices.

AIM: To determine the prevalence of endoscopic lesions unrelated with portal hypertension in patients with cirrhosis. PATIENTS AND METHODS: Cross-sectional study including a consecutive cohort of patients with liver cirrhosis enrolled in a screening program of oesophageal varices who underwent an upper gastrointestinal endoscopy from November, 2013, to November, 2018. Clinical predictors of endoscopic lesions unrelated to portal hypertension were analyzed by univariate and multivariate logistic regression. RESULTS: A total of 379 patients were included. The most frequent aetiology of liver disease was alcohol consumption (60.4%). The prevalence of endoscopic lesions unrelated with portal hypertension was 39.6% (n=150). Among 96 patients with peptic lesions, urease was obtained in 56.2% of patients (positive in 44.4% of them). The prevalence of endoscopic lesions unrelated to portal hypertension was not associated with age, gender, liver function or ultrasound findings of portal hypertension. The prevalence of endoscopic lesions unrelated to portal hypertension was not associated with age, gender, liver function or ultrasound findings of portal hypertension. Smokers had a trend to increased prevalence of endoscopic lesions unrelated to portal hypertension (43.2% vs. 34.6%; p=0.09), particularly peptic ulcer (6.4% vs. 0.6%; p=0.05) and peptic duodenitis (17.3% vs. 6.3%; p=0.002). Active smoking was the only independent predictor of peptic ulcer or duodenitis (OR=2.56; p=0.017). CONCLUSION: Active smoking is a risk factor for endoscopic lesions unrelated to portal hypertension. This finding should be further investigated to reassess endoscopic screening programs in cirrhotic smokers.

Malignancy of intraductal papillary neoplasm of the bile duct.

Intraductal papillary neoplasm of the bile duct (IPNB) is an uncommon disease which was first included in the World Health Organization classification of neoplasms in 2010. A 64-year-old female was admitted to the hospital because of a hepatic lesion incidentally diagnosed during acute cholangitis. Abdominal computed tomography (CT) and magnetic resonance imaging (MRI) showed a well delimited 70 mm mass, with a predominant cystic component and hyperenhancement of papillary internal branching, consistent with a hydatid cyst. However, malignancy could not be excluded. The patient rapidly developed an acute abdomen syndrome, thus precluding a liver biopsy. A new urgent CT was performed to rule out a complication of the cystic lesion. A left hepatectomy was performed and the anatomopathological study confirmed the diagnosis of IPNB with a focus of cholangiocarcinoma therein. During follow up, the patient developed peritoneal carcinomatosis, received palliative chemotherapy and finally died.

Impact of MELD Allocation System on Waiting List and Early Post-Liver Transplant Mortality.

BACKGROUND AND AIMS: MELD allocation system has changed the clinical consequences on waiting list (WL) for LT, but its impact on mortality has been seldom studied. We aimed to assess the ability of MELD and other prognostic scores to predict mortality after LT. METHODS: 301 consecutive patients enlisted for LT were included, and prioritized within WL by using the MELD-score according to: hepatic insufficiency (HI), refractory ascites (RA) and hepatocellular carcinoma (HCC). The analysis was performed to predict early mortality after LT (8 weeks). RESULTS: Patients were enlisted as HI (44.9%), RA (19.3%) and HCC (35.9%). The major aetiologies of liver disease were HCV (45.5%). Ninety-four patients (31.3%) were excluded from WL, with no differences among the three groups (p = 0.23). The remaining 207 patients (68.7%) underwent LT, being HI the most frequent indication (42.5%). HI patients had the shortest length within WL (113.6 days vs 215.8 and 308.9 respectively; p<0.001), but the highest early post-LT mortality rates (18.2% vs 6.8% and 6.7% respectively; p<0.001). The independent predictors of early post-LT mortality in the HI group were higher bilirubin (OR = 1.08; p = 0.038), increased iMELD (OR = 1.06; p = 0.046) and non-alcoholic cirrhosis (OR = 4.13; p = 0.017). Among the prognostic scores the iMELD had the best predictive accuracy (AUC = 0.66), which was strengthened in non-alcoholic cirrhosis (AUC = 0.77). CONCLUSION: Patients enlisted due to HI had the highest early post-LT mortality rates despite of the shortest length within WL. The iMELD had the best accuracy to predict early post-LT mortality in patients with HI, and thus it may benefit the WL management.

