Evaluation For Liver Transplantation Research Articles

Favorable Survival with Non-curative Treatments for Patients with Early-Stage Hepatocellular Carcinoma After Liver Transplant Denial

Many patients are not candidates for liver transplant for non-tumor-related reasons including medical comorbidities and non-adherence. The prognosis of patients with hepatocellular carcinoma (HCC) who are not liver transplant candidates in the era of locoregional therapy (LRT) including y90 is not well defined. This study seeks to evaluate outcomes and the natural history of early-stage HCC in patients who were denied liver transplant listing due to non-tumor reasons and instead were treated with LRT. A retrospective evaluation was performed for all patients who completed liver transplant evaluation with their tumor within Milan criteria but were denied due to non-tumor reasons and were treated with LRT at a single tertiary referral center. The 61 patients included had a favorable overall survival, with a median survival 60.3months (86.9% at 1year and 52.7% at 5years). Patients with Child-Pugh A cirrhosis (n = 34) had significantly longer overall survival compared to those with Child-Pugh B/C cirrhosis (median survival of 70.3months versus 26.1months, p = 0.005). Survival in patients with Child-Pugh A at 1, 3, and 5years was 97%, 80%, and 73%, respectively, compared to 74%, 41%, and 31% in patients with Child-Pugh B/C. In a small single-center cohort, patients with HCC who were denied liver transplant due to non-tumor reasons and underwent LRT and had Child-Pugh A cirrhosis had survival approaching the national average for patients who undergo liver transplantation. Patients with Child-Pugh B/C cirrhosis had significantly worse outcomes than those with Child-Pugh A.

Progression to Cirrhosis Leads to Improvement in Atherogenic Milieu.

The prevalence of coronary artery disease (CAD) is high among patients with cirrhosis; however, the impact of it on cardiovascular disease (CVD) is not known. The aim of the current study was to evaluate CVD events in patients with cirrhosis and impact of cirrhosis on biomarkers of atherogenesis. The study included 682 patients with decompensated cirrhosis referred for liver transplantation (LT) evaluation between 2010 and 2017. All patients were followed until they experienced a CVD event, non-cardiac death, liver transplantation or last follow-up. To evaluate mechanistic link, patients with NASH cirrhosis were propensity matched 1:2 to non-cirrhosis NASH patients and biomarkers of atherogenic risk were compared. The composite CVD outcome occurred in 23(3.4%) patients after a median follow-up period of 585days (IQR 139, 747). A strong association between presence of any CAD and CVD event was noted (HR = 6.8, 95% CI 2.9, 15.9) that was independent of age, gender, BMI, and MELD score. In competing risk model, the combined rate of LT and non-cardiac was significantly higher when compared to the rate of CVD events. Marker of insulin resistance and inflammation-related markers were similar in patients with and without cirrhosis. Patients with cirrhosis were more likely to have reduced VLDL, sdLDL-C, LDL-C, and triglycerides. Interestingly, patients with cirrhosis had an increase in serum HDL-2, the anti-atherogenic lipoprotein, and adiponectin, a protective serum adipokine. The risk of CVD events in patients with cirrhosis is low and may potentially be due to improvement in markers of atherogenic risk.

Common cardiac comorbidities and perioperative assessment modalities for liver transplant candidates – an update

Cardiac complications are currently the leading cause of early mortality following liver transplantation. Guidelines for the cardiac workup prior liver transplantation are limited. In this review we are discussing commonly modalities used for cardiovascular evaluation of liver transplant candidates.Authors declare no conflict of interest.

Hepatocyte-Specific Magnetic Resonance Imaging-Based Assessment of Indeterminate Hepatic Nodules in the Liver Transplant Evaluation of Patients With Cirrhosis.

