Evidence Of Portal Hypertension Research Articles

A201 AORTO-ESOPHAGEAL FISTULA AS A COMPLICATION OF ESOPHAGEAL VARICEAL BANDING

Abstract Aims This is the first reported case of bleeding from an aorto-esophageal fistula as a consequence of ischemic esophageal ulceration post esophageal variceal banding. Methods A 46-year-old male presented with epigastric pain for one-week, which was not associated with fever, jaundice, or symptoms of GI bleed. He was hemodynamically stable with an unremarkable exam and basic labs. He underwent a CT scan which showed chronic portal vein thrombosis extending from the main portal vein into the superior mesenteric vein and evidence of portal hypertension, along with massive esophageal varices. Anticoagulation was not started due to risk of GI bleed. Upper endoscopy confirmed the presence of three columns of large esophageal varices in the distal and mid esophagus, the largest was 3 cm in size. There was no stigmata of recent bleeding and no gastric varices. Seven bands were applied. CT abdomen was repeated on the seventh day of admission, showing extension of portal vein thrombosis. As such, the thrombosis team started a low dose of anticoagulation. (dalteparin 7500 IU). He tolerated it well and showed no signs of bleeding prior to discharge. The day after discharge, the patient presented again with an episode of melena. Examination was remarkable for tachycardia, hypotension and melena. Hemoglobin was stable. Patient was admitted with impression of upper GI bleed most likely secondary to post banding ulcer. He was started on medical therapy. Anticoagulation was held. Next day, the patient had an episode of hematemesis with large amount of blood and clots. Emergent upper endoscopy showed active bleeding in the distal esophagus. Further examination showed a full-thickness, 7 mm defect in the lower esophagus with fresh red blood emptying from it into the esophagus (Figure 1). Thoracic surgery was consulted and suspected a perforated esophageal ulceration at the site of previous banding, with an aorto-esophageal fistula. Patient deteriorated immediately and passed away. The cause of death was cardiac arrest secondary to acute blood loss anemia. Results AEF is a recognized cause of upper GI bleeding with a high mortality rate. While rare, it can occur secondary to ischemic esophageal ulcer post EV banding. Conclusions This is the first reported case of an aorto-esophageal fistula as a consequence esophageal variceal band ligation. While extremely rare, this complication should be considered as a potentially fatal complication of variceal banding, especially in the setting of a diseased or previously repaired aorta.

Evaluation of patients treated with direct-acting anti-viral therapy for chronic hepatitis C and their risk of hepatocellular carcinoma in Hong Kong

Background & aimTo evaluate the risk of early hepatocellular carcinoma (HCC) in chronic hepatitis C patients treated with direct-acting antivirals (DAAs) in Hong Kong, as it has not been studied before in this locality.MethodsThree hundred thirty-three consecutive chronic hepatitis C patients treated with DAAs from two hospitals over the past 6 years were identified. Kaplan-Meier method was used to calculate cumulative HCC incidence. Cox regression was used to identify factors associated with HCC development.ResultsDuring a median follow-up of 23.4 months after DAA started, 15 (5.4%, 95% CI 3.3–8.7%) out of 279 total included patients developed HCC. The overall sustained virological response (SVR) rate was 98.9%. The 1-year cumulative incidence for de-novo HCC and HCC recurrence were 0.8 and 30.9%, respectively (log-rank test p < 0.001). The 1-year cumulative HCC incidence for patients without and with cirrhosis were 0.7 and 5.1%, respectively (log-rank test p = 0.036). Univariate analysis showed that significant factors associated with HCC after DAA were: history of treated HCC, cirrhosis, evidence of portal hypertension, higher AFP at the start or end of DAA therapy, higher bilirubin, lower platelets, lower albumin, and older age. From receiver operating characteristic curve analysis, the optimal cut-off level of AFP for predicting HCC was 10.5 ng/mL at the start and 5.6 ng/mL at the end of DAA therapy.ConclusionsThe risk of early HCC recurrence remains high despite achieving SVR following DAA therapy, whereas the risk of early de-novo HCC occurence is low. AFP levels, both at the start and end of DAA therapy, can be useful in stratifying risks of HCC development.