Role of serum cytokine profile in ulcerative colitis assessment.

BACKGROUND: Several cytokines are overexpressed in the colonic mucosa of patients with ulcerative colitis (UC). The measurement of these parameters in plasma could be useful in diagnosis and disease assessment. METHODS: In all, 67 UC patients and 21 healthy controls were enrolled. At inclusion, clinical, endoscopic, and histological disease activity were assessed using the Ulcerative Colitis Activity Index (UCAI) and the Baron and Geboes scales, respectively. Serum cytokine concentrations were analyzed with a multiplex system (Bio-Plex pro, Bio-Rad) measuring interleukin (IL)-1-ß, IL-2, IL-6, IL-8, IL-10, IL-13, IL-17, interferon-gamma (IFN-γ), and tumor necrosis factor alpha (TNF-α). Multiple logistic regression was used to design a serum cytokines profile. RESULTS: In the UC group the disease activity was moderate to severe based on clinical evaluation in 35 patients (52.2%), by endoscopic appearance in 45 (67.2%), and in 53 patients (81.6%) using histology. With respect to controls, the multivariate analysis identified that UC patients had higher IL-8 (odds ratio [OR] = 1.37; P = 0.002) and IL-10 concentrations (OR = 3.88; P = 0.012) with lower levels of IFN-γ (OR = 0.95; P = 0.002). The model had an accuracy of 77.3%, which increased to 94.6% when only newly diagnosed patients were considered. Patients with moderate to severe disease according to their clinical score showed a higher concentration of IL-8 (OR = 1.16; P = 0.012) and IL-10 (OR = 1.76; P = 0.039) with lower levels of IL-17 (OR = 0.97; P = 0.021). The IL-8 serum concentration was also related to endoscopic and histological severity (OR = 1.10; P = 0.026 and OR = 1.33, P = 0.017, respectively). CONCLUSIONS: A serum cytokine profile may be an auxiliary tool for the diagnosis and severity assessment of UC. IL-8 seems to be a reliable biomarker, closely related to disease activity.

Radio induced cancer risk during ERCP. Is it a real clinical problem?

BACKGROUND: in recent years many factors have been shown to influence dose received by the patient during ERCP. Therefore it is necessary to update radio induced cancer risk. OBJECTIVES: to calculate lifetime attributable risk of cancer during ERCP. To compare the risk with the most common X-ray examinations. DESIGN: descriptive study with 393 consecutive ERCP performed at one center. Equipment used was Philips BV pulsera. In each exploration demographic and anthropometric variables of the patient were collected. Dosimetric quantities were calculated from exposure parameters. Effective dose was estimated using specific conversion factors. Organ doses and radio induced cancer incidence was estimated. RESULTS: dose area product was 0.82 mGym2(IQR 0.4-1.5) with an average fluoroscopy time of 2 minutes and 45 seconds. Entrance surface dose was 30.7 mGy (IQR 15-60.8) and effective dose was 0.44 mSv (IQR 0.2-0.9). Multivariate analysis identified that difficult papillary cannulation (ß 0.4; p = 0.009), patient age (ß-0.01; p = 0.001) and therapeutic applied (ß= 0.89; p < 0.001) influenced dose-area product. The ERCP dose would be equivalent to the radiation received by twenty chest radiographs and would be about fourteen times smaller than a barium enema or twenty times less than that received during an abdominal CT. Life-time attributable risk of cancer incidence was 4.08 and 16.81 per million procedures in diagnostic and therapeutic ERCP respectively. CONCLUSIONS: from the radiological point of view, ERCP is a safe technique that uses low exposure levels compared to other explorations commonly used in medicine. It implies a reasonably low risk of radio induced cancer.

[Fulminant ischemic gastropathy: the final stage of a difficult-to-diagnose disease]. / Isquemia gástrica fulminante: el estadio final de una enfermedad poco expresiva.

Ischemic gastropathy is highly infrequent in daily medical practice. In the last few years, the number of reported cases has increased. Although the guiding symptom is usually abdominal pain, the clinical spectrum of the disease is highly variable. Early diagnosis and treatment are essential to change the natural history of the disease. We present the case of a 75-year-old man with chronic abdominal pain who developed a fulminant form of necrotizing gastric ischemia and died within less than 24h.