We aimed to determine the identities in explants of indeterminate hepatic nodules (IDNs) that had been scanned by dynamic magnetic resonance imaging (MRI) to establish clinicoradiological parameters predicting which IDNs were hepatocellular carcinomas (HCCs). This study included 88 patients with cirrhosis who underwent gadoxetic acid-enhanced MRI in pre-liver transplantation (LT) workup followed within 90days by primary LT. The MRI detected 168 hepatic nodules that were classified into 6 benign tumors, 49 HCCs, and 113 IDNs, in 5, 34, and 72 patients, respectively. We compared these pre-LT radiologic diagnoses and stagings with explant pathology on a per-lesion basis to enable us to identify features of IDNs related to malignancy. Of the 168 nodules seen on MRI, 119 that were classified radiologically as consisting of 1 benign nodule (33.3%), 46 HCCs (93.9%), and 72 IDNs (63.7%) all turned out to be pathological HCCs. Of 32 patients inside Milan and 54 without HCC staged by MRI, 11 progressed beyond the criteria after LT. High serum alpha-fetoprotein level (≥20ng/mL) was the only per-patient factor significantly associated with malignant IDNs. Per-tumor analysis of the MRI signals revealed that arterial hyperintensity, hepatobiliary hypointensity, T2 -weighted mild-to-moderate intensity, and restricted diffusion-weighted images were significantly correlated with malignant IDN. A model combining these 4 MRI factors with alpha-fetoprotein level had the best performance in predicting the identification of IDNs as HCCs in explanted livers. Over 60% of the IDNs seen on dynamic images of cirrhotic livers proved to be HCCs when explanted livers were examined. It may therefore be possible to identify HCCs with reasonable accuracy by means of their hepatocyte-specific MRI features when patients are being assessed for LT.

Spotlight on Impactful Research: Utilization of Aspirin and Statin in Management of Coronary Artery Disease in Patients With Cirrhosis Undergoing Liver Transplant Evaluation.

http://aasldpubs.onlinelibrary.wiley.com/hub/journal/10.1002/(ISSN)2046-2484/video/15-2-reading-frankul a video presentation of this article http://aasldpubs.onlinelibrary.wiley.com/hub/journal/10.1002/(ISSN)2046-2484/video/15-2-interview-frenette an interview with the author Answer questions and earn https://www.wileyhealthlearning.com/Activity/7036138/disclaimerspopup.aspx.

Non-selective beta blocker use is associated with improved short-term survival in patients with cirrhosis referred for liver transplantation

BackgroundRecent evidence cautions against the use of non-selective beta-blockers (NSBB) in patients with refractory ascites or spontaneous bacterial peritonitis while other data suggests a survival benefit in patients with advanced liver disease. The aim of this study was to describe the use and impact of NSBB in patients with cirrhosis referred for liver transplantation.MethodsA single-center cohort of patients with cirrhosis, who were referred and evaluated for liver transplantation between January and June 2012 were studied for baseline characteristics and clinical outcomes. Patients were grouped according to the use of NSBB at initial evaluation, with the endpoint of 90-day mortality.ResultsSixty-five (38%) of 170 consecutive patients evaluated for liver transplantation were taking NSBB. Patients taking NSBB had higher MELD and Child Pugh score. NSBB use was associated with lower 90-day mortality (6% vs. 15%) with a risk adjusted hazard ratio of 0.27 (95%CI .09–0.88, p = .03). Patients taking NSBB developed acute kidney injury (AKI) within 90 days more frequently than patients not taking NSBB (22% vs 11%), p = 0.048). However, this was related to increased stage 1 AKI episodes, all of which resolved. Twelve (27%) of 45 patients with > 90 day follow up discontinued NSBB, most commonly for hypotension and AKI, had increased subsequent MELD and mortality.ConclusionsNSBB use in patients with cirrhosis undergoing liver transplant evaluation is associated with better short-term survival. Nevertheless, ongoing tolerance of NSBB in this population is dynamic and may select a subset of patients with better hemodynamic reserve.

Not so innocent bystander - gallbladder varices without portal vein thrombosis

Introduction. Gallbladder varices (GBV) represent a rare form of ectopic varices that usually occur in patients with portal hypertension and portal vein thrombosis. Case outline. We present a case of a 38-year-old woman with decompensated autoimmune liver cirrhosis who was referred to our institution for evaluation for liver transplantation. She was incidentally discovered to have GBV during a routine B-mode abdominal ultrasonography as part of pre-transplant evaluation. GBV were confirmed by the Color Doppler Sonography, and multi detector computed tomography angiography. Interestingly, portal vein was patent and without thrombus. Conclusion. Despite being asymptomatic in most cases, the presence of GBV is valuable information for a surgeon because they might be a source of potentially catastrophic bleeding, which is particularly poorly tolerated by patients with decompensated liver cirrhosis. Ultrasound has the irreplaceable role not only in discovering GBV, but in prompt diagnosis of rare, but unpredictable and fatal complications as well.