Liver Transplantation in a Woman with Mahvash Disease

SummaryMahvash disease is an exceedingly rare genetic disorder of glucagon signaling characterized by hyperglucagonemia, hyperaminoacidemia, and pancreatic α-cell hyperplasia. Although there is no known definitive treatment, octreotide has been used to decrease systemic glucagon levels. We describe a woman who presented to our medical center after three episodes of small-volume hematemesis. She was found to have hyperglucagonemia and pancreatic hypertrophy with genetically confirmed Mahvash disease and also had evidence of portal hypertension (recurrent portosystemic encephalopathy and variceal hemorrhage) in the absence of cirrhosis. These findings established a diagnosis of portosinusoidal vascular disease, a presinusoidal type of portal hypertension previously known as noncirrhotic portal hypertension. Liver transplantation was followed by normalization of serum glucagon and ammonia levels, reversal of pancreatic hypertrophy, and resolution of recurrent encephalopathy and bleeding varices.

Primary sclerosing cholangitis revealed by pregnancy

Introduction: Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease of poorly understood, probably dysimmune, mechanism. It is a slow-growing disease, with a tendency to worsen. We are reporting a rare case of primary sclerosing cholangitis revealed during pregnancy. Case presentation: A 30-year-old woman who had pruritus during 12 weeks of gestation with a disturbance of the liver biologic tests. Imaging investigations found a chronic liver with areas of stenosis and dilation of VBIH and right and left hepatic bile ducts suggestive of primary sclerosing cholangitis with evidence of portal hypertension. She was put under treatment by ursodeoxycholic acid and the pregnancy proceeded without maternal-fetal complications giving birth to a healthy newborn with uneventful postpartum follow-up. Conclusion: This case highlights the importance of considering pathologies other than benign intrahepatic cholestasis of pregnancy as the cause of cholestasis during pregnancy.

Bile acid increase during bulevirtide treatment of hepatitis D is not associated with a decline in HDV RNA.

Bulevirtide (BLV) is an entry inhibitor blocking entry of HBsAg into hepatocytes by interfering with the bile acid transporter Na+-taurocholate co-transporting polypeptide. We here investigated if bile acid levels before or during BLV treatment would correlate with HDV RNA declines. We studied 20 patients with compensated HDV infection receiving a daily dose of 2mg bulevirtide subcutaneously qd for at least 24 weeks. ALT levels improved in all patients including 13/20 patients showing normal ALT values at treatment Week 24. An HDV RNA drop of at least 50% was evident in 20/20 patients at Week 24 including 10 patients showing a ≥ 2 log HDV RNA decline. Elevated bile acid levels were detected already before treatment in 10 patients and further increased during BLV administration with different kinetics. Baseline bile acids were associated with higher transient elastography values (p= .0029) and evidence of portal hypertension (p= .0004). Bile acid levels before treatment were associated with HDV RNA declines throughout therapy, but not at Week 24 (rho=-0.577; p= .0078; rho=-0.635, p= .0026; rho=-0.577, p= .0077; rho=-0.519, p= .0191; rho=-0.564, p= .0119 and rho=-0.393, p= .087 at treatment Weeks 2, 8, 12, 16, 20 and 24, respectively). However, bile acid increases during treatment were not associated with HDV RNA or ALT declines at any of the time points. BLV-induced increases in bile salts do not correlate with HDV RNA declines suggesting that the inhibitory effects of BLV on NTCP differ between blocking bile acid transport and hindering HBsAg entry. If baseline bile salt levels could be useful to predict virological response remains to be confirmed.

S2963 Congenital Riedel’s Lobe of the Liver: A Case Report

Introduction: Riedel lobe of the liver is a rare anatomical variation with a reported incidence to be between 3.3% and 14.5%. We report a case of a 43-year-old female with an incidental finding of non-palpable Riedel’s lobe. Case Description/Methods: A 43-year-old female was referred for evaluation of hepatomegaly, which was revealed on MRI and CT scan dating back to 2016. Medical history notable for Irritable Bowel Syndrome (IBS), uterine fibroids, and a history of a tumor removal from her right breast. Patient denies any history of alcohol, illicit drugs, hepatotoxic medications, or pre-existing liver disease. Physical exam was unremarkable and abdominal exam did not reveal any mass or abnormalities. Routine blood examination, including LFTs and iron studies, was within normal limits. Hepatitis panel (A, B, C), anti-smooth muscle antibody, and LKM-1 IgG antibody was negative. ANA, alpha-1 antitrypsin, and tissue transglutaminase were all negative. The only lab abnormality was an elevated IgG mitochondrial M2 antibody 54.5 (normal less than 20 units). CT abdomen was significant for an enlarged liver with the right lobe extending into the pelvis and ‘not completely included in the study’. MRI abdomen revealed a markedly enlarged liver measuring up to 23.8cm in its craniocaudal dimension with extension into the pelvis with the pancreas deviated to the left, likely secondary to the prominent hepatomegaly. Venous duplex significant for normal directional flow in the portal and hepatic veins with no evidence of portal hypertension. Liver biopsy revealed signs of sinusoidal dilatation nonspecific for veno-occlusive outflow obstruction with no signs of inflammation, steatosis, or fibrosis. The patient was diagnosed with Riedel’s lobe of the liver. She was discharged from the hospital without treatment with a recommendation to repeat an MRI in 1 year, as torsion is a reported complication of Riedel’s lobe over time. Patient will be recommended to repeat LFTs and anti-mitochondrial antibody to determine progression/significance prior to follow up in 6 months. Discussion: Riedel’s lobe of the liver is a rare anatomical variant that is often incidentally found on imaging or the presence of hepatomegaly on physical exam. Although patients are usually asymptomatic, its presentation can vary, ranging from nonspecific symptoms to more severe symptoms such as torsion, obstruction, rupture, and bleeding. The range of symptoms highlights its importance in diagnosis and surveillance in this patient population.Figure 1.: (A) CT of the abdomen/pelvis from 2016. Liver measuring up to 197.3mm in the sagittal plane. (B) CT of the abdomen/pelvis from 2020. Liver measuring up to 215.4mm in the sagittal plane.

