Setting Of Portal Hypertension Research Articles

A Unique Presentation of Familial Idiopathic Colonic Varices

ABSTRACT Colonic varices typically occur in the setting of portal hypertension, and patients may present with rectal bleeding or occult anemia. Idiopathic colonic varices occur infrequently in the absence of cirrhosis and can involve the entire colon. We present a case of a 54-year-old Eastern European woman who had undergone diagnostic colonoscopy for newly diagnosed sigmoid adenocarcinoma and was incidentally found to have colonic varices with normal portal pressure gradients. Her 38-year-old daughter was found to have similar varices, raising concerns for hereditary etiology.

Portopulmonary Hypertension: A Review

Portopulmonary hypertension (POPH) is a rare complication of liver disease occurring when pulmonary arterial hypertension develops in the setting of portal hypertension. It increases the morbidity and mortality compared to patients with cirrhosis alone. POPH is classified in Group 1 pulmonary arterial hypertension, which has important implications on treatment. After aggressive treatment and in carefully selected patients, liver transplantation can be performed; this can be curative of not only their liver disease but also of their POPH. Treatment and patient selection for optimum results continues to evolve. This article provides updates on the definition, clinical course, and treatment of patients with POPH. We will also discuss the evolving data in treatment and liver transplantation in POPH.

Comparison of Prognostic Scores for Upper Gastrointestinal Bleeding in the Hepato-Gastro-Enterology Department

Objectives: The goal of this study is to assess the relative value of individual UGIB prognostic scores in predicting patient outcomes. Patients and Methods: This study was a descriptive cross-sectional analysis of data collected before. Included were patients admitted to the Al Tibri Medical college and hospital Malir Karachi Center and Fauji foundation Rawalpindi with upper GI bleeding and treated in the Gastroenterology Division. Analyses in this study compared the predictive power of five different prognostic scores (the Glasgow Coma Scale, modified Glasgow Coma Scale, coagulation risk score, and acute ischemic stroke severity score; together, these scores are known as GBS, mGBS, FRS, CRS, and AIMS65) for the occurrence of death and rebleeding within 42 days. ROC (Receiver Operating Characteristic) curves were used to compare the various scores. Results: A total of 314 individuals were enrolled in the study, with a male-to-female sex ratio of 2.48. In 70.94% of cases, fibroscopy revealed UGIB due to portal hypertension unrelated to peptic ulcer disease. The "FRS" score was the most reliable predictor of mortality or rebleeding for all patients. Compared to other scores, the "FRS" provided the best reliable forecast of whether or not patients would have spots. The "FRS" score was the most reliable one for predicting mortality. Patients deemed to be at low risk (below the threshold value) had a mortality rate of 2.2% according to the "FRS," 9.3% according to the "CRS," 0% according to the "GBS" (p = 0.565), 50% according to the "mGBS," and 11.42% according to the "AIMS65." The predictive value of UGIB scores was higher for incidental portal hypertension. Conclusion: Upper gastrointestinal hemorrhage cases can be accurately predicted using the "FRS" and "CRS" scores. But in the setting of portal hypertension, these scores did badly. UGIB Keywords: Prognostic Scores, Upper Gastrointestinal Bleeding, Rockall, Glasgow-Blatchford

Portopulmonary Hypertension: A Review of the Current Literature

Portopulmonary hypertension is defined as the development of pulmonary arterial hypertension in the setting of portal hypertension with or without liver cirrhosis. Portal hypertension-associated haemodynamic changes, including hyperdynamic state, portosystemic shunts and splanchnic vasodilation, induce significant alterations in pulmonary vascular bed and play a pivotal role in the pathogenesis of the disease. If left untreated, portopulmonary hypertension results in progressive right heart failure, with a poor prognosis. Although Doppler echocardiography is the best initial screening tool for symptomatic patients and liver transplantation candidates, right heart catheterisation remains the gold standard for the diagnosis of the disease. Severe portopulmonary hypertension exerts a prohibitive risk to liver transplantation by conferring an elevated perioperative mortality risk. It is important for haemodynamic parameters to correspond with non-severe portopulmonary hypertension before patients can proceed with the liver transplantation. Small uncontrolled studies and a recent randomised controlled trial have reported promising results with vasodilatory therapies in clinical and haemodynamic improvement of patients, allowing a proportion of patients to undergo liver transplantation. In this review, the epidemiology, pathogenesis, diagnostic approach and management of portopulmonary hypertension are discussed. We also highlight fields of ongoing investigation pertinent to risk stratification and optimal patient selection to maximise long-term benefit from currently available treatments.