Use of Telehealth Expedites Evaluation and Listing of Patients Referred for Liver Transplantation

Liver transplantation is the only treatment that increases survival times of patients with decompensated cirrhosis. Patients who live farther away from a transplant center are disadvantaged. Health care delivery via telehealth is an effective way to manage patients with decompensated cirrhosis remotely. We investigated the effects of telehealth on the liver transplant evaluation process. We performed a retrospective study of 465 patients who underwent evaluation for liver transplantation at the Richmond Veterans Affairs Medical Center from 2005 through 2017. Of these, 232 patients were evaluated via telehealth, and 233 via in-person evaluation. Using regression models, we evaluated the differential effects of telehealth vs usual care on placement on the liver transplant waitlist. We also investigated the effects of telehealth on time from referral to initial evaluation by a transplant hepatologist, liver transplantation, and mortality. Patients in the telehealth group were evaluated significantly faster than patients evaluated in person, without or with adjustment for potential confounders (21.7 vs 79.5 d; P < .01). Telehealth also was associated with a significantly shorter time on the liver transplant waitlist (138.8 vs 249 d; P < .01). After propensity-matched analysis, telehealth was associated with a reduction in the time from referral to evaluation (hazard ratio, 0.15; 95% CI, 0.09-0.21; P < .01) and listing (hazard ratio, 0.26; 95% CI, 0.12-0.40; P < .01), but not to transplantation. In the intent-to-treat analysis of all referred patients, we found no significant difference in pretransplant mortality between patients evaluated via telehealth vs in-person. There was statistically significant interaction between model for end-stage liver disease (MELD)-Na scores and time to evaluation (P= .009) and placement on the transplant waitlist (P= .002), with telehealth offering greater benefits to patients with low MELD-Na scores. Use of telehealth is associated with a substantial reduction in time from referral to initial evaluation by a hepatologist and placement on the liver transplant waitlist, especially for patients with low MELD scores, with no changes in time to transplantation or pretransplant mortality. More studies are needed, particularly outside of the Veterans Administration Health System, to confirm that telehealth is a safe and effective way to expand access for patients undergoing evaluation for liver transplantation.

Psychological Assessment Instruments for Use in Liver and Kidney Transplant Evaluations: Scarcity of Evidence and Recommendations

Psychopathology among liver and kidney transplant patients is prevalent. Although pre-surgical psychological evaluations are routinely conducted, understanding which specific psychological test to use is under-developed. The purpose of this review is to examine the psychometric properties of broadband and narrowband psychological measures in pre-surgical liver and kidney transplant evaluations. Overall, there is a paucity of research in this domain that hamper abilities to make clear recommendations on what to use alongside a clinical interview. This review highlights the need for additional research examining instruments that may predict patients’ successful recovery from transplant surgery. Despite the scarcity of research, instruments that appear to be useful in this population include the Minnesota Multiphasic Personality Inventory–2 (MMPI-2), the Personality Assessment Inventory (PAI), the Stanford Integrated Psychosocial Assessment for Transplantation (SIPAT), and the Transplant Evaluation and Rating Scale (TERS).

Exercise Training for Liver Transplant Candidates

Background and AimsFrailty is associated with increased morbidity and mortality, and this is tightly linked to liver decompensation and increased complication rates among liver transplant (LT) candidates. The aim of the study was to evaluate the efficacy of a structured in- and outpatient exercise training program for cirrhotic patients who were referred for liver transplant evaluation. MethodsWe retrospectively reviewed 458 consecutive LT patients. There were 200 patients who underwent LT prior to the implementation of an exercise training program (non-ETP) and 258 LT patients who underwent a comprehensive exercise training program (ETP).Baseline characteristics, readmission rate, and length of hospital stay (LOS) were analyzed and compared between the 2 groups. ResultsThe ETP group were more likely to have diabetes mellitus and coronary artery disease. However, there was no significant difference in the postoperative complication rates between the 2 groups except for more infections in the ETP group compared to the non-ETP group. There was a trend toward lower 90-day readmission rate in the ETP group (17.9% vs 20%) and shorter LOS (14 vs 17 days). ConclusionThere was a trend toward reduced 90-day readmission and shorter length of stay after implementation of an exercise training program.

Arterial Blood Pressure at Liver Transplant Evaluation Predicts Renal Histology in Candidates With Renal Dysfunction.

