Incidence, predictive factors and clinical significance of development of portal vein thrombosis in cirrhosis: A prospective study.

BACKGROUND AND AIMS: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. There are few prospective studies validating risk factors for development of PVT. We analysed the incidence, factors associated with PVT development and its influence on cirrhosis decompensations and orthotopic liver transplant (OLT)-free survival. METHODS: In this prospective observational study between January 2014 and March 2019, 445 consecutive patients with chronic liver disease were screened and finally 241 with cirrhosis included. Factors associated with PVT development and its influence on cirrhosis decompensations and OLT-free survival by time dependent covariate coding were analysed. RESULTS: Majority of patients belonged to Child-Pugh class A 184 (76.3%) and the average MELD score was 10 ± 5. Previous cirrhosis decompensations occurred in 125 (52.1%), 63 (26.1%) were on NSBB and 59 (27.2%) had undergone banding for bleeding prophylaxis. Median follow-up was 29 (1-58) months. Cumulative incidence of PVT was 3.7% and 7.6% at 1 and 3 years. Previous decompensation of cirrhosis and low platelet counts but not NSBB independently predicted the development of PVT. During follow-up, 82/236 (34.7%) patients developed cirrhosis decompensations. OLT-free survival was 100% and 82.8% at 3 years, with and without PVT respectively. MELD score, but not PVT, independently predicted cirrhosis decompensations (HR 1.14; 95%CI:1.09-1.19) and OLT-free survival (HR 1.16;95%CI:1.11-1.21). CONCLUSION: Previous decompensations of cirrhosis and thrombocytopenia predict PVT development in cirrhosis suggesting a pathophysiologic role for severity of portal hypertension. PVT development did not independently predict cirrhosis decompensations or lower OLT-free survival.

Anticoagulation in Cirrhosis and Portal Vein Thrombosis Is Safe and Improves Prognosis in Advanced Cirrhosis.

BACKGROUND: The role of portal vein thrombosis (PVT) in the natural history of cirrhosis is controversial. AIMS: We analyzed the safety and effect of anticoagulant therapy (AT) on PVT recanalization and orthotopic liver transplant (OLT)-free survival. METHODS: Eighty consecutive patients from a prospective registry of cirrhosis and non-tumoral PVT at a tertiary center were analyzed. AT effect on PVT recanalization and OLT-free survival was determined by time-dependent Cox regression analysis. RESULTS: Average MELD score was 15 ± 7. Portal hypertension-related complications at PVT diagnosis were present in 65 (81.3%) patients. Isolated portal vein trunk/branch thrombosis was present in 53 (66.3%) patients. AT was started in 37 patients. AT was stopped in 17 (45.9%) patients, in 4 (10.8%) due to bleeding events. No variceal bleeding occurred while on AT. Anticoagulation was restarted in 6/17 (35.2%) patients due to rethrombosis. In 67 patients with adequate follow-up imaging, AT significantly increased the rate of PVT recanalization compared with those who did not receive anticoagulation [51.4% (18/35) vs 6/32 (18.8%), p = 0.005]. OLT-free survival after a median follow-up of 25 (1-146) months was 32 (40%). Although there was no significant effect of AT on overall OLT-free survival, OLT-free survival was higher among patients with MELD ≥ 15 receiving AT compared to those who did not (p = 0.011). Baseline MELD at PVT detection independently predicted PVT recanalization (HR 1.11, 95% CI 1.01-1.21, p = 0.027) and mortality/OLT (HR 1.12, 95% CI 1.05-1.19, p < 0.001). CONCLUSIONS: Although AT did not improve overall OLT-free survival, it was associated with higher survival in advanced cirrhosis. Anticoagulation increased PVT recanalization and should be maintained after PVT recanalization to avoid rethrombosis.

Identifying palliative care needs in a Portuguese liver unit.

BACKGROUND & AIMS: Chronic liver disease is a major worldwide cause of morbidity and mortality. Palliative care policies are not clearly established in chronic liver disease. The NECPAL CCOMS-ICO© (NECesidades PALiativas/Palliative Needs) is a tool to identify palliative care needs, including a section for liver disease. AIM: The aim of this study was to identify palliative care needs in liver patients hospitalised in a tertiary referral Liver Unit. METHODS: Single-centre prospective observational study. One hundred and twenty patients with cirrhosis were included and NECPAL questionnaire was applied to all patients in a 7-month period. RESULTS: 84.2% of patients were considered as requiring palliative intervention; however, clinicians identified those needs only in 65.8% of the cases and caregivers in 6.7% of the cases; less than 8% of the patients were referred for palliative care consultation. An excessive use of healthcare resources (positive answer to question 3) was strongly associated with a positive need for palliative care (positive NECPAL): OR 7.305, CI 95% 2.54-20.995, P < .001). An excessive use of healthcare facilities has a sensitivity of 84.2% and a specificity of 42.1% for prediction of a positive NECPAL result (AUC 0.710, 95% CI 0.570-0.850, P = .004). CONCLUSIONS: The NECPAL CCOMS-ICO© represents a feasible and easy-to-use tool to identify palliative care needs in patients with chronic liver disease.