Echo Rounds: Intraoperative Doppler Assessment of Transjugular Intrahepatic Shunt Flow Using Transesophageal Echocardiography.

The use of intraoperative transesophageal echocardiography (TEE) to assess venous congestion of the liver due to right heart dysfunction is well established, predominately through the Doppler interrogation of the hepatic and portal venous waveforms. Transjugular intrahepatic portosystemic shunts (TIPSs) are artificial intraparenchymal tracts through the liver that are placed to decompress the portal circulation in the setting of portal hypertension, most commonly due to cirrhosis. Herein, we describe the Doppler interrogation of a TIPS using intraoperative TEE, and how changes in the transmitted portal venous waveform were used to assess the severity of tricuspid regurgitation and inform management. (A&A Practice. 2022;16:e01607.).

S1045 EUS-Guided Primary, Secondary Prophylaxis Treatment of Gastric Varices: A Systematic Review With Meta-Analysis

Introduction: Gastric varices (GV) increase the risk of gastrointestinal bleeding and carry a significant mortality risk, especially in the setting of portal hypertension. A major treatment modality for GV includes endoscopic ultrasound (EUS) guided glue and/ or coil injection. In this meta-analysis study, we aim to address the efficacy of EUS guided glue and/ or coil injection in the treatment of GV in primary and secondary prophylaxis. Methods: We conducted a comprehensive search of several databases including Medline, Scopus, Embase to identify studies from 2000-2019 that evaluated EUS guided therapy in the treatment of GV for primary and/or secondary prophylaxis. Our primary goals were to estimate the pooled rates of treatment efficacy, GV obliteration, GV recurrence, early re-bleeding, and late re-bleeding with EUS guided therapy of GV in primary and secondary prophylaxis. Calculation was done by standard meta-analysis methodology using the random-effects model and heterogeneity was assessed using the I2% statistics. Results: A total of 13 studies were included in the final analysis. The EUS guided treatment efficacy of primary prophylaxis was 95% (95% CI [85.7-98.4%]) and secondary prophylaxis was 94.3% [83.6-98.1]. GV obliteration was 68.3% [43.4-85.8] for primary prophylaxis and 65.8% [38.8-85.4] for secondary prophylaxis. GV recurrence was 10% [2.5-32.8] in primary prophylaxis and 4.3% [1.7-10.5] in secondary prophylaxis. Early re-bleeding was seen in 6.7% [1.7-23.1] of primary prophylaxis cases and 5.5% [2.3-12.6] of secondary prophylaxis. Late re-bleeding was seen in 6% [2.3-14.9] of primary prophylaxis cases and 12.6% [9.3-16.8] of secondary prophylaxis cases. We conducted sub-group analysis with respect to studies published as abstracts and full manuscripts, and by sample-size. The results summarized in Table 1. Conclusion: Our meta-analysis study illustrates that EUS-guided therapy of GV may demonstrate excellent short and long-term clinical outcomes in both primary and secondary prophylaxis. This was shown by high EUS guided treatment efficacy of 95% and 94.3% in primary and secondary prophylaxis respectively. In addition, a low GV recurrence of 4.5% in secondary prophylaxis is a key positive finding; however, the late re-bleeding rate of 12.6% in secondary prophylaxis needs further evaluation and future study. Nevertheless, our study highlights that early EUS-guided therapy should be considered in the treatment of GV regardless of primary or secondary prophylaxis.Table 1.: Summary of pooled results

Outcomes of Liver Transplantation in Treated Portopulmonary Hypertension Patients With a Mean Pulmonary Arterial Pressure ≥35 mm Hg.

