Evidence Of Cirrhosis Research Articles

Tuberculosis presenting as a liver mass in a pregnant lady.

A 20-year-old primigravida (19 weeks of gestation) presented with dull pain in the right upper quadrant for 2 weeks. She had no history of jaundice, features of hepatic decompensation, constitutional symptoms or use of oral contraceptives. An ultrasound examination revealed a heteroechoic, solid mass in the right lobe of liver (7cm x 8cm) without any evidence of cirrhosis. Liver function tests were normal, and tests for hepatitis B, hepatitis C and HIV were non-reactive. Serum alpha-fetoprotein was 99 ng/ml (<55 ng/ml at 19 weeks of gestation).

Variceal Bleed and Portal Hypertensive Gastropathy in a Noncirrhotic Patient with Isolated Splenomegaly

Portal hypertension caused by cirrhosis is the most common etiology of esophageal varices. However, abnormalities of the splenoportal axis in the absence of liver disease may also cause portal hypertension resulting in varices. We report a rare case of esophageal variceal bleed in a noncirrhotic patient with isolated splenomegaly secondary to chronic granulocyte colony stimulating factor (G-CSF) therapy. The patient is a 26-year-old male with Cohen syndrome who required long-term G-CSF treatment for chronic neutropenia. He presented with large volume hematemesis and pancytopenia in the setting of known splenomegaly with no evidence of cirrhosis. An urgent EGD revealed active variceal bleeding and portal hypertensive gastropathy. The patient was appropriately resuscitated and underwent a successful transjugular intrahepatic portosystemic shunt and CT-guided coil placement for the bleeding varices. We are the first to report variceal bleed as a complication of long-term G-CSF use, a life-threatening consequence that requires urgent intervention.

Brief Report: Accuracy of FIB-4 for Cirrhosis in People Living With HIV and Hepatocellular Carcinoma.

Hepatocellular carcinoma (HCC) may develop in the absence of cirrhosis in HIV, and determining how often this occurs can provide insights into mechanisms of carcinogenesis. Studies evaluating the prevalence of cirrhosis in the setting of HCC among people living with HIV (PLWH) often rely on noninvasive markers, such as the Fibrosis-4 Index for Hepatic Fibrosis (FIB-4). However, the accuracy of FIB-4 for cirrhosis in the setting of HCC has not been determined among PLWH. We conducted a cross-sectional study among PLWH in the Veterans Aging Cohort Study with VA cancer registry-confirmed HCC diagnosed between 1999 and 2015. FIB-4 was calculated using the age, alanine aminotransferase, aspartate aminotransferase, and platelet count obtained closest to, but within 1 year before, HCC diagnosis. Medical records were reviewed within 1 year before HCC diagnosis to determine the cirrhosis status. We evaluated the area under the receiver-operating characteristic curve and performance characteristics of FIB-4 for confirmed cirrhosis. Incident HCC was diagnosed in 302 PLWH. After medical record review, 203 (67.2%, 95% confidence interval: 61.6% to 72.5%) had evidence of cirrhosis. FIB-4 identified patients with cirrhosis with an area under the receiver-operating characteristic curve of 0.67 (95% confidence interval: 0.60 to 0.73). FIB-4 scores >5.0 had a positive predictive value >80% and specificity of >77%, negative predictive value of <41%, and sensitivity of <45%. The accuracy of FIB-4 for cirrhosis in the setting of HIV and HCC is modest and may result in misclassification of cirrhosis in this population.

Response to combination of sofosbuvir and daclatasvir in chronic hepatitis C infection.

Objectives: To determine efficacy of sofosbuvir and daclatasvir in the treatment of chronic hepatitis C infection. Study Design: Open label uncontrolled interventional study. Setting: Hepatitis Clinic, Sheikh Zayed Medical College/Hospital, Rahim Yar Khan. Period: June to December 2018. Material &amp; Methods: Five hundred treatment naïve chronic hepatitis C patients including those with compensated cirrhosis were included in the study. They were given sofosbuvir 400 mg daily and daclatasvir 60 mg daily. Weight based ribavirin was added if patient has evidence of cirrhosis. Treatment duration was 12 weeks for non-cirrhotic and 24 weeks for cirrhotics. End of treatment response (ETR) was recorded. Results: Mean age of the included patients was 41±11.69 with range from 8 to 82 years, while 217 (43.4 %) patients were male and 283 (56.6 %) were female. Cirrhosis was present in 59 (11.8 %) patients; among these 35.6 % were in Child A and 64.4 % in early Child B. End of treatment response occurred in 491 (98.2 %) patients and there was no significant difference in ETR between male and female patients, and between cirrhotic and non-cirrhotic. Similarly, there was no significant difference in age between those having ETR and those having no ETR. Fatigue was experienced by 13.2 % and headache by 4.2 % patients. Conclusion: The combination of sofosbuvir and daclatasvir has high response rate in chronic hepatitis C patients of our population.