Renal dysfunction is common in liver transplantation (LT) candidates, but differentiating between reversible and irreversible renal injury can be difficult. Kidney biopsy might be helpful in differentiating reversible from irreversible renal injury, but it is associated with significant complications. We aimed to identify pre-LT predictors of potentially reversible renal injury using histological information obtained on pre-LT renal biopsy. Data on 128 LT candidates who underwent pre-LT kidney biopsy were retrospectively collected and correlated with renal histological findings. Indications for kidney biopsy were iothalamate glomerular filtration rate (iGFR) ≤40mL/minute, proteinuria >500mg/day, and/or hematuria. According to the biopsy diagnosis, patients were grouped into the following categories: normal (n=13); acute tubular necrosis (ATN; n=25); membranoproliferative glomerulonephritis (n=19); minimal histological changes (n=24); and advanced interstitial fibrosis (IF) and glomerulosclerosis (GS) (n=47). Compared with patients having advanced IF/GS, patients with normal biopsies and those with ATN had lower systolic blood pressure (SBP) and diastolic blood pressure (DBP) and higher international normalized ratio and total bilirubin levels (<0.05 for all). Both SBP and DBP directly correlated with the degree of IF and GS (R=0.3, P≤0.02 for all). SBP ≤90mmHg was 100% sensitive and 98% specific in correlating with normal biopsies or ATN, whereas SBP ≥140mmHg was 22% sensitive and 90% specific in correlating with advanced IF/GS. Model for End-Stage Liver Disease score, serum creatinine, iGFR, urinary sodium excretion, and renal size did not correlate with biopsy diagnosis or degree of IF or GS. In conclusion, SBP at the time of LT evaluation correlates with renal histology, and it should be included along with other clinical and laboratory markers in the decision-making process to list patients with renal dysfunction for LT alone versus simultaneous liver-kidney transplantation.

1049 The Palliative Performance Scale Among Patients Evaluated for Liver Transplantation

INTRODUCTION: Patients being evaluated for liver transplantation (LT) often suffer from poor health-related quality of life. While several quality measures exist for palliative care in cancer patients, there are limited cirrhosis-specific palliative care tools. The Palliative Performance Scale (PPS) is a prognostication tool that measures a patient’s functional status based on ability to ambulate, activity level, evidence of disease, self-care abilities, oral intake, and level of consciousness, where a patient with a PPS of 100% is functionally healthy. We aim to observe how PPS at initial transplant evaluation relates to patient demographics, decompensation, and transplant outcomes to establish its potential role in the LT population. METHODS: We examined the records of patients who completed LT evaluation at our mid-size liver transplant center. Between March and December 2018, we calculated the PPS as a part of new outpatient LT evaluations. Descriptive statistics were used to evaluate relationships between patient PPS scores and MELD, disease characteristics, and outcomes. RESULTS: Forty-nine patients completed LT evaluation, of which 71% (N = 35) were male and 80% (N = 39) were white. The average age at the time of evaluation was 63. The most common etiology of cirrhosis was NASH (35%, N = 17), followed by alcohol (29%, N = 14), and HCV (25%, N = 12). Among the three most common etiologies, those with alcoholic cirrhosis had the lowest average PPS scores (76), patients with HCV had the highest (90). During the study period, 8 patients were transplanted. Their average PPS at the time of evaluation was 76 compared to 80 among those who were not transplanted. When compared to men, women had a higher MELD (15 vs 13), and lower PPS (74 vs 82). The rates of decompensation among patients with a PPS of 100, 80, and 70 were 55%, 86%, and 93%, respectively. CONCLUSION: This pilot study examines the utility of PPS for patients undergoing LT evaluations, and several trends were elucidated warranting further evaluation. There was not a clear linear relationship between MELD and PPS, indicating that patients with end-stage liver disease have a variable symptom burden even at advanced disease states. The functional status of patients appeared to vary significantly between etiology, gender, and transplantation. Importantly, our data suggests that patients with documented decompensations had lower PPS, indicating a possible role for the PPS to better characterize the functional status and disease burden of these patients.