Background.Portopulmonary hypertension (POPH), pulmonary arterial hypertension (PAH) that develops in the setting of portal hypertension, affects 5%–6% of patients with liver disease and is associated with significant morbidity and mortality. A mean pulmonary arterial pressure (mPAP) threshold of 35 mm Hg is used to stratify perioperative risk and liver transplant eligibility in treated POPH patients but does not take into account the specific factors that contribute to the pressure elevation.Methods.In this case series, we describe the characteristics and posttransplant outcomes of patients with treated POPH and an mPAP ≥35 mm Hg and pulmonary vascular resistance (PVR) <250 dynes-s-cm−5 at or just before liver transplantation (LT). We also describe the effect of PAH therapy on pulmonary hemodynamics in patients with POPH.Results.Sixteen patients were included. All patients were on PAH therapy at the time of LT. PAH therapy resulted in a decrease of mPAP (median 18.4%; interquartile range [IQR] 8.9%–27.0%) with a reduction in PVR (median 50.5%; IQR, 45.4%–70.7%), and an increase in both cardiac output (CO) (median 28.1%; IQR 5.7%–63.8%) and PAWP (median 50.0%; IQR 16.7%–108.3%) before LT. One year posttransplant survival was 69% (11/16); however, only 1 death was attributed to POPH. At 1-year posttransplant, 63.6% (7/11) of patients were weaned off all PAH therapy with clinical and echocardiographic resolution of POPH.Conclusions.In treated POPH patients with an mPAP ≥35 mm Hg and PVR < 250 dynes-s-cm−5 before LT, 1-year posttransplant survival was 69% and the majority of patients were able to discontinue PAH therapy.

S2111 Recurrent Bleeding From Peristomal Varices: A Management Dilemma

INTRODUCTION: Gastroesophageal varices are seen in the setting of portal hypertension, however, ectopic varices may arise elsewhere such as around ostomy site. These account for more severe complications as there is no standardized management for them. Peristomal varices can lead to massive bleeding due to an increase in the portal pressure gradient and resulting mortality in as high as 40% cases (higher in cases with rebleeding). CASE DESCRIPTION/METHODS: A 65 year old male with a history of rectal adenocarcinoma status post colostomy four years prior to admission, peristomal varices status post suture ligation and sclerotherapy that re-bled and underwent coiling embolization was admitted to the hospital for acute severe abdominal pain, fatigue and heavy bleeding into his ostomy pouch for 2 days. Lab work showed a hemoglobin of 6.0 g/dL (9.5 g/dL one month prior). Physical exam demonstrated bright red blood from the stomal edge and clots in the ostomy pouch. A CT abdomen showed extensive varices in the perigastric region. Colonoscopy showed bleeding from stomal varices in the 5 o'clock position. Patient underwent percutaneous embolization of the superficial and deep varices around the edge of stoma which resolved the bleed. However, three months later, patient presented to the hospital with rebleeding, progressive metastasis of the rectal adenocarcinoma and worsening portal hypertension failing medical management. Due to worsening prognosis and recurrent bleeding, patient opted for hospice care. DISCUSSION: Ectopic varices account for 4–5% of variceal bleeds and the mortality risk for an initial episode is as high as 40% as it is often difficult to differentiate ectopic variceal bleeding from other causes of lower gastroesophageal bleeds causing a delay in management. Our patient had recurrent stomal variceal bleeds and had become transfusion dependent. Moreover, his initial non-invasive management ended in recurrent bleeding which occurs in 75% of cases. All patients do achieve full resolution even after coiling embolization as new variceal formation occurs. Treatment options can range from conservative management to invasive procedures. Transhepatic coil embolization or transjugular transhepatic approach to cease the bleeding are the proposed treatment modalities. However, this case presents a management dilemma for such patients due to differing management modalities and exemplifies the need for the establishment of a standard protocol.Figure 1.: Colonoscopy displaying area of bleed.Figure 2.: status post coil embolization.

The Myths and Realities of Portopulmonary Hypertension.

Portopulmonary hypertension (POPH), defined as an increased mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) in the setting of portal hypertension, is a commonly misunderstood pulmonary vascular complication of liver disease. Recent literature has expanded our knowledge regarding disease pathogenesis, the role of pulmonary arterial hypertension (PAH) targeted therapy and liver transplantation (LT) in management, and long-term outcomes. However, many myths and misconceptions regarding POPH prognosis and treatment remain. The aim of this review is to examine the prevailing myths and current realities regarding POPH.

Role of interventional radiology in intractable bleeding rectal varices.

In the setting of portal hypertension, intractable bleeding from anorectal varices is a rare occurrence. In this review, clinical presentation and management of this dilemma are presented in a case-based fashion. Bleeding may occur in the absence of prior history of cirrhosis or gastroesophageal varices and measurement of hepatic venous pressure gradient and liver biopsy could help to establish the diagnosis. Successful treatment outcome necessitates tailoring treatment to the patient's anatomy and imaging findings. A multidisciplinary algorithmic approach is also proposed to aid clinicians in this regard.