Outcome of Conservative Therapy in Coronavirus disease-2019 Patients Presenting With Gastrointestinal Bleeding

Background/ObjectiveThere is a paucity of data on the management of gastrointestinal (GI) bleeding in patients with Coronavirus disease -2019 (COVID-19) amid concerns about the risk of transmission during endoscopic procedures. We aimed to study the outcomes of conservative treatment for GI bleeding in patients with COVID-19.MethodsIn this retrospective analysis, 24 of 1342 (1.8%) patients with COVID-19, presenting with GI bleeding from 22nd April to 22nd July 2020, were included.ResultsThe mean age of patients was 45.8 ± 12.7 years; 17 (70.8%) were males; upper GI (UGI) bleeding: lower GI (LGI) 23:1. Twenty-two (91.6%) patients had evidence of cirrhosis- 21 presented with UGI bleeding while one had bleeding from hemorrhoids. Two patients without cirrhosis were presumed to have non-variceal bleeding. The medical therapy for UGI bleeding included vasoconstrictors-somatostatin in 17 (73.9%) and terlipressin in 4 (17.4%) patients. All patients with UGI bleeding received proton pump inhibitors and antibiotics. Packed red blood cells (PRBCs), fresh frozen plasma (FFPs) and platelets were transfused in 14 (60.9%), 3 (13.0%) and 3 (13.0%), respectively. The median PRBCs transfused was 1 (0–3) unit(s). The initial control of UGI bleeding was achieved in all 23 patients and none required an emergency endoscopy. At 5-day follow-up, none rebled or died. Two patients later rebled, one had intermittent bleed due to gastric antral vascular ectasia, while another had rebleed 19 days after discharge. Three (12.5%) cirrhosis patients succumbed to acute hypoxemic respiratory failure during hospital stay.ConclusionConservative management strategies including pharmacotherapy, restrictive transfusion strategy, and close hemodynamic monitoring can successfully manage GI bleeding in COVID-19 patients and reduce need for urgent endoscopy. The decision for proceeding with endoscopy should be taken by a multidisciplinary team after consideration of the patient's condition, response to treatment, resources and the risks involved, on a case to case basis.

BYPASSING THE UREA CYCLE IN THE ICU

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Hyperammonemic encephalopathy is a rare, yet devastating complication of Rou-en-Y gastric bypass surgery. Though the mechanisms which underlie this complication are not completely understood, a partial underlying inborn error of metabolism has been proposed. In this case we describe a patient who presented with worsening altered mental status and ultimately improved after empiric treatment for a partial urea cycle disorder. CASE PRESENTATION: A 45 year-old female with a history of Roux-en-Y gastric bypass and recently diagnosed bilateral pulmonary emboli presented with altered mental status. One day prior to admission, she became lethargic and ultimately unresponsive, prompting her family to bring her to the hospital. She had 60 lbs of weight loss in the last 2 months, which her family attributed to poor oral intake. In the last 6 months, she had multiple episodes of dizziness, falls, and syncope as well as multiple admissions for nausea, intractable vomiting, and abdominal pain. On presentation, the patient was hemodynamically stable.Abdominal ultrasound revealed changes consistent with steatosis without evidence of cirrhosis. Laboratory results were significant for elevated ammonia (169 µmol/L) and INR (1.7). The patient was started on lactulose and rifaximin for suspected hepatic encephalopathy. After three days of therapy, the patient’s mental status declined and ammonia levels had increased to 192 µmol/L. She was intubated and started on continuous renal replacement therapy (CRRT) for ammonia clearance. Mental status improved. However, cessation of CRRT resulted in increased ammonia levels and worsening mental status. Further laboratory studies revealed deficiencies in zinc, selenium, and copper. Given the refractory hyperammonemia, encephalopathy, and history of gastric bypass surgery, the patient was started on treatment for a suspected partial urea cycle disorder, including arginine and sodium benzoate, as well as supplementation of zinc and copper, resulting in improvement of ammonia levels and mental status. Ultimately, the patient was discharged to an outside facility. DISCUSSION: Encephalopathy in the ICU patient is a frequent occurrence, and may be due to a myriad of metabolic derangements. Inborn errors of metabolism are not common in adult ICU patients, but should be considered in those with a history of gastric bypass surgery presenting with refractory hyperammonemia. As seen in our case, this deadly complication may be reversible with prompt initiation of empiric treatment. CONCLUSIONS: A rare complication of Rou-en Y gastric bypass surgery, hyperammonemic encephalopathy should be considered in patients with refractory hyperammonemia and a history of gastric bypass surgery. Reference #1: Fenves, A.Z., Shchelochkov, O.A. and Mehta, A. (2015), Hyperammonemic syndrome after Roux-en-Y gastric bypass. Obesity, 23: 746-749. doi:10.1002/oby.21037 Reference #2: Nagarur, A., & Fenves, A. Z. (2017). Late Presentation of Fatal Hyperammonemic Encephalopathy After Roux-En-Y Gastric Bypass. Baylor University Medical Center Proceedings, 30(1), 41–43. doi: 10.1080/08998280.2017.11929521 DISCLOSURES: No relevant relationships by Abigail Go, source=Web Response No relevant relationships by John Mwangi, source=Web Response No relevant relationships by Ara Vartanyan, source=Web Response

S1401 Malignant Mesenteric Venous Obstruction From Pancreatic Cancer, an Under-Recognized Condition Associated With Significant Morbidity, Can Be Treated With Venous Stenting