Identification and clinical significance of nodular regenerative hyperplasia in primary sclerosing cholangitis.

Background and AimPrimary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts. PSC is frequently associated with inflammatory bowel disease (IBD). Nodular regenerative hyperplasia (NRH) can occur in IBD with the use or even in the absence of thiopurine treatment. We aimed to study the significance of the presence of NRH and obliterative portal venopathy (OPV), both causes of non‐cirrhotic portal hypertension (NCPH), in patients having PSC.MethodsPatients with PSC and concurrent NRH on liver biopsy were identified from the digital pathology database covering the period 2003–2019. Evaluation of liver biopsy and the original diagnoses were confirmed on review based on standard histological features diagnostic for NRH and OPV. Clinical and laboratory data were obtained from electronic medical records.ResultsThirty‐one patients (21 male, 10 female; median age at biopsy 40.1 years) were included in the study. Twelve (38.7%) patients had OPV in addition to NRH on the liver biopsy. Nineteen (61.2%) patients had IBD including 11 with Crohn's disease (CD), 7 with ulcerative colitis (UC), and 1 with indeterminate colitis. Thirteen (41.9%) patients had evidence of portal hypertension, 10 (32.2%) with esophageal varices, 4 (12.9%) with history of variceal bleeding, 6 (19.3%) with ascites, and 14 (12.9%) with splenomegaly. Eleven (35.4%) patients had a cirrhotic‐appearing liver on imaging. Twelve (38.7%) patients had a history of prior or current thiopurine use.ConclusionsThe current study suggests that NRH with or without OPV independently occurs in patients having PSC and may lead to NCPH, even in the absence of concurrent IBD and/or thiopurine therapy.

A Study on FibroScan and Endoscopic Finding in Patients of Chronic Liver Disease attending Tripura Medical College and Dr. B.R. Ambedkar Memorial Teaching Hospital.

Chronic liver disease (CLD) represents different liver disorders of varying severity and etiology in which hepatic inflammation and fibrosis continue at least for 6 months. Portal hypertension is one of the important complications of CLD and its early recognition is of paramount importance. Though liver biopsy remains the gold standard for diagnosing liver fibrosis and upper gastrointestinal (GI) endoscopy plays an important role in diagnosing different findings of portal hypertension, various noninvasive methods like FibroScan are being increasingly used to diagnose liver fibrosis. Study the FibroScan and endoscopic findings in patients of CLDs and the objectives are to find the prevalence of portal hypertension and to find various grades of esophageal varix and portal hypertensive gastropathy (PHG) and its relationship with liver fibrosis by FibroScan. A total of 114 patients of CLD and compensated cirrhosis having childturcotte- pugh (CTP) stages A and B were included in the study fulfilling inclusion and exclusion criteria, after calculating the sample size of 100. All the patients underwent detailed history, physical and gastrointestinal examination. Complete blood count (CBC), liver function test (LFT), kidney function test (KFT), viral markers were done. Aspartate aminotransferase (AST) to platelet ratio index (APRI) score was calculated, liver fibrosis was estimated by FibroScan and evidence of portal hypertension was documented by upper GI endoscopy. Cutoff value of FibroScan, APRI score, and model for end-stage liver disease (MELD) score for portal hypertension was decided by receiver operating characteristic (ROC) curve. Alcoholic liver disease (ALD) was the most common cause (43%) of CLD closely followed by nonalcoholic fatty liver disease (NAFLD) in 42% cases followed by chronic viral hepatitis, 75% patients had evidence of portal hypertension with PHG being the most common followed by esophageal varix. F4 fibrosis was found in 73% of cases followed by F3, F2, and F1 fibrosis. FibroScan value of 12.2 kPa was predictive of presence of portal hypertension and value of 26.6 mm predicted the presence of large esophageal varices.

