bluuebunny/crossref_metadata_2025 huggingface dataset:
2450 Gastroenterology, a Guiding Light in Hemophagocytic Lymphohistiocytosis
Authors: Viswanathan Sahityan , Capati Jeffrey , Hoang Long
Published: October 2019

INTRODUCTION:

Transaminitis is not an uncommon consult for a gastroenterologist. The presence of profound transaminitis in the setting of septic shock often skews our etiology towards shock liver and other more common causes of acute liver injury in the absence of previous known liver pathology. HLH is a rare cause of acute liver injury but lack of prompt diagnosis and treatment can make the difference in outcome.

CASE DESCRIPTION/METHODS:

Patient is a 27 year old male with no known past medical history who presented with a two week history of fever in which he took acetaminophen every four hours. Reported history of alcohol consumption of 12 beers per day for three years. History remarkable for a camping trip to Oklahoma two to three weeks prior to admission. No history of NSAID or herbal use. No personal or family history of liver disease. Patient admitted to the ICU and treated for septic shock and subsequently started on N-acetylcysteine in the setting of acetaminophen use and acute liver injury. Patient had worsening renal function requiring initiation of dialysis, acute respiratory failure requiring intubation during evaluation. Gastroenterology consulted on hospital day two in which complete hepatic evaluation was initiated with remarkable findings of markedly elevated ferritin and positive Ebstein-Barr virus (EBV). Suspicion of Hemophagocytic lymphohistiocytosis (HLH) noted per Gastroenterology given high ferritin and diagnostic criteria being met. This prompted recommendations for evaluation by Hematology in which bone marrow biopsy confirmed HLH and prompt treatment with Rituxan, Dexamethasone and subsequent clinical improvement.

DISCUSSION:

EBV-HLH is an uncommon entity that is encountered on a daily basis. However, the mortality associated with the disease process is high if timely intervention is not implemented. A constellation of clinical features and laboratory results comprise clinical diagnosis of HLH combined with multi-speciality contribution to formulate the diagnosis. Transaminitis, elevated ferritin, cytopeniae should not skew a clinician's thought process towards shock liver altogether and EBV-HLH should be high on the differential early in the clinical course. Gastroenterology will be involved frequently in management due to transaminits and gastrointestinal bleeding manifesting frequently. Early treatment of EBV-HLH with immunosuppressants has shown to reduce mortality by 40% mandating more awareness about the disease in Gastroenterology and be a guiding light in the management.
2452 Intrahepatic Portosystemic Venous Shunt: A Clinically Significant Rare Anomaly
Authors: Nagaraj Savitha V. , John Febin , Etienne Denzil
Published: October 2019

INTRODUCTION:

Intrahepatic Portosystemic Venous Shunts (IPSVS) are rare vascular malformations between the hepatic and portal veins. Most of the porto-venous shunts are asymptomatic and found incidentally on radiology imaging. These can be either congenital or acquired. Acquired shunts are typically less than 2 mm in size. Occasionally, complications such as encephalopathy, heart failure, pulmonary hypertension and elevated ammonia levels can occur. We report a case of incidental IPSVS in a patient who presented with complaints of fatigue and failure to thrive.

CASE DESCRIPTION/METHODS:

82-year-old African American male with hypertension, diabetes mellitus and prostate cancer (treated) presented with complaints of fatigue and loss of appetite for one-month. Laboratory work up showed a hemoglobin of 11.6 g/dl, normal platelet count, and elevated aspartate aminotransferase of 45 U/L and decreased albumin of 2.0 g/dl. Serum ammonia level was normal. He was immune to hepatitis A, non immune to hepatitis B and hepatitis C antibody was negative. A computed tomography scan of the abdomen showed a contrast enhanced tubular shaped vascular connection between middle hepatic vein and the anterior branch of the right portal vein. The size of the shunt was approximately 10 mm (Figure 1). Liver appeared normal except for small calcifications within the parenchyma. Ultrasound Doppler of hepatic and portal veins were performed, and the porto-hepatic shunt was visualized (Figure 2). Patient had a complicated hospital course secondary to sepsis; however, he did not develop hyperammonemia or hepatic encephalopathy during the hospital stay.