INTRODUCTION: Portal vein (PV) and superior mesenteric vein (SMV) stenosis can result from various pathologic processes including pancreatic cancer. Their clinical manifestations include portal hypertension (PH) with life-threatening variceal bleeding, refractory ascites, and mesenteric ischemia. In this abstract, 4 cases who underwent stenting are presented. CASE DESCRIPTION/METHODS: Case 1 is a 58-year-old woman with a history of pancreatic cancer status post Whipple resection, presented with worsening diarrhea, ascites, and malnutrition. Stool testing, colonoscopy with biopsies were negative. Ascitic fluid analysis was consistent with PH with no evidence of cirrhosis. Abdominal imaging showed ill-defined pancreatic body infiltrative mass with significant narrowing of the PV with resultant small bowel and colonic thickening. She underwent SMV/PV stenting which resulted in the resolution of diarrhea and bowel thickening. She went off total parenteral nutrition with 22 kg weight gain and was able to receive systemic anticancer therapy (SAT) and survived for 18 months afterward.Three other cases with similar preservations and varying degrees of refractory ascites, diarrhea, malnutrition, and bowel wall thickening on imaging. They all underwent PV or SMV stenting. Case 2 is a 68-year-old woman who experienced relief of symptoms but elected hospice care and passed away a month later, after developing SMV stent thrombosis despite oral anticoagulation. Case 3 is a 58-year-old woman who had PV and SMV stenoses. She developed refractory bleeding immediately after stenting and consequently died. Case 4 is a 66-year-old man who achieved symptoms resolution. His symptoms recurred due to stent thrombosis 6 weeks later. After thrombectomy, he improved and was alive at 4 months, on aspirin, enoxaparin, and SAT, with no recurrence of symptoms. DISCUSSION: PV and SMV stenosis caused by pancreatic cancer can result in varying degrees of bowel ischemia, malnutrition, refractory ascites and often leads to death. It is often unrecognized and clinical findings are attributed to the cancer itself. PV/SMV stenting is possible and can reverse PH, ascites, bowel ischemia, and malnutrition. Anticoagulation can decrease the risk of venous thrombosis associated with stenting but coexistent PH presents a challenge to anticoagulation. Further studies are needed to determine the targeted population that will benefit from stenting and when to start anticoagulation or antiplatelet therapy.Figure 1.: A) Abdominal CT scan showing PV stenosis B) PV stent placement process. Contrast failed to pass through the narrowed area.Figure 2.: C) Stent is successfully placed. D) Abdominal CT scan status post PV stent placement.

S2543 Polycystic Liver Disease, Portal Hypertension, and Refractory Ascites: Is PLD the Cause?

INTRODUCTION: Polycystic liver disease (PLD) is a rare genetic disorder typically characterized by the presence of more than twenty hepatic cysts. The prevalence of autosomal dominant polycystic kidney disease (ADPKD) associated PLD is 0.2%. We present an unusual case of PLD associated with refractory ascites in the absence of liver fibrosis or hepatic venous outflow obstruction. CASE DESCRIPTION/METHODS: This is a 54-year-old female with a history of ADPKD status post kidney transplant without evidence of cirrhosis or any decompensating events pre-transplant. Her post-transplant course was complicated by cecal and mesenteric volvulus status post right hemicolectomy. Roughly five weeks later, she developed severe ascites. Initial lab evaluation revealed no hepatocellular dysfunction. Diagnostic paracentesis indicated a SAAG of 1.4, ascitic total protein 2.1 and no evidence of SBP. Portal package showed abnormally tortuous venous vasculature. Right hepatic vein was difficult to wedge but measured 70-80 mm Hg; free right hepatic vein pressure was 5 mm Hg, IVC pressure was 4 mm Hg and a right atrial pressure was 5 mm Hg; overall, findings were consistent with portal hypertension. Liver biopsy showed no significant fibrosis or evidence of congenital hepatic fibrosis, but did show mild sinusoidal dilatation, potentially indicative of a vascular outflow abnormality. An abdominal CT was without evidence of extrinsic compression of the large hepatic veins. Due to anatomic difficulties, TIPS was not technically feasible, so she had a Denver shunt placed for refractory ascites with good control thereafter. DISCUSSION: Portal hypertension and ascites in the presence of PLD typically arises from three etiologies: liver cirrhosis, congenital hepatic fibrosis and hepatic venous outflow obstruction. This patient had no imaging to support large hepatic venous outflow obstruction, but given her histologic findings it is possible that her presentation is due to cystic compression of small venules, which cannot be visualized on cross sectional imaging. However, there is not any literature to support hepatic venule compression leading to portal hypertension. Due to chronic injury from elevated outflow pressures, these patients can develop fibrotic replacement of the hepatic parenchyma and may ultimately develop cirrhosis. Recognition and surveillance of these patients is necessary, as liver transplant may need to be considered if progressive cirrhosis complications become apparent.

Cystatin C and Sarcopenia Predict Acute on Chronic Liver Failure Development and Mortality in Patients on the Liver Transplant Waiting List.

Cystatin C (CysC) is an early biomarker of renal dysfunction scarcely studied in patients awaiting liver transplantation (LT). Sarcopenia is frequent in cirrhosis and impacts prognosis. We aimed to assess the capability of these factors to predict survival and acute-on-chronic liver failure (ACLF) in patients awaiting LT, as well as early post-LT outcomes. Single-center study that included all cirrhotic patients listed for LT between 2014 and 2017. Competing risk regression analysis was used to evaluate the capability of liver-, kidney-, and global status-related variables at waitlist (WL) inclusion to predict WL mortality and ACLF. Variables associated with post-LT outcomes were evaluated with logistic regression analysis. One-hundred-and-eighty patients were included. Fifty-six (31%) patients developed ACLF, 54 (30%) underwent LT and 35 (19%) died. In the adjusted competing risk regression analysis, CysC ≥ 1.5 mg/L, sarcopenia and MELD-Na were independent predictors of ACLF in the WL, while CysC ≥ 1.5 mg/L, sarcopenia and albumin were independent predictors of mortality. The cumulative incidence of ACLF and mortality at 12 months were 50% and 34% in patients with sarcopenia and CysC ≥1.5 mg/L. An estimated glomerular filtration rate by chronic kidney disease (CKD)-EPI-CysC-creatinine <60 mL/min/1.73 m at WL inclusion was an independent predictor of the need for renal replacement therapy (RRT) in the first month post-LT. Higher levels of CysC and sarcopenia are strongly associated with the ACLF and mortality in WL. The assessment of both risk factors may improve the prognostic evaluation and allow identifying a group of patients with a very high risk of poor outcomes while awaiting LT.