Fontan-associated liver disease.

Fontan-associated liver disease.

20-Year-Old Man With Elevated Liver Tests

20-Year-Old Man With Elevated Liver Tests

Abstract P5-18-03: Hepatopulmonary syndrome with long term use of ado-trastuzumab emtansine (T-DM1)

Abstract Introduction: Hepatopulmonary syndrome (HPS) is characterized by hypoxemia, portal hypertension, and intrapulmonary shunting and manifests as dyspnea. The portal hypertension may be a result of cirrhosis or non-cirrhotic causes such as nodular regenerative hyperplasia of the liver that is associated with drug therapy. Progressive hypoxemia often occurs despite stable liver function. Ado-trastuzumab emtansine (T-DM1; KADCYLA) is an antibody-drug conjugate comprised of a humanized monoclonal antibody trastuzumab covalently linked to the cytotoxic agent maytansine. T-DM1 is prescribed in the postneoadjuvant setting in persons with residual invasive HER2-positive (HER2+) breast cancer (BC) as well as in persons with metastatic HER2+ BC who were previously treated with trastuzumab and a taxane. Liver toxicities including an asymptomatic, transient increase in transaminases and nodular regenerative hyperplasia that results in portal hypertension have been described; however, HPS secondary to noncirrhotic portal hypertension occurring with long-term exposure to T-DM1 has not been reported. We have identified patients (pts) with this infrequent, yet serious complication, and we aim to better characterize their clinical course to guide earlier detection and hopefully prevent irreversible hypoxic respiratory failure. Methods: Institutional search tools were utilized to identify pts at Mayo Clinic with HER2+ BC treated with T-DM1 in either the postneoadjuvant or metastatic setting. Electronic health records were manually reviewed for HPS criteria including 1) hypoxemia 2) portal hypertension and 3) intrapulmonary shunt/vasodilation confirmed by contrast echocardiogram or macroaggregated albumin lung perfusion scan. For pts meeting criteria for HPS, imaging was independently reviewed by a hepatologist to determine whether there was preexisting liver disease or if changes occurred after initiation of T-DM1. Results: We identified 259 pts with HER2+ BC treated with T-DM1 in the postneoadjuvant (n=70) or metastatic setting (n=185). Four pts met criteria for HPS (1.5%) while receiving T-DM1 for metastatic disease (Table 1; listed in chronological order of onset of HPS). Median age was 43 (range 35 - 53 years), median prior lines of treatment was 1.5 (range 0 - 7), and the median number of cycles of T-DM1 prior to discontinuation was 68.5 (range 51 - 90). All pts had evidence of left atrial enlargement on echocardiogram, and evidence of portal hypertension as determined by the presence of varices, and splenomegaly. Three pts had documented telangiectasias as well as Grade 1-2 elevations in serum transaminases and bilirubin. Elevations in hemoglobin were noted in 2 pts during treatment with T-DM1. All pts had a confirmed intrapulmonary shunt via contrast echocardiogram and a lung perfusion scan. Three pts discontinued T-DM1 after diagnosis of HPS and experienced some clinical improvement in hypoxia. One pt died two months after stopping T-DM1 due to progression of BC. Conclusion: HPS may occur in the setting of prolonged exposure to T-DM1 and can be associated with severe hypoxic respiratory failure. Further data are required to characterize the spectrum of liver injury that occurs with long-term use of T-DM1 to provide further guidance to clinicians as regards monitoring for these adverse effects. Patients who developed hepatopulmonary syndrome while on T-DM1Age, yrsSites of metastases# lines of prior txCycles of T-DM1Presenting sign or symptomsNon-contrast ECHO findingsSupplemental oxygenTransaminitis; hyperbilirubinemiaEvidence of portal hypertensionTelangiectasiasHgb prior to T-DM1Hgb end of txLung perfusion studyContrast ECHOPt 153LN, bone090DOE, hypoxemia, clubbingleft atrial enlargement (62 mg/m2)YesNovarices, splenomegalyNot documented12.9 g/dL10.7 g/dL13.5%Yes, small shuntPt 235LN, bone, liver751DOEleft atrial enlargement (54 mg/m2)YesGrade 1; Grade 2varices, splenomegaly; thrombocytopeniaYes, face and handsn/a11.9 g/dL24%Yes, large shuntPt 338LN, bone251Hyperbilirubinemiaborderline left atrial enlargementYesGrade 1; Grade 2varices, splenomegalyYes, trunk, extremities, face11.7 g/dL16.2 g/dL17.8%Yes, moderate shuntPt 448LN, liver186DOEleft atrial enlargement (49 mg/m2)NoGrade 1; Grade 2varices, splenomegalyYes, trunk12.5 g/dL16.1 g/dL7.9%Yes, large shunt Citation Format: Ally Higgins, Adham K Alkurashi, Patrick S Kamath, Vivek N Iyer, Ciara O'Sullivan, Tufia C Haddad. Hepatopulmonary syndrome with long term use of ado-trastuzumab emtansine (T-DM1) [abstract]. In: Proceedings of the 2021 San Antonio Breast Cancer Symposium; 2021 Dec 7-10; San Antonio, TX. Philadelphia (PA): AACR; Cancer Res 2022;82(4 Suppl):Abstract nr P5-18-03.