DISCUSSION:

IPSVS in our patient was likely a congenital anomaly given the presentation, size and lack of related symptoms. IPSVS is divided into 4 types; Type 1- Shunt between portal vein and IVC; Type 2- connections between peripheral branches of portal and hepatic veins in one hepatic segment; Type 3- Aneurysmal connection between portal and hepatic vein; Type 4- multiple communications of portal and hepatic veins in both major lobes of the liver. Doppler sonogram with pulse doppler is highly sensitive and specific to evaluate for IPSVS. In symptomatic IPSVS, shunt closure has shown improvement in symptoms and correction of hyperammonemia. Clinicians should consider this rare vascular malformation in patients presenting with hepatic encephalopathy or hyperammonemia in the absence of cirrhosis where shunt closure can be curative.
2457 Khat-Induced Hepatotoxicity
Authors: Kumar Kishore , Bhandari Peter , Essrani Rajesh Kumar , Chime Chukwunonso U. , Ahmed Rafeeq , Tariq Hassan , Patel Harish
Published: October 2019

INTRODUCTION:

Khat is a commonly used recreational substance in Somali, Yemeni and East African countries due to its stimulant properties. The active ingredients of Khat are structurally similar to amphetamine and phenylethylamines respectively. Physiologic effects of Khat includes increased alertness, euphoria and hypertension. Khat use has been associated with subacute hepatitis and severe hepatitis leading to adverse outcomes including death. Rarely it can cause psychosis, acute coronary syndrome and stroke. Therefore, many countries have banned the possession and use of Khat, because of its potential for health and social harm. Here we present a case of hepatoxicity in Yemeni man after Khat consumption.

CASE DESCRIPTION/METHODS:

32-year-old man with no prior medical co-morbid condition presented to the ER with right upper quadrant abdominal pain for 1 day. The review of system is negative for fever, nausea, vomiting, diarrhea and weight loss. He denies use of herbal medication or over the counter medication. He has no personal or family history of liver disease. Social history was significant for recreational use of Khat and last use was few months prior to current illness. He denied alcohol and tobacco use. The physical examination revealed normal vital signs and right upper quadrant tenderness. Initial workup showed hepatocellular liver injury, indirect hyperbilirubinemia and gallstone on abdominal sonogram (Table 1). He had normal biliary tree on ultrasound and MRCP. Comprehensive workup for liver disease remained unrevealing subsequently he underwent liver biopsy which revealed lobular hepatitis with many eosinophils consistent with drug induced liver disease. The liver biopsy was negative for iron overload and significant steatosis. The presence of lobular hepatitis on liver biopsy and chronologic use of Khat in the absence of alternate etiology suggest Khat related hepatoxicity in our patient.

DISCUSSION:

The WHO classified Khat as a substance of abuse that can lead to severe dependence. While viral and alcoholic etiologies are the most common cause of hepatitis, Khat induced hepatitis is easily missed and often undiscovered. The mechanism of Khat-related hepatotoxicity remains to be elucidated though histopathologic evidence showed central vein congestion and hepatocellular degeneration and regeneration. The characteristic clinical picture is of acute or subacute hepatitis that resolves on cessation of Khat use. It is essential that other causes for liver disease be ruled out.
2459 Is Shorter Course of Gleceprevir and Pibrentasvir (MAVYRET) for Hepatitis C Treatment Possible? A Case Series
Authors: Jindal Robin , Sidhu Rajvinder , Martinez Anthony
Published: October 2019

INTRODUCTION:

Direct-acting oral antiviral agents (DAA) including a combination of Gleceprevir and Pibrentasvir (Mavyret), among others, is the current standard of care therapy for chronic hepatitis C (HCV) infection. These medications have demonstrated high rates of sustained virological response at 12 weeks post-therapy (SVR12) in all genotypes of HCV. More specifically for Mavyret, multiple clinical trials have demonstrated SVR12 in HCV patients after 8, 12, or 16 weeks of therapy. There is, however, no data to support a shorter therapy course. In this small case series, we report 5 subjects who achieved SVR12 with Mavyret after 2 or 4 weeks of therapy.