Prevalence of Nonalcoholic Fatty liver Disease (NAFLD) and its association with Cardio-metabolic risk factors in Type 2 Diabetes Mellitus

Aim/Background: Type 2 diabetes mellitus (T2DM) and Nonalcoholic fatty liver disease (NAFLD) have risen globally to epidemic proportions. The study was aimed to determine the prevalence of NAFLD and its association with cardio-metabolic risk factors in T2DM subjects. Method: In a case-control study, 212 consecutive, T2DM subjects with age ≥ 30 years, were evaluated from December 2017 to December 2018 at Mahatma Gandhi Medical College & Hospital, Jaipur, Rajasthan. Subjects with a history of significant alcohol consumption, evidence of cirrhosis, hepatotoxic drugs, and other known causes of fatty liver were excluded. The T2DM subjects were divided into (1) NAFLD - patients with USG evidence of fatty changes in the liver (2) Non-NAFLD – patients without any USG evidence of fatty changes in the liver. Coronary artery disease was screened by any past medical history of CAD or electrocardiographic or angiography evaluation. Continuous variables were expressed as mean with standard deviation. Comparison of continuous data between subgroups was done by using Independent student’s t-test or Mann Whitney U test. All statistical analysis was carried out by SPSS version 25. Result: 55.66% diabetics had comorbid NAFLD. Both study groups had comparable mean age (p 0.719) and gender distribution (p 0.482). Subjects with NAFLD had significantly higher BMI (p 0.0001), mean waist circumference (p <0.001), waist hip ratio (p <0.001), systolic BP (p 0.001), diastolic BP (p 0.024), HbA1c (p 0.021), total cholesterol (p 0.0426) & triglyceride (p 0.02). Mean LDL (p 0.054), VLDL (p 0.235) & HDL (p 0.113) values were comparable in two study groups, suggesting no significant association with NAFLD. 32 (15.09%) subjects had coronary artery disease (CAD). Diabetics with NAFLD had significantly higher CAD (22.03% vs 9.57%, p 0.02). Conclusion: Diabetics with NAFLD had significantly higher cardio-metabolic risk factors, leading to the increased associated risk of CAD.

Efficacy and safety of daclatasvir-containing regimens in the treatment of chronic hepatitis C patients in Damietta

Background Chronic hepatitis C virus infection is one of the most important health problems in Egypt. Since November 2015, generic sofosbuvir (SOF) with daclatasvir (DCV)±ribavirin (RBV) became the standard treatment in all National Committee for Control of Viral Hepatitis centers. Yet, the long-term efficacy, safety, and late complications of generic direct-acting antiviral drugs have not been studied. Aims Evaluation of the efficacy and safety of generic DCV-containing regimens in the treatment of chronic hepatitis C patients after 72 weeks of close follow-up. Patients and methods A total of 150 chronic hepatitis C virus patients were classified into easy-to-treat group (naive patients with no evidence of cirrhosis) and difficult-to-treat group (treatment experienced or have any evidence of cirrhosis) and treated for 12 weeks with generic medications: DCV 60 mg and SOF 400 mg±RBV in easy and difficult-to-treat groups, respectively. A close follow-up of up to 72 weeks was done to assess the efficacy and safety of the generic drugs. Results Overall sustained virologic response (SVR12) was 94.0%, while the end of treatment response rate was 94.7%. SVR12 rates in easy-to-treat and difficult-to-treat groups were 97.06 and 91.46%, respectively. Multivariate analysis revealed that absence of cirrhosis, higher baseline albumin, lower baseline international normalized ratio, higher baseline platelet count, lower baseline alpha-fetoprotein, normal splenic size, and naivety to previous antiviral therapy were associated with SVR12. PCR after 72 weeks for those who achieve SVR12 is still undetectable. Three cases died during the follow-up period and were related to their advanced liver disease. No serious adverse events were reported. Conclusion Generic DCV, SOF±RBV regimen is safe and effective for treatment of chronic hepatitis C with minimal adverse events and SVR12 is efficient to predict persistent viral clearance.

2351 NAC of All Trades: Use of N-acetylcysteine in Cocaine-Induced Acute Liver Failure

INTRODUCTION: Among patients with acute liver failure (ALF), drug-induced liver injury (DILI) presents a challenging clinical syndrome with limited therapeutic options. N-acetylcysteine (NAC) is a mainstay of treatment in acetaminophen-induced ALF, acting as an antioxidant, a vasodilator and a glutathione precursor. However, it has been used with success in other toxic ingestions causing ALF, and its beneficial effects do not seem to be unique to acetaminophen-induced hepatotoxicity. We present a case of ALF after acute cocaine ingestion with rapid recovery after administration of IV NAC. CASE DESCRIPTION/METHODS: A 58 year-old-male with a past medical history of chronic hepatitis C without cirrhosis and polysubstance abuse presented with acute psychosis. On arrival he was tachycardic but hemodynamically stable, with psychomotor agitation and fear delusions. He endorsed recent heavy cocaine ingestion. Initial labs demonstrated acute kidney injury, rhabdomyolysis and mild transaminitis; AST 59, ALT 56. Toxicology workup was positive for cocaine with undetectable acetaminophen, ethanol and salicylate levels. The following day however, he developed fulminant hepatic failure, AST &gt;7000, ALT 2,568 (Figure 1) with progressive coagulopathy, INR 2.14, albumin 2.9, platelets 120. Of note, despite chronic hepatitis C, there was no evidence of cirrhosis and preserved liver synthetic function at presentation, INR 1.10, albumin 4.7, platelets 254. Our patient was not a candidate for liver transplant (LT), and decision was made to initiate IV NAC therapy, 140 mg/kg bolus followed by 70 mg/kg daily. Transaminases rapidly normalized after only 3 days of therapy, and he was discharged in stable condition. DISCUSSION: ALF is a rare but challenging syndrome, for which LT is the only curative treatment available. NAC is well established as an antidote in acetaminophen ALF, yet its mechanisms are applicable to other forms of DILI as well. Cocaine hepatotoxicity has been reported in humans, and is well studied in animal models. Studies in mice suggest toxic metabolites play a critical role, much like acetaminophen ALF. These species deplete glutathione, decrease hepatic blood flow, generate reactive oxygen species and cause lipid peroxidation of membranes. We present a case of ALF secondary to cocaine ingestion in the setting of underlying chronic hepatitis C, with rapid recovery after fluid resuscitation and IV NAC. NAC therapy has minimal adverse effects, and may be lifesaving in patients with ALF who are not candidates for LT.