Sex Differences in Portopulmonary Hypertension

Portopulmonary hypertension (POPH), pulmonary arterial hypertension that develops in the setting of portal hypertension, can lead to right-sided heart failure and death. Being female is a known risk factor for POPH, but little is known about the effect of sex on clinical manifestations, hemodynamics, treatment response, and survival. We sought to characterize sex differences in clinical characteristics, pulmonary hemodynamics, treatment response, and survival in patients with POPH. We performed a retrospective cohort study of adult candidates for liver transplant (LT) who had POPH within the Organ Procurement and Transplantation Network database. Females and males were compared. Multivariate regression was performed to assess the association between sex and pulmonary vascular resistance (PVR) and survival. Patients were also stratified by age (50 years) to determine how age modifies the relationship between sex and hemodynamics and survival. We included 190 adults (103 male, 87 female). Compared with men, women had a lower model for end-stage liver disease (MELD) score (12.1± 4.2 vs13.8 ± 4.9; P= .01) and were more likely to have autoimmune liver disease. Women had a higher baseline PVR (610.6 ± 366.6 vs461.0 ± 185.3 dynes-s-cm-5; P< .001) and posttreatment PVR (244.6 ± 119.5 vs202.0 ± 87.7 dynes-s-cm-5; P= .008) and a greater treatment response (ΔPVR) (-359.3 ± 381.9 vs-260.2 ± 177.3 dynes-s-cm-5; P= .03). In multivariate analysis, female sex (or gender) remained associated with a higher baseline PVR (P= .008). Women and men had overall similar survival (P > .05). When patients were stratified by age, being female was independently associated with worse waiting list survival after adjusting for MELD and PVR in younger patients (HR, 6.61; 95%CI, 1.25-35.08; P= .03) but not in older patients. Compared with male candidates, female candidates for LT who had POPH had a higher PVR and lower MELD score and were more likely to have autoimmune liver disease. Women and men had similar overall survival, but female sex (or gender) was associated with worse survival in younger patients.

PORTAL HYPERTENSIVE GASTROPATHY: A COMPREHENSIVE REVIEW

Portal hypertensive gastropathy (PHG) encompasses the gastric mucosal changes occurring in the setting of portal hypertension,both cirrhotic and non-cirrhotic. Its significance lies in causing acute gastrointestinal bleeding and insidious chronic blood loss presenting as iron deficiency anemia. Diagnosis of PHG is straight-forward, made endoscopically often characterized by a mosaic-like pattern resembling ‘snake-skin’, with or without red spots. Treatment of acute GI bleed is hemodynamic stabilization, vasoconstrictor therapy, antibiotic prophylaxis, non-selective beta-blockers. Endoscopic treatment like APC has a small role. In severe cases, TIPS and shunt surgery can be offered. Secondary prophylaxis of PHG bleeding with non-selective b-blockers is recommended.&#x0D; Keywords: Portal hypertension­, Gastrointestinal bleeding, Cirrhosis, Beta-blockers

Hepatopulmonary Syndrome and Portopulmonary Hypertension: Management in Liver Transplantation in the Horizon 2020

Hepatopulmonary syndrome (HPS) is characterized by intrapulmonary microvasculature dilatation that causes intrapulmonary shunting and leads to a gas exchange abnormality in the presence of liver diseases, which is the most common cause of respiratory insufficiency in these patients. HPS doubles the risk of death, and liver transplantation (LT) is the only curative therapeutic option so it should be considered in patients with severe HPS, with excellent survival rates post-LT. However, pretransplant Pao2 <45 mm Hg has been associated with an increase in post-transplant morbidity and mortality, but it does not imply a contraindication for LT. The resolution of HPS usually occurs within 6 months post-LT, but it can take 1 year.Portopulmonary hypertension (PoPH) is defined as pulmonary arterial hypertension (PAH) that develops in the setting of portal hypertension with or without liver disease in the absence of other causes of PAH. The prevalence of PoPH is 5% to 10% among liver transplant (LT) candidates. The impact of LT on PoPH is unpredictable. Therefore, despite conferring a high morbidity and mortality, PoPH itself is not an indication for liver transplantation. It may be considered a contraindication for LT in severe cases.