DISSEMINATED NOCARDIOSIS PRESENTING AS ACUTE LIVER FAILURE AND INTRAPARENCHYMAL HEMORRHAGE

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Nocardiosis is an opportunistic infection that most commonly manifests as pulmonary disease. Disseminated nocardiosis is rare and is associated with high mortality. We present a case of a woman with disseminated nocardiosis presenting with acute liver failure complicated by intraparenchymal hemorrhage. CASE PRESENTATION: A 30-year-old woman with a history of alcohol abuse presented with jaundice, bloating, and diarrhea for 2 weeks. Laboratory examination revealed hemoglobin of 5.2 g/dL, platelets 60,000/µL, INR 3.4, AST 113 U/L, ALT 38 U/L, and total bilirubin of 30 mg/dL. MRCP revealed a normal biliary tree, nodular liver contour and evidence of portal hypertension. Acetaminophen level and acute viral hepatitis panel were negative. HIV serology and autoimmune hepatitis panel were negative. Chest CT revealed a 5 x 3.5 cm subpleural mass in the left upper lobe with areas of gas density (Fig 1). Her hospital course was complicated by a rapid deterioration in mental status and DIC. Head CT revealed an acute left occipital-parietal intraparenchymal hemorrhage. She was intubated and transferred to the ICU. Bronchoscopy with BAL grew Nocardia otitidiscaviarum. Brain MRI revealed FLAIR signal hyperintensity lining the ventricles, concerning for ventriculitis or abscess (Fig 2). An external ventricular drain was placed for interval development of hydrocephalus. The patient received IV trimethoprim-sulfamethoxazole and intrathecal amikacin. Given her poor prognosis, her family elected to withdraw care. DISCUSSION: Nocardia is a branching, filamentous gram-positive bacilli found ubiquitously in soil. Infection usually occurs by inhalation of aerosolized bacteria resulting in predominant lung involvement with potential hematogenous spread to other organs. Patients with impaired T-cell mediated immunity, such as HIV, chronic steroid use, diabetes, or alcoholism are at risk of developing severe infection. Our patient had a longstanding history of heavy alcohol use, likely predisposing her to disseminated infection. Nocardiosis is considered disseminated when two or more nocardial abscesses are found at two or more locations. Up to 20% of all Nocardia infections involve the brain. Current guidelines recommend imaging of the brain for any patient with confirmed or suspected pulmonary nocardiosis. Prognosis is usually poor, and mortality can be as high as 64%. First-line treatment is trimethoprim-sulfamethoxazole. However some species, such as N. otitidiscaviarum found in our patient, are occasionally resistant. Due to suspected CNS involvement, dual coverage was recommended with the addition of IV amikacin at dosing for CNS penetrance. CONCLUSIONS: In patients presenting with a cavitary lung lesion and associated brain lesion, nocardiosis should be considered in the differential. Early brain imaging may be warranted in patients presenting with severe disease. REFERENCE #1: Pulmonary nocardiosis: risk factors and outcomes. AU Martínez Tomás R, Menéndez Villanueva R, Reyes Calzada S, Santos Durantez M, Vallés Tarazona JM, Modesto Alapont M, Gobernado Serrano M SO Respirology. 2007;12(3):394. REFERENCE #2: Conville PS, Witebsky FG. Nocardia, Rhodococcus, Gordonia, Actinomadura, Streptomyces, and other Aerobic Actinomycetes. In: Murray PR, Baron EJ, Jorgensen JH, Landry ML, Pfaller MA, editors. Manual of Clinical Microbiology. 9th ed. Washington, DC: ASM Press; 2007. p. 515. DISCLOSURES: No relevant relationships by Muhammad Arif, source=Web Response No relevant relationships by Abdul Rahman Halawa, source=Web Response No relevant relationships by MAYKEL IRANDOST, source=Web Response no disclosure on file for Sean Marco; No relevant relationships by Andrew Talon, source=Web Response No relevant relationships by Anthony Vaccarello, source=Web Response