2332 Large Hepatic Arteriovenous Fistula Leading to Non-Cirrhotic Portal Hypertension and Associated Complications

INTRODUCTION: Portal hypertension (and its complications including bleeding, ascites, encephalopathy) often occur in patients with liver cirrhosis as a result of intrahepatic resistance to portal blood flow and increased portal collateral blood flow. Non-cirrhotic portal hypertension (NCPH) is less common, but can also lead to similar complications. Arteriovenous fistulas are an uncommon presinusoidal intrahepatic cause of NCPH. We present a rare case of a patient with a remote history of a liver biopsy who presented with new onset ascites and found on imaging to have a large hepatic arteriovenous fistula which was subsequently treated with embolization. CASE DESCRIPTION/METHODS: A 61 year old male with no significant past medical history presented to clinic for evaluation of progressively worsening abdominal and lower extremity swelling. Laboratory findings on presentation were remarkable for hemoglobin 7.4 g/dL, platelets 59,000/microliter, INR 1.6, Na 137mmol/L, Cr 1.56 mg/dL T bilirubin 2.4 mg/dL. Paracentesis fluid studies showed high serum-ascites albumin gradient (SAAG) and high protein without evidence of heart failure on echocardiogram. Magnetic resonance imaging of the abdomen showed a 6.3 × 4 centimeters hepatic arteriovenous fistula within segment VIII of the liver; the portal vein was noted to be patent. Liver biopsy showed focal perivenular and subsinusoidal fibrosis without bridging (fibrosis stage 1/6) and nodular regenerative hyperplasia changes with bands of hepatocyte atrophy without any evidence of cirrhosis. Right hepatic arteriogram demonstrated a dilated and tortuous vessel with early filling of the portal venous system and a large aneurysm at the communication point between the arterial and venous fistula. Successful embolization of the anterior segmental branch of the right hepatic artery was achieved. The patient’s hospital course was also complicated by upper gastrointestinal bleeding secondary to esophageal varices which required banding x5. DISCUSSION: The presence of portal hypertensive changes with minimal hepatic fibrosis on liver biopsy is best categorized as non-cirrhotic portal hypertension (NCPH). This patient manifested complications of portal hypertension including variceal bleeding and recurrent ascites secondary to a large hepatic arteriovenous fistula which likely formed following his liver biopsy. Closure of large arteriovenous fistulas can be beneficial in preventing worsening portal hypertension and associated potentially fatal complications.

1357 Pseudocirrhosis in Subacute/Chronic Budd-Chiari Syndrome With JAK 2 Mutation

INTRODUCTION: Budd Chiari Syndrome (BCS) is a rare disorder often characterized by hepatic venous outflow obstruction. Myeloproliferative disorders especially V617F-JAK2 mutations account for almost 40% of these patients. BCS may present differently depending on location, extent, severity of obstruction and existence of collaterals. The course of disease may be fulminant, acute, subacute or chronic, ranging from incidental findings to fatal acute hepatic failure. CASE DESCRIPTION/METHODS: A 74-year-old female with remote history of alcoholism, presented to our facility with bleeding per rectum for 2 days. Labs on admission revealed WBC 18.4, HGB 16.8, PLT 588, AST 570, ALT 804 and TB 4.6. Ultrasound Abdomen with Doppler revealed ascites and portal vein thrombosis. Follow-up MRI Abdomen confirmed thrombus in main portal vein and non-opacification of major intrahepatic veins suggesting Budd Chiari Syndrome. Hypercoagulability workup showed positive JAK2 mutation. She was started on IV Heparin immediately. Diagnostic paracentesis revealed transudative fluid and liver elastography confirmed cirrhosis. Grade 2 esophageal varices were seen on EGD with a negative colonoscopy. Other possible etiology of congestive hepatopathy/cirrhosis were excluded. Patient was discharged on daily oral Rivaroxaban with intermittent therapeutic paracentesis. Over next 3 months, she had recurrent admissions for decompensated liver disease. During her last visit however, we noted preserved synthetic liver function and inconsistencies in her presentation thus a liver biopsy was obtained which revealed centrilobular hepatocellular necrosis, sinusoidal dilatation and mild portal fibrosis consistent with hepatic venous outflow obstruction. However, there was no evidence of cirrhosis. She was transferred to an advanced facility for portal vein reconstruction to relieve the obstruction from splanchnic venous thrombosis and possibly reverse the “pseudocirrhosis”. DISCUSSION: Pseudocirrhosis is often associated with metastatic cancers in response to chemotherapy induced ischemic injury. Although it radiologically and clinically mimics liver cirrhosis, it can be prevented with early detection. To our knowledge, this is the first reported case of pseudocirrhosis in BCS. Subacute/chronic BCS may present with cirrhotic complications like portal hypertension, ascites and variceal bleeding. Apart from anticoagulation, these patients may require other interventions to bypass the obstruction like Direct Intrahepatic Portosystemic Shunt (DIPS) placement.