Lipids, glucose and iron metabolic alterations in chronic hepatitis C after viral eradication - comparison of the new direct-acting antiviral agents with the old regimens.

BACKGROUND: Hepatitis C virus (HCV) is a unique virus which interacts with cholesterol, iron and insulin metabolism. There is limited data on the effects of direct-acting antiviral agents (DAAs) on metabolic profiles. We aimed at evaluating the behavior of metabolic risk factors of chronically HCV-infected patients after sustained virologic response (SVR), comparing the outcomes with the new DAAs versus the old treatment regimen Peg-interferon ± ribavirin. METHODS: A total of 178 patients who achieved SVR and completed one year of follow-up were prospectively included in this study: group 1 with 105 patients treated with DAAs and group 2 with 73 patients treated with old regimens. Outcomes included lipid, glucose and iron metabolism variation after SVR. RESULTS: There was a significant increase in total cholesterol in both groups (group 1: p < .001, 95% CI: 0.41-0.78; group 2: p < .001, 95% CI: 0.24-0.69). Triglyceride levels significantly decreased (p = .015, 95% CI: -0.33-0.04) in group 1 and increased (p = .014, 95% CI: 0.07-0.59) in group 2. LDL levels increased in group 1 (p = .029, 95% CI: 0.05-0.88), but no significant variation was found in group 2. No significant variation in HDL, fast glucose and iron was seen in both groups. There was a significant increase of HOMA (p = .002, 95% CI: 0.17592-0.72317) only in group 2. Ferritin serum levels significantly decreased (p < .001, 95% CI:-138.3-74.4) in group 1 but no significant variation was found in group 2. CONCLUSION: Patients who have achieved SVR may have increased risk of cardiovascular outcomes due to development of hyperlipidemia and insulin resistance.

A rare cause of acute liver failure- a case report.

BACKGROUND: Acute liver failure (ALF) induced by diffuse metastatic disease has rarely been reported. CASE PRESENTATION: We present a 51-years-old woman with relevant clinical history for breast cancer. The patient was admitted in the emergency department with jaundice, dark urine and pale stools. She was on the 10th day of hormonotherapy for recurrence of breast cancer, diagnosed 7 years previously. Usual causes of acute liver failure were excluded, all drugs were stopped and the imaging studies performed were positive only for steatosis. Nonetheless, ALF progressed and the patient died 4 days later. Autopsy demonstrated a massive intrasinusoidal infiltration of the liver by breast cancer cells. CONCLUSION: We highlight a rare cause of ALF. Although uncommon, physicians should be alert for this situation as the diagnosis can be challenging and the imaging studies can remain normal.

An unusual cause of Boerhaave´s syndrome in a young patient.

An 18-year-old male patient with a history of atopy and intermittent dysphagia for solids, presented to the emergency department with sudden onset total dysphagia followed by hematemesis, after ingesting an ibuprofen tablet. Urgent upper gastrointestinal endoscopy revealed a deep laceration just above the tablet impacted in the distal esophagus. Abdominal CT-scan confirmed the suspicion of an esophageal perforation. The impacted tablet was broken up with biopsy forceps, and a covered metallic stent (Hanarostent® 60/100x20/26mm) was placed across the cardia effectively excluding the fistula. Recovery was uneventful and the stent was easily removed 6 weeks later. Follow-up biopsies showed marked mucosal infiltration by eosinophils confirming the diagnosis of eosinophilic esophagitis (EE). The patient was treated with oral budesonide and remains asymptomatic.

Acute cholangitis in an old patient with Crigler-Najjar syndrome type II - a case report.