A Case of Gluconacetobacter liquefaciens Bacteremia Associated with Sugarcane Juice Ingestion

Gluconacetobacter liquefaciens is a Gram-negative bacterium that has previously been described as a plant pathogen. To the best of our knowledge, only one previous case of human infection with Gluconacetobacter species has been described and was associated with severe immunodeficiency. We describe a case of recurrent Gluconacetobacter liquefaciens bacteremia in a patient with advanced liver cirrhosis and propose that gut translocation in the setting of portal hypertension and frequent sugarcane juice ingestion may have been the etiology. This case expands on our knowledge of Gluconacetobacter liquefaciens as a potential emerging human pathogen in certain situations and describes the difficulty encountered in antimicrobial testing and management. J Microbiol Infect Dis 2020; 10(1):62-67.

Efficacy and safety of endoscopic ultrasound-guided therapy versus direct endoscopic glue injection therapy for gastric varices: systematic review and meta-analysis.

Gastric variceal bleeding carries significant mortality in the setting of portal hypertension. Among the endoscopic treatment options, endoscopic ultrasound (EUS)-guided glue and/or coil injection is a novel approach, but its role in the treatment of gastric varices is not established due to a lack of robust data. We conducted a comprehensive search of several databases (inception to June 2019) to identify studies evaluating EUS in the treatment of gastric varices. Our primary goals were to estimate the pooled rates of treatment efficacy, obliteration and recurrence of gastric varices, early and late rebleeding, and adverse events with EUS-guided therapy in gastric varices. We also searched for studies that evaluated direct endoscopic glue (END-glue) injection for treatment of gastric varices, and used the pooled rates as comparators. 23 studies (851 patients) evaluating EUS-guided therapy were included. The pooled treatment efficacy was 93.7 % (95 % confidence interval [CI] 89.5 - 96.3, I 2 = 53.7), gastric varices obliteration was 84.4 % (95 %CI 74.8 - 90.9, I 2 = 77), gastric varices recurrence was 9.1 % (95 %CI 5.2 - 15.7, I 2 = 32), early rebleeding was 7.0 % (95 %CI 4.6 - 10.7, I 2 = 0), and late rebleeding was 11.6 % (95 %CI 8.8 - 15.1, I 2 = 22). The rates were comparable to END-glue therapy (28 studies, 3467 patients) except for obliteration, which was significantly better with EUS-guided therapy. On subgroup analysis, EUS-coil/glue combination showed superior outcomes. EUS-guided therapy demonstrated clinical efficacy for treatment of gastric varices in terms of obliteration, recurrence, and long-term rebleeding, and may be superior to END-glue.

Lower Gastrointestinal Bleeding in Patients With Cirrhosis—Etiology and Outcomes

Lower gastrointestinal bleeding (LGIB) is a common clinical problem, and may be more prevalent among patients with cirrhosis, especially in the setting of portal hypertension and coagulopathy. However, there is extremely little data available on the subject of LGIB in patients with cirrhosis. Therefore, the primary objective of this study was to better understand the etiology and outcomes of cirrhotic patients hospitalized with LGIB. We analyzed 3,735 cirrhotic patients admitted to the Medical University of South Carolina between January 2011 and September 2018, and identified patients admitted with a primary diagnosis of hematochezia or bright red blood per rectum. Thirty patients with cirrhosis and LGIB were included in the cohort. The mean age was 56 ± 13 years, with 30% women. The mean model of end stage liver disease score was 22, and Child-Pugh (CP) scores were C: 41%, B: 33% and A: 26%. The mean Charlson Comorbidity Index was 5.6. Twenty-four (80%) patients had a clinical decompensating event (hepatic encephalopathy, ascites, esophageal varices); the mean hepatic venous pressure gradient was 14.1 mm Hg (n=8). In 33% of patients, LGIB was considered significant bleeding that necessitated blood transfusion. The most common cause of LGIB was hemorrhoids (11 patients, 37%), followed by portal hypertensive enteropathy or colopathy (7 patients, 23%). Hemoglobin levels on admission were lower in patients with CP B/C cirrhosis than in those with CP A (P < 0.001). The length of stay was 9 ± 10 days, and 5 patients died (mortality, 17%). Despite being uncommon, LGIB in cirrhotic patients is associated with a high mortality rate.