2431 Legionella Pneumonia Masquerading as Acute Hepatitis and Rhabdomyolysis

INTRODUCTION: Legionella pneumonia commonly presents with myalgia, fatigue, and dyspnea and accounts to about 10-15% of all pneumonia cases. With less than 100 unusual cases of Legionella reported, this is a unique presentation with fulminant hepatic failure, acute kidney failure, and rhabdomyolysis. CASE DESCRIPTION/METHODS: 52-year-old African-American male with no medical history presented to the emergency room with nausea, diarrhea, and fatigue. He was febrile and tachycardic on presentation. On further evaluation, he reported exposure to a new air-conditioner at work. Chest x-ray (CXR) revealed a right lower lobe pneumonia. Blood work was remarkable for elevated liver enzymes, hyponatremia, acute kidney injury, and elevated creatinine phosphokinase (CPK). His urine drug screen and alcohol level was normal. Liver ultrasound (US) showed fatty infiltration, but no cirrhosis, confirming his social history of being non-alcoholic. Hepatitis (A, B and C), Cytomegalovirus (CMV) IgM and HIV serologies were all negative. Patient was started on fluid resuscitation and antibiotics. Despite a lobar consolidation, Legionella urine antigen returned positive. This confirmed Legionella induced fulminant liver failure and rhabdomyolysis. Patient later developed respiratory symptoms and his repeat Chest x-ray revealed patchy infiltrates in multiple regions, consistent with Legionella. His liver enzymes and CPK trended down, and he was discharged home. He was followed as outpatient with resolution of the fatty infiltration on the Liver US. DISCUSSION: Legionella is transmitted through inhalation of aerosols derived from water or soil. It mimics alcoholic hepatitis and is distinguished from other types of hepatitis by having no evidence of cirrhosis. Presentation with gastro-intestinal symptoms, consolidation on imaging, rhabdomyolysis, and acute kidney injury makes Legionella hepatitis most likely etiology. Exact mechanism is still unclear; however, release of endotoxins and bacterial invasion have been reported in many studies. Diagnosis is made with rapid PCR or urine antigen. Levofloxacin or Azithromycin are the preferred antibiotics as these agents are bactericidal, achieve high intracellular concentrations, penetrate lung tissue, and are active against all sub-type species. Treatment usually is for 7-10 days but for immunocompromised patients is 14 days due to risk of re-infection and relapse. Legionella should be high in the differential with the other hepatitis causing diseases like HIV, Hepatitis A, B or C and CMV.

2295 Shunt Hunting: A Case of Long-Standing Encephalopathy Secondary to Anatomic Porto-Systemic Shunt

INTRODUCTION: Abernethy malformation is a very rare congenital vascular malformation defined by diversion of portal blood away from liver [1]. Morgen and Superina classified extrahepatic portosystemic shunt into 2 types, as seen in Table 1 [3]. We describe in this case a type II extrahepatic portosystemic shunt. CASE DESCRIPTION/METHODS: A 67-year-old female with history of untreated hepatitis C, major depression disorder, hypertension, peripheral vascular disease, GERD, rheumatoid arthritis and HFrEF was admitted for altered mental status and elevated ammonia level on outpatient labs. This patient has a longstanding history of unexplained encephalopathy and elevated ammonia, treated with lactulose. She has no known history of cirrhosis and denied fevers, chills, nausea, vomiting or GI bleed. Patient was hypertensive on admission at 173/89, tachycardic to 109 and afebrile. She was saturating 98% on 2L O2. Exam was significant for bilateral coarse crackles, RUQ and epigastric tenderness and JVD. Ammonia was 106 on admission. Alkaline phosphatase 112, ALT 39, AST 84. Abdominal ultrasound revealed mildly heterogenous echogenicity of the liver with no evidence of cirrhosis. CT Abdomen was significant for congenital abnormalities including intestinal malrotation, hypoplastic IVC and abnormal pelvic variceal communication with the internal iliac vein, splenic vein and portal venous system (image). Patient underwent transjugular biopsy of liver with hepatic wedge pressures measured at 29 mmHg (gradient 14 mmHg). Pathology showed bridging fibrosis consistent with chronic viral hepatitis infection. Her encephalopathy improved to baseline after treatment with lactulose. DISCUSSION: We present a case of long-standing encephalopathy without cirrhosis, treated with lactulose. The patient also had markedly elevated hepatic portal pressures in the setting of HFrEF, without findings of cirrhosis on pathology. On this admission, CT abdomen/pelvis revealed an anatomic porto-systemic shunt, revealing the etiology of the patient’s unexplained “hepatic” encephalopathy. Multiple intra-abdominal congenital abnormalities were found on CT abdomen/pelvis, including a hypoplastic IVC, intestinal malrotation, and most significantly abnormal variceal communication with the internal iliac vein, splenic vein and portal venous system. The patient's collateral portosystemic shunts describes a type II extrahepatic portosystemic shunt.