2370 Acute Liver Failure After Foraging for Wild Mushrooms: A Family Affair?

INTRODUCTION: Ingestion of cytotoxic mushrooms is associated with a high mortality rate due to acute liver failure (ALF). Amanita phalloides (AP) and related species account for over 90% of fatal cases, and can be easily mistaken with edible species by inexperienced foragers. There is currently no gold standard of therapy, and often liver transplantation (LT) is required. We present 5 cases of mushroom ingestion and associated ALF who were treated at a quaternary LT center in Northeast Ohio. CASE DESCRIPTION/METHODS: Five members of a family were referred to our Medical Intensive Liver Unit in September 2018 after ingesting a shared meal prepared with wild mushrooms from a neighbor’s garden. Median age was 43.6 years (32-61 years) and all patients were female. All patients presented to a local hospital with nausea, vomiting, diarrhea and abdominal pain approximately 6 hours after ingestion and were transferred to our center 1 day later. Mean peak laboratory values were as follows: alanine aminotransferase 6530 U/L (range 3750-10506 U/L), aspartate aminotransferase 10974 U/L (3780-19737 U/L), bilirubin 7.2 mg/dL (4.6-11.3 mg/dL) and international normalized ratio 3.4 (1.8-7.1). Mushroom specimens were obtained for inspection and visually identified as AP. The 5 patients were treated with aggressive supportive care, including N-Acetylcysteine, octreotide and penicillin G infusions, followed by experimental silibinin obtained under emergent institutional review board approval. All patients progressed to ALF and underwent emergent LT evaluation; all 5 were listed for LT (3 as status 1A). Three patients improved with medical therapy, while 2 underwent successful LT. Pathology of the explanted livers revealed extensive necrosis. The local departments of health were notified and educational programs implemented throughout Northeast Ohio. DISCUSSION: We present 3 cases of successful medical therapy associated with transplant-free survival, and 2 cases of LT for severe ALF related to mushroom ingestion. In some Southeast Asia cultures, foraging for wild mushrooms is common practice. Mortality has been reduced in recent decades due to improved supportive and intensive care, as well as the introduction of experimental therapies. A high index of suspicion for diagnosis is important, as timing of initiation of therapy is paramount. Public education for populations in which foraging is common will be important to promote proper identification of wild toxic mushrooms.

2281 Symptomatic Isolated Polycystic Liver Disease Causing Respiratory Compromise

INTRODUCTION: Isolated Polycystic Liver Disease (PCLD) is defined by the presence of more than 20 hepatic cysts. It has an autosomal dominant inheritance and is largely asymptomatic with only 5% of patients who report pain or other complaints. The disorder is considered rare with an estimated incidence of &lt; 0.01%. We present a case of isolated PCLD causing respiratory failure. CASE DESCRIPTION/METHODS: A 45-year-old woman with history of an isolated liver cyst, was admitted for acute respiratory failure. No significant family history. On physical exam, oxygen saturation was 98% on 2 liters nasal cannula. She had prolonged expiratory phase, decreased breath sounds and marked hepatomegaly. FEV1 = 23%, FEV1/FVC = 117%. Liver studies were significant for alkaline phosphatase 131 IU/L and albumin 3.3 gm/dl. Abdominal CT showed a markedly enlarged liver measuring 21 cm, liver volume of 3588 ml and the presence of numerous thin-walled-fluid-filled cysts ranging from 2 mm to 4 cm, consistent with PCLD. It was determined that the mass effect from the liver pressing against the diaphragm was the cause of her respiratory failure. She was started on BiPAP with improvement of respiratory function. She underwent evaluation for orthotopic liver transplantation(OLT) but was not a candidate due to high risk for hypercarbic respiratory failure, and prolonged mechanical ventilation. She was discharged on non-invasive ventilation with oxygen supplementation and was scheduled for re-evaluation for OLT. DISCUSSION: Isolated PCLD is caused by germline mutations of the PRKCSH and SEC63 genes. Genetic testing is rarely necessary and does not affect management. Liver function is often altered once symptoms develop. The number and size of liver cysts often increase with age. Women often present with a massive and symptomatic cystic liver more frequently than men and symptoms are likely attributed to the compression of adjacent organs due to mass effect, which often leads to poor body image and depression. When symptoms develop they include pain, nausea, early satiety, vomiting, or dyspnea. Complications are rare and can include infection, portal hypertension, and Budd-Chiari Syndrome. Treatment includes aspiration-sclerotherapy, laparoscopic or open surgical fenestration techniques, and hepatectomy. Somatostatin analogues show promise. Liver transplantation may be required in extensive disease. One should be cognizant that symptomatic isolated PCLD often leads to greatly diminished quality of life.