CASE DESCRIPTION/METHODS:

Clinical data from a large cohort of patients treated for HCV was retrospectively reviewed for patients treated from 2015-2019. Data extracted and analyzed included patient demographics, HCV treatment regimen, HCV genotype, HCV RNA levels, fibrosis state and sustained virological response (SVR).

DISCUSSION:

Results: Among the 5 patients identified there were 4 males and 1 female. In total, 4 patients had genotype 1a and 1 patient had genotype 1b. The single patient with SVR12 after 2 weeks of therapy was genotype 1a. The remaining patients received 4 weeks of therapy. Fibrosis Score ranged from F0 to F1, HCV RNA levels ranged from 83,400-8,420,000 and all patients had a documented history of intravenous drug use (Table 1). Conclusion: In this small case series, we present 5 patients who achieved SVR12 after only 2 or 4 weeks of Mavyret therapy. When considering factors such as the cost of therapy, possible history of substance abuse, and/or poor socioeconomic status, opting for a shorter course of therapy may be beneficial. Further large-scale prospective clinical trials are needed to support these findings.
2460 A Father-Son Pair With APOB and PNPLA3 Double Heterozygosity Resulting in Advanced Nonalcoholic Fatty Liver Disease
Authors: Jansson-Knodell Claire L. , Gawrieh Samer , McIntyre Adam D. , Hegele Robert A. , Chalasani Naga P.
Published: October 2019

INTRODUCTION:

Mutations in apolipoprotein (apo) B cripple the machinery and transport needed for lipoprotein export from the liver resulting in familial hypobetalipoproteinemia (FHBL). In heterozygotes, the normal allele allows for sufficient transport and mobilization of fat from the liver, but homozygotes are unable to compensate, leading to elevated intrahepatic triglycerides, NASH, and cirrhosis. We report a father-son pair with heterozygote FHBL who developed advanced NASH which we believe is due to its compound heterozygosity with PNPLA3, a gene important in the pathogenesis of NAFLD and NASH.

CASE DESCRIPTION/METHODS:

A Caucasian father and son of European ancestry were referred to our hepatology clinic. The son is a 46-year-old male who was evaluated for elevated transaminases and very low LDL-cholesterol. There was no significant alcohol consumption. Family history was significant for a father with cirrhosis and paternal grandmother who died of an unknown liver complication. Physical exam revealed obesity with hepatomegaly and acanthosis nigricans. Laboratory studies were notable for an LDL-cholesterol 15 mg/dL, triglycerides 30 mg/dL, and ALT 109 U/L. Increased liver echogenicity was found on ultrasound. Transient Elastography showed CAP of 327 dB/m (severe steatosis) and liver stiffness of 8.5 kPa (F2 fibrosis). Genetic testing revealed heterozygosity for both APOB (c.1830 -1G >A) and PNPLA3 (p.I148M) genes. The father is a 71-year-old male who was diagnosed with liver disease during evaluation for thrombocytopenia. There was no significant alcohol consumption. Physical examination revealed abdominal obesity and splenomegaly. Laboratory studies were notable for an LDL-cholesterol of 28 mg/dL and platelets of 83 k/mm3. Liver biopsy confirmed cirrhosis due to steatohepatitis and his HVPG was 12 mmHg. Recent imaging identified two small liver lesions with arterial enhancement, concerning for early liver cancer. Genetic testing revealed variants in both APOB and PNPLA3 genes identical to his son's mutations.