A RARE CASE OF EPSTEIN BARR VIRAL MYOCARDITIS-INDUCED CARDIOGENIC SHOCK

SESSION TITLE: Case Report Semifinalists 8 SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/21/2019 02:30 PM - 03:15 PM INTRODUCTION: Epstein barr virus (EBV) is a ubiquitous virus whose clinical association ranges from infectious mononucleosis to lymphomas and nasopharyngeal carcinomas. We present a case of EBV associated myocarditis, hepatitis, disseminated intravascular coagulation (DIC) and multiple system organ failure (MSOF). CASE PRESENTATION: 63-year-old man presented with 1 week of chest pain and dyspnea to our Emergency Room. On initial examination, the patient was alert and oriented, with mild respiratory distress. EKG showed low voltage QRS and diffuse ST depression. CXR showed mild interstitial edema. Serum chemistries showed Hemoglobin 4, White count 0.92, platelet count of 72,000 , Creatinine 2.4, BUN 56, high sensitivity troponin 2665 ng/L, proBNP 29,766 pg/ml, AST 1435, ALT 738, Total bilirubin 17.3, GGTP 26. Hepatobiliary imaging was without evidence of cirrhosis, it did show cholelithiasis, however the patient did not have clinical evidence of acute biliary pathology. He rapidly deteriorated and developed acute hypoxic respiratory failure and encephalopathy requiring admission to the ICU. He was intubated and mechanically ventilated. Point of Care Ultrasound showed diffuse biventricular hypokinesis. His CXR showed worsening pulmonary edema. He was initiated on dobutamine and Lasix infusions. He was also noted to be oozing from all IV sites and his work up was consistent with DIC. He received blood products for correction of coagulopathy. The clinical suspicion for an acute coronary syndrome was low, and he was considered a poor candidate for cardiac catheterization given DIC and renal failure. High dose steroids were initiated for suspicion for myocarditis. Complete infectious workup revealed EBV DNA by PCR. Given profound pancytopenia a bone marrow biopsy was performed which showed reactive plasmacytosis, no blast cells and few histiocytes engulfing red cells. While Hemophagocytic lymphohistiocytosis was considered based on his bone marrow biopsy results, further cytological studies revealed 20q chromosomal deletion consistent with myelodysplastic syndrome (MDS). The patient responded to therapy and was successfully extubated, dobutamine infusion was weaned off and renal function recovered. Unfortunately the patient developed multiple embolic infarcts. Patient and his family opted for discharge to hospice where he died one week later. DISCUSSION: EBV related myocarditis and MSOF represents a rare, potentially fatal complication of a usually benign, self-limiting disease. In our patient, acute EBV infection may have been reactivated in the setting of immunocompromise, however the possibility that chronic EBV infection played a role in the development of MDS cannot be excluded. CONCLUSIONS: EBV is a rare cause of myocarditis and MSOF. Appreciation of the potential virulence of the pathogen, prompt diagnosis and better therapeutics are necessary for management of this fatal presentation of EBV. Reference #1: Angelini, A., Calzolari, V., Boffa, G., Maddalena, F., Chioin, R., & Thiene, G. (2000). Myocarditis mimicking acute myocardial infarction: role of endomyocardial biopsy in the differential diagnosis. Heart (84), 245-250. Reference #2: Miller, R., Ward, C., Amsterdam, E., Mason, D., & Zelis, R. (1973). Focal mononucleosis myopericarditis simulating myocardial infarction. . Chest (63), 102-105. Reference #3: Sabbatini, S., Manfredi, R., Ortolani, P., Trapani, F., & Viale, P. (2012). Myopericarditis during a primary Epstein-Barr virus infection in an otherwise healthy young adult. An unusual and insidious complication. Case report and a 60 year literature review. Le Infezioni in Medicina (2), 75-81. DISCLOSURES: No relevant relationships by Shaeesta Khan, source=Web Response No relevant relationships by Ritika Paul, source=Web Response

3182 Spontaneous Bacterial Peritonitis in Cardiac Ascites: A Rare but Deadly Occurrence

INTRODUCTION: Spontaneous bacterial peritonitis (SBP) is frequently described in cirrhotic patients who develop infected ascitic fluid. The phenomenon of SBP in cardiac ascites is rare. Here we present a unique case of a patient who was admitted for advanced cardiorenal syndrome in the setting of a gastroenteritis that likely promoted a bacterial translocation resulting in SBP. CASE DESCRIPTION/METHODS: An 85M presented from a nursing facility with lethargy, confusion and non-bloody diarrhea. Medical history was significant for congestive heart failure (CHF) and chronic kidney disease. No history of alcohol use. Upon arrival to the hospital, he was noted to be confused with a mildly distended abdomen, asterixis and two plus lower extremity pitting edema. The patient was admitted for a CHF exacerbation and metabolic encephalopathy. Initial labs revealed a BNP 3915, WBC 11,500, BUN 89, creatinine 3.5, total bilirubin 2, AST 22, ALT 9, and INR 1.27. An abdominal CT scan showed a moderate amount of intraperitoneal fluid. A diagnostic paracentesis was performed. The aspirated fluid was dark yellow and opaque with a serum ascites albumin gradient of 1.9 g/dL, total fluid protein 3.3 g/dL, 1,115 PMNs cells/mm3, 49% polymorphonuclear leukocytes. To much surprise, these findings demonstrated cardiac ascites resulting in SBP. There was no evidence of cirrhosis. Antibiotic treatment was initiated. Unfortunately, the patient exhibited a rapid decline renal function, and expired shortly afterwards. DISCUSSION: Due to the liver's extensive vascular supply it is prone to hemodynamic disturbances which can result in passive hepatic congestion from right sided heart failure. Recent studies have supported the diagnostic utility of serum BNP levels to distinguish ascites from cirrhosis or heart failure. It has been generally well accepted that the low occurrence of SBP in cardiac ascites may be related to the protein rich ascitic fluid that has both opsonic and bactericidal activity. Interestingly, the “gut hypothesis” suggests that intestinal hypoperfusion and congestion can lead to altered gut morphology, permeability, and the growth of microbiota that may ultimately stimulate bacterial translocation and endotoxin release. This case tends to shed light on a few quintessential points for clinicians to be aware of, including the potential intersection between the microbiota and metabolic effects of CHF and the necessity to lower the diagnostic threshold for SBP cardiac ascites in patient's presenting for a CHF exacerbation.