2347 Acute Liver Failure: Drug-Induced Liver Injury and a Perfect Thyroid Storm

INTRODUCTION: Acute liver failure (ALF) is a rare but serious condition defined by severe acute liver injury with coagulopathy (INR ≥ 1.5) and encephalopathy without preexisting cirrhosis. ALF carries a high morbidity and mortality. Liver transplantation (LT) is the only definitive treatment for patients that fail other supportive therapies. CASE DESCRIPTION/METHODS: A 28 year-old female with hyperthyroidism due to Grave disease was transferred to our institution for acute liver failure (ALF) and transplant evaluation. One month prior, she developed jaundice and pruritus. She had been treated with propylthiouracil (PTU) for 1 year but that was stopped 1 week prior to transfer due to elevated liver enzymes. On evaluation she was found to have elevated AST/ALT (2800/1900), hyperbilirubinemia (28.6) and an elevated INR (2.85). The patient progressively worsened and developed encephalopathy thus fulfilling the criteria for ALF. On admission, her MELD-Na was 33 and she had grade III hepatic encephalopathy. Her thyroid studies were found to be TSH &lt;0.010, free T3 17.9 and free T4 &gt;5.6. Her Burch-Wartofsky Point Scale was &gt;45, which is highly suggestive of thyroid storm. She was subsequently started on plasmapheresis, methimazole, hydrocortisone and cholestyramine for thyroid dysfunction. Patient continued to clinically decompensate requiring an emergent thyroidectomy followed by LT. Post-operatively patient had an acute change in her neurological status with loss of reflexes. CT head showed diffuse global cerebral edema with brainstem compression and herniation. Medical care was withdrawn and patient expired. DISCUSSION: This is a case of ALF from PTU drug induced liver injury complicated by thyroid storm. PTU is the second most common non-acetaminophen related cause of DILI that requires liver transplantation. PTU-related ALF can occur at any point of treatment and the onset is usually sudden with rapid progression. While extremely rare, thyroid storm is also a reported cause of ALF. Excess thyroid hormone may cause hepatocyte dysfunction. This case poses a diagnostic dilemma given the coexistence of thyroid storm and PTU therapy. It is possible that the initial hepatic injury due to PTU-related DILI made the liver more susceptible to fulminant hepatic failure from thyroid storm. Despite the rapid listing and transplantation within 48 hours of arrival, the patient did not survive. This case extends the spectrum of the presentation of ALF in the setting of thyroid dysfunction.

2405 Even When All Hell Breaks Loose…We Have a Plan!

INTRODUCTION: Acute variceal bleeding is a severe complication of portal hypertension. Endoscopic banding successfully controls the vast majority of acute variceal bleeding. Refractory variceal bleeding refers to a small percentage of acute variceal bleeding not controlled by endoscopic therapy, requiring more aggressive modalities such as a Sengstaken–Blakemore tube. We present a case series of two patients with hematemesis secondary to varices, requiring the use of Self-expandable metal stents (SEMS) with successful hemostasis. CASE DESCRIPTION/METHODS: Patient 1: A 38-year-old male with decompensated cirrhosis in the setting of alcohol use complicated by esophageal varices presented with hematemesis. On presentation, he was hypotensive and tachycardic with several large bouts of hematemesis prompting endotracheal intubation for airway protection. He required a massive transfusion protocol and was started on vasopressor support. Initial upper endoscopy showed five columns of large varices (&gt;5 mm) in the middle third and lower third of the esophagus. One varix was actively spurting at about 38 cm from the incisor, for which four bands were successfully placed with resolution of the active bleeding. The patient had a repeat episode of massive hematemesis the following day requiring upper endoscopy during which active bleeding was appreciated but a source could not be accurately identified. Due to lack of visualization, banding could not be repeated and a fully covered 23 mm × 105 mm esophageal stent was placed starting at 30 cm from the incisor to the GE junction with excellent hemostasis. The patient had no further episodes of hematemesis and was transferred for liver transplant evaluation. Patient 2: A 30-year-old male with incompletely treated hepatitis B complicated by hepatocellular carcinoma invading the portal vein and extending to the Superior Mesenteric Vein presented with hematemesis. He was intubated and required upper endoscopies on two consecutive days for management of active bleeding from esophageal varices with banding. He was considered not to be a candidate for TIPS. On day 3, he had another episode of hematemesis causing hypotension. An upper endoscopy was performed and a fully covered esophageal stent was placed to tamponade the varices. He did not have any more episodes of bleeding in the remaining hospitalization. DISCUSSION: SEMS should be considered as an option for management of refractory variceal bleeding.