DISCUSSION:

We describe a father-son pair with double heterozygosity for APOB and PNPLA3 genes in the setting of obesity who developed progressive NASH. Neither APOB heterozygosity nor PNPLA3 heterozygosity is associated with advanced NAFLD, but their double heterozygosity may cause progressive NASH and cirrhosis, as illustrated here. A strong family history and very low serum LDL-cholesterol levels were helpful in working up this father-son pair.
2463 Infliximab Drug-Induced Autoimmune Hepatitis in Patient With Crohn's Ileocolitis
Authors: Karanfilian Briette , Mahpour Noah , Reja Debashis , Sarkar Avik
Published: October 2019

INTRODUCTION:

Infliximab is an anti-tumor necrosis factor alpha inhibitor commonly used in the treatment of Crohn's disease. It is known to produce mild elevations in liver enzymes, however, severe damage and marked elevations in transaminases related to drug-induced autoimmune hepatitis (AIH) are rare. We present a case of a patient with Crohn's disease who recently began infliximab and presented with severe infliximab-induced autoimmune hepatitis.

CASE DESCRIPTION/METHODS:

A 37-year-old man with Crohn's ileocolitis, diagnosed at age 28 and recently inducted with infliximab, presented with fatigue, weakness, and epigastric pain. He had previously been on 6-mercaptopurine, adalimumab, and ustekinumab, but had most recently been on azathioprine (AZT) and began infliximab two months prior to presentation. Approximately one month prior to presentation, he developed elevated transaminases with aspartate transaminase (AST) and alanine transaminase (ALT) in the 300s, so AZT was discontinued as this was thought to be the cause. Despite discontinuation of AZT, his liver function tests (LFTs) rose dramatically, prompting hospitalization. Labs on admission revealed a hepatocellular pattern with total bilirubin 7.1, AST 2153, ALT 2931, alkaline phosphatase 156, and international normalized ratio 1.25. Notably, antinuclear antibody was positive 1:1280, smooth muscle antibody was weakly positive, and cytomegalovirus (CMV) IgM was elevated. Hepatobiliary scan and magnetic resonance cholangiopancreatography were consistent with hepatocellular disease. Core liver biopsy revealed active hepatitis with moderate activity (interface moderate), cholestasis, and an infiltrate of eosinophils and plasma cells. CMV staining was negative. Overall, the testing was consistent with drug-induced AIH. Infliximab was stopped and steroids were begun, resulting in normalization of LFTs.

DISCUSSION:

Infliximab has been implicated in mild cases of hepatotoxicity, and therefore, it is recommended to check LFTs before and after beginning the medication. There are very few reports of severe AIH that have been linked specifically to infliximab, as cases of hepatotoxicity are often secondary to reactivation of viral hepatitis or CMV infections, or related to other biologic therapies. This is a rare case of infliximab-induced AIH, and it is an important consideration in patients with elevated LFTs after starting this drug. Treatment involves cessation of infliximab and treatment with steroids, which usually leads to resolution of the hepatitis.
2470 Mysterious Ascites
Authors: Fernandez Carlos , Diaz Carolina , Bofill Aliana , De Jesús Henry , Caceres William , Martin-Ortiz Jose , Santiago Loscar
Published: October 2019

INTRODUCTION:

Hypereosinophilic Syndrome (HES) is a rare disorder most commonly diagnosed in patients 20-50 years of age. It is defined as a persistent absolute eosinophil count greater than 1500 cells/microL and/or pathologic confirmation of tissue hypereosinophilia, as well as evidence of end-organ dysfunction. Prior to making a diagnosis, allergic, rheumatologic, infectious, and neoplastic etiologies should be excluded.