Abstract 5046: Gallbladder disease and the risk of hepatocellular carcinoma: US case-control study

Abstract BACKGROUND: Hepatocellular carcinoma (HCC) is the most common primary cancer of the liver with a five-year survival rate of less than 5 percent. Gallbladder disease (GBD) is defined as the presence of cholelithiasis or cholecystitis. Both cholelithiasis and cholecystitis have been reported as risk factors in the development of gastrointestinal cancers, such as biliary tract and pancreatic cancers. Moreover, patients with GBD tend to develop chronic liver diseases, which may progress to cirrhosis. However, the role of GBD in the development of non-cirrhotic related HCC has not been elucidated. We aimed to investigate the role of GBD and the risk of HCC development in the absence of cirrhosis. METHODS: We conducted a case-control study at The University of Texas M.D. Anderson Cancer Center. Cases were defined as pathologically confirmed HCC. Controls were healthy individuals without prior history of cancer, who were spouses of other cancer patients seen at MD Anderson. All subjects were USA residents. Participants were personally interviewed for prior history of GBD and for several HCC risk factors (environmental, behavioral, chronic medical conditions, and family history of cancer). Blood samples of all participants were tested for markers of hepatitis B and C viruses. We reviewed the pathology and radiology records of HCC patients to assess presence and absence of cirrhosis. Multivariable logistic regression analysis was conducted to estimate the adjusted odds ratio (AOR) and 95% confidence interval (CI) for the association between GBD and HCC in the absence of cirrhosis and with adjustment for potential confounders. RESULTS: Between 2000 and 2017, a total of 1333 cases and 1104 controls were enrolled in the study. After review, 347 HCC case patients showed no evidence of cirrhosis and were eligible for this analysis. The prevalence of GBD based on the participants’ recall during personal interview was 22.7% in the cases. Upon further assessment by review of medical records, the prevalence of GBD was 40.3% in the cases. Individuals with GBD were two times more likely to develop HCC than individuals with no history of GBD (AOR = 2.0; 95% CI: 1.4 – 2.8). The estimated AOR did not meaningfully change when we rely on the prevalence of GBD recalled by cases (22.7%) versus prevalence of GBD obtained from medical records (40.3%). The association between GBD and HCC continue to be significant in the absence of viral hepatitis, through a restricted analysis among non-viral non-cirrhotic population after controlling for age, sex race, alcohol use, cigarette smoking, diabetes mellitus, and family history of cancer. CONCLUSIONS: We conclude that GBD is a significant risk factor for HCC development in absence of cirrhosis. Future research aiming at investigating the underlying mechanism of GBD-induced HCC in absence of cirrhosis should be warranted. In addition, patients with GBD should be screened for evidence of cirrhosis. Citation Format: Kenda Al-Assi, Rikita Hatia, Vijayashri Rallapalli, Yehia I. Abugabal, Ahmed Abdelhakeem, Reham Abdel-Wahab, Kanwal Raghav, Prasun K. Jalal, Ahmed Kaseb, Asif Rashid, Donghui Li, Manal Hassan. Gallbladder disease and the risk of hepatocellular carcinoma: US case-control study [abstract]. In: Proceedings of the American Association for Cancer Research Annual Meeting 2019; 2019 Mar 29-Apr 3; Atlanta, GA. Philadelphia (PA): AACR; Cancer Res 2019;79(13 Suppl):Abstract nr 5046.

Predictors and Cumulative Frequency of Hepatocellular Carcinoma in High and Intermediate LI-RADS Lesions: A Cohort Study from a Canadian Academic Institution.

The frequency and predictors of hepatocellular carcinoma (HCC) within each liver imaging reporting and data system (LI-RADS) category remains unclear. We sought to estimate the cumulative frequency of HCC in LI-RADS observations of high/intermediate category and identify clinical/radiographic features associated with HCC. Our diagnostic imaging database was searched for computed tomography/magnetic resonance imaging reports of patients with evidence of cirrhosis and liver observations. LI-RADS categories were determined by imaging review, while demographic and clinical outcomes were assigned by chart review. A composite outcome of clinical/radiographic confirmation of HCC was used. We used multivariable analysis to identify features associated with HCC, and competing risks regression to estimate the cumulative frequency of HCC in each category. Our search returned 95 patients with 137 observations (LR2 = 4, LR3 = 53, LR4 = 37, and LR5 = 43). On multivariable analysis, increasing age (hazard ratio [HR] 1.76 per 10years, p = 0.049), washout (HR 5.34, p < 0.002), and increasing size (size < 10mm reference, 10-20mm, HR 3.93, p = 0.014; size > 20mm, HR 21.69, p < 0.001) were associated with HCC. Median time to diagnosis was 6.13months (interquartile range [IQR] 4.6-13.1), 4.7months (IQR 2.5-14.5), and 3.6months (IQR 1.9-6.6) for LR3, 4, and 5 category observations, respectively. The cumulative frequency of HCC was 59.8% in LR3, 84.62% in LR4, and 99.84% in LR5, at last follow-up. The frequency of HCC within each LI-RADS category reflects the intended purpose, intermediate probability for LR3, probable HCC for LR4, and definite HCC for LR5.