904 EUS-Guided Treatment of Gastric Varices: A Systematic Review and Meta-Analysis With Indirect Comparison to Endoscopic Therapy

INTRODUCTION: Gastric variceal (GV) bleeding carries a significant mortality in the setting of portal hypertension. Emergency band ligation and/ or transjugular intrahepatic portosystemic shunt is the current recommended treatment. Other treatment options include direct endoscopic (END) glue injection and endoscopic ultrasound (EUS) guided glue and/ or coil injection. The role of EUS in the treatment of GV is not established and remains an active area of investigation. The aim of this study was to describe the performance of EUS in the treatment of GV. METHODS: We conducted a comprehensive search of several databases (inception to Dec 2018) to identify studies that evaluated END-glue and EUS in the treatment of GV. Our goals were to estimate the pooled rates of treatment efficacy, GV obliteration, GV recurrence, early re-bleeding, late re-bleeding and adverse events with EUS guided therapy in GV. The pooled outcomes of END-glue was used as a comparator group. RESULTS: Total of 35 studies (2777 patients) were included. Mean age range was between 40 and 65 years, with 68% males. N-butyl-2-cyanoacrylate was the most commonly used glue. Use of lipiodol varied among the studies. 28% had GOV1, 48% had GOV2, and 24% had IGV1 (Sarin classification of GV). 63% of the patients had cirrhosis. 30% were due to alcohol and 31% due to viral hepatitis. 11% of the included patients had concomitant hepatocellular carcinoma. GV obliteration with EUS-guided therapy was 84.7% (95% CI 66.8-93.8, I2 = 84.2), and with END-glue was 57.1% (38.2-74.1, I2 = 94.3), P = 0.03. GV recurrence with EUS was 6.1% (95% CI 2.6-13.7, I2 = 51.4, and with END-glue was 22.6% (13.8-34.9, I2 = 77.1), P = 0.006. Late re-bleeding with EUS was 10.6% (95% CI 6.3-17.3, I2 = 45.7), and with END-glue was 20% (14.7-26.6, I2 = 89.30), P = 0.03. The pooled rates of immediate treatment efficacy and early re-bleeding were comparable. On subgroup analysis, EUS-coil/glue combination showed superior outcomes when compared to END-glue (Table 1). The pooled rate of overall adverse events with EUS-glue was 16.5% (9.7-26.9). The rates of adverse event subtypes, and how it compared to END-glue is given in Table 2. CONCLUSION: Based on our meta-analysis, EUS-guided therapies seems to demonstrate benefit in terms of GV obliteration, GV recurrence and late GV re-bleeding. They were significantly superior when indirectly compared to END therapy. The immediate treatment efficacy and early re-bleeding rates were comparable. EUS-coil/glue, in particular, seems to be the best modality.

Esophageal Ulcers in Primary Biliary Cholangitis: A Rare Cause of Hematemesis in the Setting of Portal Hypertension and Esophageal Varices

A previously unseen manifestation of primary biliary cholangitis with portal hypertension and a new type of esophageal ulcer that should be explored as a possible alternate source of bleeding in PBC patients to expedite early recognition and treatment. We present a case of a 71-year-old Caucasian female with PBC presented with hematemesis. EGD revealed cratered and linear ulcers with irregular shapes and blood intermittently oozing from the center in the esophagus in between >5mm non-bleeding esophageal varices in the middle and lower one third of the esophagus. Bipolar cautery was used successfully to halt bleeding from the ulcers and she was discharged with appropriate outpatient follow up with recommendations of non-selective beta blockers and follow-up EGD. Our patient had unexpected and unusual cause of hematemesis in the form of multiple esophageal ulcers in-between variceal columns. Nonvariceal bleeding in the presence of varices is extremely uncommon in PBC. Cirrhosis causing portal hypertension can cause an abnormally enlarged and thickened submucosal veins known as varices. In events of ischemia, these thickened veins cannot compensate further as they have reached maximal dilatation and have minimal reserve, thus resulting in ulcers. Other differentials such as endoscopic band ligation, sclerotherapy, recent nasogastric tube placement, stress ulcers, caustic ingestion, antibiotics use, chronic non-steroidal anti-inflammatory drugs (NSAID) usage, CMV, HSV, Candida, GERD, or Behcet's were ruled out. Given that upon endoscopic examination, the esophageal ulcers were found to bleeding instead of the varices, they should be considered in the differential in the etiology of hematemesis in primary biliary cirrhosis and thoroughly searched for during an endoscopic procedure for early treatment and subsequent secondary prevention.1813_A Figure 1. Esophageal Ulcers and Varices in Primary Biliary Cholangitis1813_B Figure 2. Ulcers in between Esophageal Varices