CASE DESCRIPTION/METHODS:

This is the case of an 87 -year-old male with past medical history of malaria treated with chloroquine who presented with altered mental status, dry cough and ascites de novo. Associated symptoms included unintentional weight loss of 10-15 lbs and intermittent episodes of non- bloody, watery diarrhea. He denied history of alcohol or drug use. Physical exam was remarkable for a distended abdomen with a positive fluid wave and pitting edema +1 in the lower extremities bilaterally. Labs showed a platelet count of 53,000, hypoalbuminemia (1.9 g/dl), increasing creatinine (2.1 mg/dl), alkaline phosphatase of 444 u/l, total bilirubin of 2.8 mg/dl with indirect predominance, AST of 59 u/l, ALT of 55 u/l, and eosinophilia of 72% (absolute eosinophil count of 6,048). Abdominal sonogram with doppler failed to show obstructive pathology and no evidence of hepatic vein thrombosis. Paracentesis showed absence of malignant cells, SAAG >1.1, 0.79 g/dl of protein and abundant eosinophils. Hepatitis, Iron Saturation levels, AMA, ASMA, Schistosoma and Strongyloides workup were negative. Hematology oncology service was consulted for bone marrow biopsy to asses for myeloid and lymphoid disorders. Results showed a hypercellular bone marrow with marked eosinophilia and 50% nucleated cells but C-KIT, PGFR Alpha, PGFRB, BCR/ABL, JAK 2 and TCR were all negative. A diagnosis of Hypereosinophilic Syndrome with suspected eosinophilic liver infiltration was made and the patient was started on prednisone therapy. All prior described multisystemic symptoms resolved. Eosinophilia, thrombocytopenia and all liver enzymes normalized within 24-48 hours after initiation of therapy. Ascites also resolved for which diuretic therapy was discontinued.

DISCUSSION:

This case is interesting as HES has an incidence of 0.36 per 100,000, and hepatic involvement accounts solely for an estimated 30% of patients. This serves to illustrate that HES should form part of the differential diagnosis when hepatic involvement and eosinophilia are concerned as prompt evaluation and treatment can lead to rapid resolution.
2471 UnDRESSing Elevated Liver Enzymes
Authors: Hakim Christopher M. , Melitas Constantine , Ngo Kha
Published: October 2019

INTRODUCTION:

The differential diagnosis for liver transaminases over 1000 units/liters typically includes liver ischemia, acute viral hepatitis, acetaminophen-induced liver toxicity, autoimmune hepatitis, Wilsonian crisis, or acute Budd-Chiari. Prompt evaluation and exclusion of these causes must be completed as many of these etiologies can ultimately lead to fulminant liver failure. We present a case of liver transaminases over 1000 units/liter from an atypical etiology.

CASE DESCRIPTION/METHODS:

A 52-year-old male with hypertension, CKD stage 5, gastroesophageal reflux disease, and gout presented with intermittent fevers of 39C. He was recently hospitalized for Clostridium difficile colitis and treated with oral vancomycin. During this hospitalization he was started on allopurinol for an acute gout flare. Soon after starting allopurinol, he developed a diffuse morbilliform rash. On initial exam, vitals were within normal limits and temperature was 39C. Laboratory data revealed white blood cell count within normal limits, hemoglobin 7.9 gm/dL, platelet count 71 K/mcl, INR 1.1, AST 878 unit/L, ALT 1,117 unit/L, AKI, and peripheral eosinophilia. Viral hepatitis serologies, autoimmunity studies, as well as viral markers for EBV, CMV, HSV, and varicella were negative. Ceruloplasmin, Alpha 1 antitrypsin, and acetaminophen levels were within normal limits. N-acetylcysteine was administered due to suspicion for drug-induced liver injury. Given that he had taken allopurinol two weeks before presentation, presented with fevers, rash, and renal impairment and now developed peripheral eosinophilia, DRESS syndrome secondary to allopurinol was diagnosed.