Clinical practice guidelines for hepatocellular carcinoma between Korea, Europe, and United States

Clinical practice guidelines for hepatocellular carcinoma between Korea, Europe, and United States

Increased canine pancreatic lipase immunoreactivity (cPLI) and 1,2-o-dilauryl-rac-glycero-3-glutaric acid-(6'-methylresorufin) ester (DGGR) lipase in dogs with evidence of portal hypertension and normal pancreatic histology: a pilot study.

The clinical presentations of both liver disease and pancreatitis are nonspecific and overlapping, which may cause difficulty in diagnosis. In our retrospective pilot study, we assessed whether dogs with evidence of portal hypertension and absence of pancreatitis on pancreatic histology have increases in canine pancreatic lipase immunoreactivity (cPLI) and 1,2-o-dilauryl-rac-glycero-3-glutaric acid-(6'-methylresorufin) ester (DGGR) lipase. We included dogs that had been presented between 2008 and 2019 if they had normal pancreatic histology, histologically confirmed hepatopathy, and if canine pancreas-specific lipase (Spec cPL; Idexx) or DGGR lipase had been measured. Only dogs with portal hypertension were included. Six dogs fulfilled the inclusion criteria. Four of 6 and 2 of 6 dogs had Spec cPL and DGGR lipase exceeding the upper reference limit, respectively. From the 4 dogs with increased Spec cPL, 2 had concentrations of 200-400 µg/L and 2 had concentrations ≥ 400 µg/L. Our results suggest that canine portal hypertension might lead to increased Spec cPL and DGGR lipase values in the absence of pancreatitis on histology. Until more evidence in a larger number of dogs with portal hypertension is available, both tests should be interpreted cautiously in the presence of portal hypertension.

Hepatic vein thrombosis secondary to COVID-19

COVID-19 is known to cause both arterial and venous thromboembolism. Although a lot of attention is being given to those presenting with myocardial infarction and stroke, splanchnic vein thrombosis which includes portal, mesenteric, splenic, and hepatic vein thrombosis is often underreported. A 21-year-old female presented with progressive abdominal distension and obstipation for the past 2 weeks. The patient was diagnosed as a case of moderate COVID-19 illness 2 weeks before the onset of these symptoms and recovered on receiving treatment according to the COVID protocol. Physical examination revealed pallor and bilateral pitting pedal edema up to ankles. Systemic examination revealed uniformly distended abdomen with signs of free fluid and tenderness in the right hypochondrium. Hematological investigations showed moderate-grade microcytic hypochromic anemia. Biochemically, indirect hyperbilirubinemia and transaminitis with moderately elevated D-dimers and Lactate dehydrogenase (LDH) was noted. Radiological imaging of the abdomen (including USG with doppler and CT contrast Scans) revealed gross ascites, nutmeg appearance of liver with completely obscured hepatic veins indicating no flow of blood, multiple intrahepatic portosystemic collaterals and a partially thrombosed inferior vena cava with no evidence of portal hypertension. All other workup to rule out other causes of hepatic vein thrombosis was inconclusive. The patient was treated with low-molecular-weight heparin and then continued with oral warfarin at discharge. On a 4-week follow-up, the patient showed marked clinical improvement with partial resolution of presenting symptoms.

An Interesting Case of Autoimmune Liver Disease

Abstract Autoimmune liver diseases (AILD) are part of a broad spectrum of liver diseases with autoimmune etiology, usually present individually but at times have overlapping features. We present the case of a 60-year-old lady presenting with fatigue, itching and right upper quadrant abdominal pain. Further investigation showed cholestatic pattern of liver enzymes and evidence of portal hypertension without any evidence of extrahepatic obstruction. Autoimmune markers and liver biopsy showed overlapping features of both autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC), which is a separate diagnosis known as overlap syndrome, but by applying appropriate criterion, we were able to make a definite diagnosis of PBC. Differentiating PBC from overlap syndrome was important as therapy of both are different.