A Rare Case of Endotipsitis with Multiorgan Involvement

Transjugular intrahepatic portosystemic shunt (TIPS) is commonly used in the setting of portal hypertension and associated symptoms such as variceal bleeding. Infection of the TIPS, or Endotipsitis, is a condition being increasingly recognized. Endotipsitis has an estimated incidence of 1.5% and carries a very high mortality rate. However, there are no guidelines to manage this condition. Here we describe a case of endotipsitis with multiple causative organisms and complications involving several organs. A 41 yo male with history of autoimmune pancreatitis, biliary strictures, and portal hypertension with gastroesophageal varices underwent banding and a TIPS procedure complicated by thrombosis which required revision. He subsequently developed a polymicrobial bacteremia growing Enterobacter cloacae, Klebsiella pneumonia, and Enterococcus faecalis/faecium. TTE and CT Abdomen/Pelvis were negative. Patient was diagnosed with endotipsitis and discharged on a 6-week course of IV daptomycin and ertapenem. Two weeks later, he presented febrile (102°). He was otherwise asymptomatic. Blood cultures were positive for gram positive cocci. Fluid around TIPS was drained. Both blood cultures and the TIPS fluid cultures were growing Enterococcus faecium and Candida lusitaniae. He was also diagnosed with endocarditis by TTE and fungal chorioretinitis. He was to complete a 6-week course of ampicillin and ceftriaxone, fluconazole, and ciprofloxacin. Exact diagnostic criteria of endotipsitis are still being debated, though persistent bacteremia and fever without clear alternative source of infection following TIPS procedure is highly suggestive. Additionally, TIPS thrombosis, as demonstrated in our patient, is a strong risk factor for endotipsitis. As of mid-2017, approximately 56 cases of endotipsitis have been reported with a 32% mortality rate. A wide range of causative organisms have been documented. However, the most common organisms responsible for early endotipsitis (within 120 days) were gram positive bacteria, including staph and enterococci. Candida infections have been associated with the highest mortality at >60%. While anti-microbial agents are the mainstay of treatment, it is unclear how liver transplantation and chronic suppressive antibiotics fit into the long-term management. Given the increased identification of this condition, clinical practice guidelines for the diagnosis and management for endotipsitis are urgently needed.

Focus on histological abnormalities of intrahepatic vasculature in chronic viral hepatitis.

The histological intrahepatic microvasculature lesions have not been deeply investigated outside the setting of portal hypertension. The aim of this study was to analyse the type and the prevalence of microvasculature abnormalities and their correlation with inflammatory activity, fibrosis stage and tissue markers of fibrogenesis, angiogenesis and oxidative DNA damage in liver biopsies obtained from patients with chronic viral hepatitis. Seventy-four liver biopsies from untreated patients affected by hepatitis B (22 cases) and C (52 cases) were included. The presence of microvascular changes was correlated with (i) the severity of the activity and fibrosis; (ii) immunohistochemical markers of angiogenesis (CD34) and hepatic stellate cells activation (alpha-smooth muscle actin); (iii) a tissue marker of oxidative damage (8-OHdG adducts). Sixty-five out of 74 biopsies (87.8%) showed vascular lesions. Portal angiomatosis was the most prevalent (62.2%) and it was associated with, on 1 side, the fibrosis stage at both univariate (P<.0001) and multivariate analysis (P=.01, OR=9.4 [1.6-54]) and, on the other, with angiogenesis (P=.05) and hepatic stellate cells activation (P=.002). Interestingly, 36/46 cases with portal angiomatosis were at early/intermediate fibrosis stage. The hepatic stellate cells activation was also associated with the presence of aberrant periportal vessels (P=.01). The histological alterations of intrahepatic microvasculature, usually seen in cirrhosis and portal hypertension, occur in chronic viral hepatitis even at early/intermediate fibrosis stages. Their correlation with angiogenesis and fibrogenesis supports a possible involvement in disease progression.