2368 A Rare Case of Segmental Arterial Mediolysis in a Patient With Alpha-1 Antitrypsin Deficiency and Alcoholic Cirrhosis

INTRODUCTION: Alpha-1 antitrypsin deficiency (AAT deficiency) is a systemic disease characterized by lack of the protease inhibitor, alpha-1 antitrypsin and typically manifests as severe emphysema and/or liver disease. There are several case reports describing vascular compromise in patients with AAT deficiency, however mostly in the context of systemic vasculitis and connective tissue disease. Segemental arterial medioloysis (SAM) is a rare non-vasculitis, non-atherosclerotic arteriopathy of middle and small arteries that can lead to aneurysmal dilation or arterial stenosis. We report the first case of a patient with cirrhosis secondary to AAT deficiency noted to have arteriopathy due to SAM. CASE DESCRIPTION/METHODS: A 30 year old patient with end stage renal disease (ESRD) on hemodialysis, alcoholic cirrhosis and underlying AAT deficiency presented to our tertiary care center for liver transplant evaluation with a presenting MELD score of 39. Evaluation for other causes of cirrhosis revealed AAT deficiency with a homozygous ZZ phenotype and undetectable serum levels. As part of his pre-transplantation workup at our institution, he underwent computed tomography angiography (CTA) of the abdomen and pelvis which revealed severe irregular narrowing of the celiac axis. Similar narrowing was noted of the renal arteries and splenic artery and focal aneurysmal dilation of the hepatic and gastroduodenal arteries was additionally seen (Image 1). Autoimmune serologies were negative. Patient proceeded to orthotropic liver transplantation during his hospitalization. Intraoperatively, hepatic artery appeared visually vasculitic but histopathologic report was consistent with diagnosis of SAM. Due to concern for potential graft compromise that could result from the recipient hepatic artery aneurysm, an aortic conduit was performed using the donor vessels with anastomosis to the recipient supraceliac aorta. DISCUSSION: To our knowledge, there is only one other published report describing SAM in a patient with co-existing AAT deficiency. SAM in this case resulted in hepatic aneurysm which complicated liver transplantation, necessitating alternative graft revascularization via an aortic conduit. Although doppler evaluation 3 days after transplantation showed hepatic artery anastomosis was still patent, the rate of complications related to aorto-hepatic bypass grafts is high, and close follow up remains prudent.

95 Prevalence of Intrapapillary Mucinous Neoplasm in Patients With Chronic Kidney Disease

INTRODUCTION: The prevalence of intraductal papillary mucinous neoplasm (IPMN) in the general population ranges from 3.3%–9.8%. In patients undergoing liver transplant evaluation, overall prevalence is documented to be 6.6%. So far, the suggested risk factors for IPMN include diabetes, family history of pancreatic adenocarcinoma, and tobacco use. We aim to study the prevalence and risk factors for IPMN formation in patients with chronic kidney disease (CKD). METHODS: We performed a retrospective cross-sectional study of patients with CKD who had MRIs of the abdomen performed between the dates of 1/2018 and 12/2018. Independent chart review was conducted and various demographic, clinical, and imaging metrics were extracted. Overall, the prevalence of IPMN was calculated. A student's t-test and chi-square test were used to compare the IPMN group to non-IPMN group. RESULTS: A total of 400 patients were reviewed. The total prevalence of IPMN in CKD patients was 13.7% (55/400). In 68% of the study patients, the MRI was done for a kidney transplant evaluation. The average age of patients in the study was 58 ± 14 years, 59% were male and 52% patients were on dialysis. Diabetes and Hypertension accounted for 26% and 45% of CKD respectively. In patients with IPMNs, the average size was 10 ± 5.7 mm; 45% were located in the head, 33% in the body and 22% in the tail. Notably, 16% were main duct IPMNs, 80% were side branch IPMNs and 4% patients had mixed (MD + SB) IPMNs. Amongst the patients with IPMNs, 7.5% of patients had associated high-risk features and were referred for endoscopic ultrasound. Comparison of the IPMN group to non-IPMN group is shown in Table 1. IPMNs were most commonly observed in elderly patients. Gender, dialysis status, etiology of CKD, smoking and alcohol intake did not seem to impact the prevalence of IPMN in CKD patients. CONCLUSION: Patients with CKD have a higher prevalence of IPMNs than the general population. We further aim to confirm this interval data with our age and gender matched control group without CKD.