Non-invasive splenic parameters of portal hypertension: Assessment and utility

BACKGROUNDPortal hypertension is a major complication of cirrhosis that is associated with significant morbidity and mortality. The present gold-standard method to risk stratify and observe cirrhosis patients with portal hypertension is hepatic venous pressure gradient measurement or esophagogastroduodenoscopy. However, these methods are invasive, carry a risk of complications and are associated with significant patient discomfort. Therefore, non-invasive splenic parameters are of clinical interest as potential useful markers in determining the presence of portal hypertension. However, diagnostic accuracy and reproducibility remains unvalidated.AIMTo assess the diagnostic accuracy of spleen stiffness, area and diameter in predicting the presence of portal hypertension.METHODSOf 50 patients with varying liver disease pathologies were prospectively recruited from the St. Mary’s Hospital Liver Unit in London; 25 with evidence of portal hypertension and 25 with no evidence of portal hypertension. Liver stiffness, spleen stiffness, spleen diameter and spleen area were measured using the Philips Affiniti 70 elastography point quantification point shear wave elastography system. The aspartate aminotransferase-to-platelet-ratio-index (APRI) score was also calculated. Performance measures, univariate and multivariate logistic regression were used to evaluate demographic, clinical and elastography variables. Interclass correlation coefficient was used to determine the reproducibility of splenic area and diameter.RESULTSOn univariate and individual performance, platelet count [area under the receiver operating characteristic (AUROC) 0.846, P value < 0.001], spleen area (AUROC 0.828, P value = 0.002) and APRI score (AUROC 0.827, P value < 0.001) were the most accurate variables in identifying the presence of portal hypertension. On multivariate logistic regression models constructed, the combination of spleen area greater than 57.90 cm2 and platelet count less than 126 × 109 had 63.2% sensitivity and 100% specificity, 100% positive predictive value and 100% negative predictive value. An alternative combination of spleen stiffness greater than 29.99 kPa and platelet count less than 126 × 109 had 88% sensitivity, 75% specificity, 78.6% positive predictive value and 85.7% negative predictive value. An interclass correlation coefficient value of 0.98 (95%CI: 0.94-0.99, P value < 0.001) and 0.96 (95%CI: 0.91-0.99, P value < 0.001) were determined for inter-operator variability for spleen area and diameter respectively.CONCLUSIONSpleen area, spleen stiffness and platelet count may be useful markers to assess the presence of portal hypertension in patients of various etiologies.

S1544 An Interesting Presentation of Cholangiocarcinoma: Esophageal Varices

INTRODUCTION: Esophageal varices are an uncommon presentation for cholangiocarcinoma. CASE DESCRIPTION/METHODS: A 75 year old female with a past medical history of anemia presented to her primary care physician with a complaint of fatigue for two months. The patient took NSAIDs regularly, but denied any melena, hematochezia or hematemesis. Lab work confirmed iron deficiency anemia, she was referred for upper endoscopy and colonoscopy. Findings were notable for grade I varices in the lower third of the esophagus and grade I internal hemorrhoids. Given the findings of varices, she was referred for a RUQ ultrasound with findings of cirrhosis without evidence of portal hypertension. The patient had no history of alcohol abuse or viral hepatitis, so these findings were concerning for a diagnosis of NASH. The patient was hospitalized after developing jaundice, abdominal distension and a 15-pound weight loss with associated clay colored stools and dark urine. At the time of admission, her total bilirubin was 22. MRI of the abdomen revealed intrahepatic and extrahepatic bile duct dilation with abrupt narrowing suspicious for stricture. Other findings included cirrhosis, esophageal varices, mild ascites, and mild splenomegaly. The patient underwent ERCP with findings of moderate dilation of the upper and middle third of the main bile duct, left and right hepatic ducts, all intrahepatic branches and the common hepatic duct. A biliary sphincterotomy was performed and 1 stent placed. Brushings of the common bile duct were notable for cholangiocarcinoma. A subsequent PET scan demonstrated mild avidity in the area of the CBD but no discrete lesion and no metastatic disease. DISCUSSION: Cancer arising from the perihilar region accounts for 60-70% of extrahepatic cholangiocarcinomas and are further classified according to the pattern of involvement of the hepatic ducts. Tumors involving the hepatic duct bifurcation, hilar cholangiocarcinomas, are collectively called Klatskin tumors. Patients with extrahepatic cholangiocarcinomas become symptomatic when there is obstruction of the biliary drainage system. Typical presenting symptoms include dark urine, jaundice, clay colored stools, weight loss, and abdominal pain. Small extrahepatic bile duct cancers may not be visualized on imaging, but an obstructing malignant lesion is suggested by ductal dilation in the absence of stones. Proximal lesions cause dilation of just the intrahepatic ducts, while distal lesions cause dilations of both the intrahepatic and extrahepatic ducts.Figure 1.: Grade I Esophageal Varices.Figure 2.: Esophageal varices in the lower third of the esophagus.Figure 3.: Common Bile Duct Dilation on MRI.