BACKGROUND: Crigler-Najjar syndrome (CN) is a very rare genetic disorder characterized by an inability to conjugate bilirubin. Contrary to CN type I, patients with CN II exhibit residual capacity to conjugate bilirubin and may present a normal life expectancy. CASE PRESENTATION: We report an unusual late diagnosis of CN type II in an 80-year-old female admitted with severe acute cholangitis. While the patient present typical clinical and radiologic signs of bile duct obstruction and cholangitis, her blood analysis showed severe unconjugated hyperbilirubinemia. Endoscopic retrograde cholangiopancreatography confirmed the diagnosis and allowed therapeutic intervention. The anatomopathologic examination of her gallbladder following cholecystectomy showed signs of chronic cholecystitis. CONCLUSION: The risk of gallstone disease may be increased in patients with CN syndrome. While unusual, we alert to this curious and potential life-threatening presentation.

Ascitic Calprotectin is a Novel and Accurate Marker for Spontaneous Bacterial Peritonitis.

BACKGROUND: Spontaneous bacterial peritonitis (SBP) is a known complication of advanced cirrhosis and presents a high mortality rate. A polymorphonuclear (PMN) cell count >250/µl in the ascitic fluid is the current gold standard for diagnosing SBP. AIM: We evaluated the accuracy of a point-of-care test (POCT) for ascitic calprotectin in diagnosing patients with SBP. METHODS: Eighty-eight patients admitted with decompensation of liver cirrhosis were studied including 41 patients (46.6%) with SBP. Ascitic calprotectin was measured using a quantitative POCT developed by Bühlmann® . RESULTS: Calprotectin levels correlated with PMN cell count and other inflammatory markers and were significantly higher in patients with SBP. An optimal cutoff of calprotectin above 1.57 µg/ml presented high sensitivity (87.8%), specificity (97.9%), and positive (97.3%) and negative (90.2%) predictive values for diagnosing SBP. Using calprotectin selectively in patients with a serum albumin-ascites gradient above 11 g/l further increased the sensitivity and negative predictive values of the test. CONCLUSION: Ascitic calprotectin appears to be a reliable method for diagnosing SBP in patients with liver cirrhosis. It may present an alternative to other conventional diagnostic methods.

Hemangioma of the rectum - How misleading can hematochezia be?

We present the case of an 18-year-old male patient that was referred to our gastrenterology department with history of intermittent painless hematochezia since childhood. During such instances, he was diagnosed with bowel intussusception, eosinophilic gastroenteritis and inflammatory bowel disease at 4, 6 and 8 years old, respectively. He underwent treatment with 5-aminosalicylic acid for two years, without improvement of symptoms. He was then lost to follow-up until our observation. His physical examination was unremarkable except for digital rectal examination which found a nodular compressible mass by the palpating finger. Blood tests revealed a mild iron deficiency anemia. The colonoscopy showed an extended reddish and bluish multinodular submucosal mass in the rectum, suggesting diffuse cavernous hemangioma of the rectum (DHCR). The Magnetic Resonance Imaging, showed diffuse thickening of the entire rectum extending into the distal sigmoid with the mesorectum revealing multiple serpiginous structures, corresponding to abnormal blood vessels. After discussion, we considered to perform a sphincter-sparing procedure, namely pull through transection and coloanal anastomosis. However, intervention was ruled out by the patient because of his fear of anal incontinence and permanent colostomy. We adopted a conservative strategy with clinical surveillance and iron supplementation. At the present, the patient remains with intermittent rectal bleeding, referring poor quality of life due to his ongoing symptoms. This is a rare case of DHCR. Despite of being a benign disease, the management of DHCR requires a sphincter mucosectomy and pull-through coloanal sleeve anastomosis which has become the first-line procedure. The surgical outcomes are non-expectable in 32% with permanent sphincter lesion or with incomplete DHCR removal. As in this case, the surgeons or patients refusal to perform the intervention is common which represents a challenge to the clinical follow-up.

Severe ischemic colitis following olanzapine use - A case report.

Ischemic colitis is the most common subtype of intestinal ischemia usually resulting from vasospasm, vessel occlusion or mesenteric hypoperfusion. Neuroleptics have seldom been linked to ischemic colitis by blocking peripheral anticholinergic and antiserotonergic receptors inducing severe gastrointestinal paresis. We report a young patient with severe ischemic colitis requiring surgery due to necrosis of the bowel. After exclusion of other potential causes, olanzapine was admitted as the cause of ischemia. Clinicians should be aware of how to recognize and treat the potentially life-threatening effects of neuroleptics.

Primary liposarcoma of the sigmoid presenting as colonic intussusception - A case report.