2350 An Unlikely Presentation of Diffuse Large B-Cell Lymphoma Manifesting as Hepatic Failure

INTRODUCTION: Primary Hepatic Lymphoma (PHL) is a very rare subtype of Diffuse Large B-Cell Lymphoma (DLBCL), accounting for less than 1% of all cases. In rare instances, a patient can present with fulminant hepatic failure. The prognosis is grim with an average survival of 10.7 days from admission. The mainstay of diagnosis is liver biopsy, which must be performed as early as possible to prevent progression to acute liver failure. CASE DESCRIPTION/METHODS: A 55-year-old male with no pertinent medical history presented with a two week history of abdominal pain, fever, lethargy and mild jaundice. He was a former smoker and drinker who denied any alcohol use for the past 15 years. Upon presentation, the patient’s labs showed acute liver injury, anemia, thrombocytopenia and acute kidney injury. The patient’s model for end-stage liver disease (MELD) was 29. He was subsequently admitted to the medical intensive care unit (ICU) where emergent liver transplant evaluation was initiated. A full work up for the etiology of his liver failure was initially unrevealing so a liver biopsy was performed, which came back positive for DLBCL. The patient was then started on a chemotherapeutic regimen consisting of cyclophosphamide, vincristine, etoposide and high dose steroids. The patient’s condition further deteriorated during his ICU stay, leading to the need for mechanical ventilation, vasopressor therapy for circulatory shock, continuous renal replacement therapy for worsening renal failure and transfusion of blood products for worsening coagulopathy. Despite all of these aggressive measures, the patient’s clinical status only continued to worsen. The family decided to institute comfort measures and the patient died on ICU day 12. DISCUSSION: DLBCL predominantly affects Caucasian men over the age of 50. Some viruses, specifically EBV, HBV and HCV, have shown correlation with Non-Hodgkin Lymphoma (NHL); our patient had serologies indicative of past exposure to EBV and HBV. The only way to definitively diagnose DLBCL is via liver biopsy with immunohistochemical staining that reveals CD20, CD45 and BCL-6. The prevailing treatment modality is the use of Dose Adjusted Etoposide, Prednisone, Oncovin, Cyclophosphamide, Hydorxydauorubicin and Rituximab (DA-EPOCH R) Therapy. Due to its poor prognosis, early diagnosis and initiation of treatment for PHL is crucial. For this reason, it must be considered early in the differential when evaluating a patient with acute liver failure.

2473 The Mystery of the “Vanishing” Bile Ducts

INTRODUCTION: The etiologies of cholestatic liver disease is extensive, including immunological, ischemic, infectious, metabolic, and toxic causes. We present a rare case of a patient who was diagnosed with idiopathic vanishing bile duct syndrome (VBDS) after a thorough work-up. CASE DESCRIPTION/METHODS: A 61-year-old female with a history of hypertension, type II diabetes mellitus, congestive heart failure, and end stage renal disease presented with generalized pruritus and jaundice. Laboratory tests revealed a total bilirubin 10.5 mg/dL, AST 27 IU/L, ALT 27 IU/L, alkaline phosphatase 596 IU/L. Further workup was negative for viral hepatitis, autoimmune hepatitis, hemochromatosis, alpha-1 antitrypsin deficiency, and Wilson's Disease. An MRCP showed hepatosplenomegaly without ductal dilatation, and upper endoscopy with ultrasound revealed a normal biliary duct without evidence of obstruction. Pathology from fine needle biopsy was significant for chronic cholestatic changes with ductopenia (43% duct loss), obliterative portal venopathy, and congestive hepatopathy. Without an identifiable cause, these findings are most consistent with idiopathic VBDS. The patient was referred for liver transplant evaluation due to concern for progression to cirrhosis. DISCUSSION: VBDS is a rare hepatic condition characterized by progressive loss of the interlobular and septal bile ducts resulting in cholestasis and potentially biliary cirrhosis and liver failure. The cause of bile duct destruction is poorly understood but likely results from biliary apoptosis due to immunologic, ischemic, infectious, metabolic, and toxic assaults. 1 Approximately 1% of cases classify as idiopathic VBDS, which likely comprises of several disease processes with atypical features, such as late-onset non-syndromic paucity of intrahepatic bile ducts, small-duct primary sclerosing cholangitis without large duct involvement, non-suppurative viral cholangitis, and autoimmune cholangitis without typical autoantibodies. 2 The disease course is unpredictable, ranging from benign to fatal or necessitating transplantation.2 Treatment of VBDS includes drug withdrawal, choleretic agents, and immunosuppression. The uncertain etiology of idiopathic VBDS precludes a causative treatment. Liver transplantation has been the only successful treatment. 2