S1426 Double Trouble: Chronic Pancreatitis and an Overlooked Superior Mesenteric Vein Thrombosis

INTRODUCTION: Chronic pancreatitis is a challenging disease process to manage. Abdominal pain is the most common, debilitating symptom for these patients. Here, we outline the outpatient approach and management of a patient with chronic pancreatitis who also had concomitant mesenteric venous thrombosis. CASE DESCRIPTION\METHODS: A 56-year-old man with history of tonsillar squamous cell carcinoma and non-small cell lung cancer treated with radiation, coronary artery disease, heavy tobacco use, cholecystectomy, and chronic pancreatitis complicated by pseudocysts and exocrine insufficiency presented to internal medicine clinic with persistent epigastric pain. Three years ago, he was diagnosed with chronic pancreatitis, secondary to heavy alcohol and tobacco use. In the interim, he quit alcohol, reduced smoking, and went through multiple therapeutic regimens including gabapentin, opioids, pancreatic enzyme replacement, and celiac plexus block. On exam, he was hemodynamically stable but in discomfort due to pain. He was cachectic and had epigastric tenderness without rebound tenderness or guarding. Laboratory tests revealed cholestatic transaminitis. We referred him to a gastroenterologist. MRCP showed irregular contour of the pancreas with smooth narrowing in the pancreatic neck concerning for a stricture. EGD and EUS were planned. Duloxetine was added to his regimen. However, MRI revealed an occlusion of the superior mesenteric vein at the level of the portal confluence with multiple collateral vessels at the root of the mesentery. Prior CT scan showed the presence of thrombus two years ago. Medical record did not reveal any management of the thrombus and the patient was unaware of the condition. He did not have evidence of portal hypertension. Given the severity of the symptoms and lack of prior anticoagulation, we started him on rivaroxaban. Despite this intervention, he continued to have flares of chronic pancreatitis in the subsequent months and will undergo another celiac plexus block. DISCUSSION: Chronic mesenteric venous thrombosis is often an incidental finding on imaging and usually asymptomatic. This is mainly considered a consequence of venous stasis and endothelial injury, compounded by risk factors such as local inflammation (e.g., pancreatitis), abdominal tumors or pseudocysts, and acquired thrombophilia. Anticoagulation is generally recommended. In the absence of predisposing illness or a hypercoagulable state, further work-up for portal hypertension and referral to hematology are indicated.

Simeprevir, daclatasvir, and sofosbuvir for hepatitis C virus-infected patients: Long-term follow-up results from the open-label, Phase II IMPACT study.

Background and aimsDirect‐acting antiviral agents (DAAs) for hepatitis C virus (HCV) infection have resulted in high rates of sustained virologic response (SVR) following 8 to 24 weeks of treatment. However, difficult‐to‐cure/cirrhotic patients typically require a longer treatment duration and less is known regarding the long‐term durability of SVR or effect on liver disease progression; to assess this, the IMPACT study followed patients for a 3‐year period after end of treatment.MethodsThe Phase II, open‐label, nonrandomized IMPACT study assessed the efficacy, safety, and pharmacokinetics of the combination of three DAAs (simeprevir, sofosbuvir, and daclatasvir) in HCV genotype 1/4‐infected, treatment‐naïve/‐experienced cirrhotic patients with portal hypertension or decompensated liver disease. Patients from a single site in the United States were assigned to one of two groups by Child–Pugh (CP) score: CP A, CP score less than 7 and evidence of portal hypertension; CP B, CP score of 7 to 9. All patients received simeprevir 150 mg, daclatasvir 60 mg, and sofosbuvir 400 mg once‐daily for 12 weeks between September 2014 and August 2015. All 40 patients included in the study (male, 63%; median age, 58.5 years) achieved SVR 12 and 24 weeks after end of treatment, and the combination was well tolerated.ResultsAll patients who reached the 3‐year follow‐up timepoint maintained SVR (CP A, 15/15; CP B, 18/18). CP scores and Model for End‐stage Liver Disease scores remained relatively stable, and mean FibroScan and FibroTest scores declined. No new safety signals were identified.ConclusionsIn the IMPACT study, virologic response to simeprevir, sofosbuvir, and daclatasvir was durable over 3 years (http://ClinicalTrials.gov number: NCT02262728).