Liposarcomas are malignant soft tissue neoplasms usually located in the retroperitoneum, head, neck and extremities. Although secondary invasion of the gastrointestinal tract by retroperitoneal sarcomas is frequent, primary involvement is uncommon. We report a young patient with Crohn's disease in remission presenting with intussusception of the colon due to a primary liposarcoma of the sigmoid. Emergency resection confirmed the diagnosis. The patient remains in remission after five years. This represents the youngest diagnosis of liposarcoma to date and the first occurring in the sigmoid. We also highlight the concomitant diagnosis of Crohn´s disease.

The use of a segmental endoscopic score may improve the prediction of clinical outcomes in acute severe ulcerative colitis.

BACKGROUND: Acute severe colitis (ASC) remains a challenging complication of ulcerative colitis. The early identification of patients who will not respond to optimal therapy is warranted. Increasing evidence suggests that endoscopy may play a role in predicting important outcomes in acute severe colitis. METHODS: The endoscopic activity of consecutive patients with acute severe colitis was evaluated using the Mayo endoscopic sub-score (Mayo) and the ulcerative colitis endoscopic index of severity (UCEIS). Two segmental indexes were also produced by summing the scores of the rectum and sigmoid (seg-Mayo and seg-UCEIS, respectively). Endpoints included the need for salvage therapy with infliximab or cyclosporine, refractoriness to corticosteroids, and colectomy. RESULTS: Of one hundred and eight patients enrolled in the study, 60 (55.6%) were male; with a median age of 34.5 years (range 15-80). All patients received intravenous steroids. Fifty-nine patients (55.6%) showed an incomplete or absent response to steroids, 35 patients (34.3%) received salvage therapy with infliximab or cyclosporine and 38 patients (33.3%) were colectomized during the index hospitalization or within the first year of follow-up. All scores were able to predict the need for surgery, but only the seg-UCEIS significantly predicted refractoriness to steroids. CONCLUSIONS: There was a strong correlation between endoscopic severity and unfavorable outcomes. The UCEIS outperformed the Mayo endoscopic sub-score in all important outcomes. Segmental scoring further improved the performance of the UCEIS.

The burden of alcoholism in fifteen years of cirrhosis hospital admissions in Portugal.

BACKGROUND & AIMS: Deploying a longitudinal perspective, we observe how cirrhosis caused mortality rates in Portugal are converging with the levels reported in the European Union (15 countries). However, we still lack analysis of the burden of alcoholic cirrhosis in terms of hospital admissions and associated mortality. As Portugal may be considered a paradigmatic case in Europe, our aim was to characterize the evolution of hospital admissions for alcoholic cirrhosis between 1993 and 2008 and draw conclusions for other countries. METHODS: Retrospective analysis of the hepatic cirrhosis admissions in 97 Portuguese state hospitals was carried out based on the National Registry. RESULTS: We report a convergence in terms of mortality rates resulting from cirrhosis between Portugal and European Union (a differential of 6.7 deaths per 100 000 habitants in 1994 to 0.4 in 2008). We accounted for 81 543 hospital admissions for cirrhosis: 84% for alcoholic cirrhosis and 16% for non-alcoholic cirrhosis. Hospital admissions have increased 29% in men and with no increase in women. In the male, alcoholic cirrhosis patient group aged between 40 and 54, the rise in hospital admissions was more pronounced with an increase of around 45%. These patients underwent longer lengths of stay and reported higher mortality rates and passing away 20 years earlier than the average national expectancy of life. CONCLUSIONS: These data draw attention to the burden of alcohol consumption not only in Portugal but also in other countries and its impacts on hospital systems and on policy making.

A case of esophageal squamous cell carcinoma with positive HPV 11.

HPV (human pappiloma virus) infection is an etiologic risk factor for esophageal carcinoma with several studies supporting its carcinogenic role. The main strains are HPV16 and 18 and the less frequent strains 11, 6, 31 and 36. We report the case of a 58 year old male, smoker, admitted to our hospital for progressive dysphagia. Previous endoscopies and biopsies were compatible with a hyperkeratotic esophagus, negative for dysplasia. Second endoscopy showed a hyperkeratotic, verrucous, esophagus with two circumferential stenosis which were dilated with TTS (Through the Scope) balloon. Biopsies revealed multiple squamous cell abnormalities, negative for dysplasia and positive for HPV 11. Symptoms recurred, and he was submitted to another upper gastrointestinal endoscopy with dilation of the stenosis and esophageal stent placement. Two months later, esophagectomy was performed, and the histopathological analysis revealed a squamous cell carcinoma of the esophagus (T2, N0, M0, G1). The patient died after surgery due to acute respiratory distress syndrome.