DISCUSSION:

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a rare, potentially life-threatening, drug-induced hypersensitivity reaction. Diagnosis is suspected in a patient who received a drug treatment in the previous two to six weeks and subsequently presents with characteristic symptoms including skin rash, liver involvement, hypereosinophilia, and lymphadenopathy. Dress typically causes transaminase elevations, but usually not in the 1000s, as was seen in our patient. The pathogenesis is hypothesized to involve a genetically deficient detoxifying enzyme resulting in an accumulation of drug metabolites. Supportive therapy and prompt withdrawal of the offending agent is the primary treatment for drug hypersensitivity reactions. Additionally, N-acetylcysteine has been shown to significantly increase survival in cases of drug-induced liver failure.
2472 Right Ventricular Mass in a Patient With Newly Diagnosed Cirrhosis
Authors: Li Muyi , O'Brien Corey , Syed Bakhtiar
Published: October 2019

INTRODUCTION:

While most cases of hepatocellular carcinoma (HCC) metastasis present in the lung, lymph nodes, adrenals, or bones (18%), hematogenous spread to the heart is also possible via the inferior vena cava (IVC). Worldwide prevalence of HCC is 180 million people, however only 2% present with cardiac involvement. Prognosis is typically poor due to high chance of cardiopulmonary collapse. Here we describe a patient presenting with cardiac mass concerning for HCC.

CASE DESCRIPTION/METHODS:

58-yo male with polysubstance abuse initially presented to the ED with fatigue, dyspnea on exertion, and 30 lb weight loss over a period of 6 months. On admission his labs were notable for elevated LFTs, albumin 2.7, and INR 1.28. He received an echo to rule-out endocarditis, and echo was concerning for a right ventricular mass. He also received CT chest as work-up for dyspnea, which not only revealed emphysematous changes but also a nodular liver. To better characterize the mass, he underwent cardiac MRI showing large RV mass extending into right ventricular outflow tract and the pulmonary trunk with an adjacent thrombus. Hepatitis panel was also positive for active hepatitis C. Given newly diagnosed hepatitis C and imaging/lab findings suggestive of cirrhosis, patient underwent triple phase MRI with findings of infiltrating HCC as well as extensive tumor thrombus involving both the right and left portal veins. The right ventricular mass was thought to be in setting of HCC metastasis with invasive liver disease. Patient had goals of care conversation and stated he did not want any further testing or procedures. He was discharged home with lovenox 100 mg daily for the right ventricular thrombus.

DISCUSSION:

This case highlights a rare presenting feature of HCC, as only 2% of HCC cases have cardiac involvement. Typical cardiac involvement occurs with hematogenous extension into the IVC, and while HCC with tumor extension to the portal system is quite common invasion to the IVC is rare. This patient was at risk for HCC with his untreated HCV cirrhosis, with typical 25 to 30-year latency period from exposure to development of HCC. Prognosis with cardiac involvement of HCC is poor due to high chance of cardiopulmonary collapse. Treatment considerations include systemic therapy such as sorafenib or lenvatinib in Child A patients, tyrosine kinase inhibitors that reduce angiogenesis and increases apotosis of HCC cells. Palliative cardiac surgery can also be considered in cases of obstruction.
2477 Strongyloidiasis Presenting as Isolated Hepatic Mass in an Immunocompetent Host: A Case Report
Authors: Wong Natalie Z. , Stotts Matthew J. , Daskalaki Irini , Siegelman Evan S. , Rothstein Kenneth D.
Published: October 2019

INTRODUCTION:

Strongyloidiasis is caused by an infection with Strongyloides stercoralis. Manifestations range from absent to minimal symptoms in immunocompetent persons to a hyperinfection syndrome in immunocompromised persons. Hepatobiliary complications have been described as granulomatous hepatitis and biliary obstruction. We report a case of strongyloidiasis presenting as a solitary hepatic mass in an immunocompetent patient.

CASE DESCRIPTION/METHODS:

A 27-year-old woman with no past medical history or medications and recent travel to Costa Rica presented with intermittent right upper quadrant pain after trauma while skiing. She had no hepatomegaly, tenderness, or signs of chronic liver disease on exam. Laboratory analysis showed WBC 24.7 103/mL, absolute eosinophils 15,314 cells/mL, AST 21 U/L, ALT 35 U/L, ALP 106 IU/L, albumin 3.9 g/dL, and total bilirubin 0.3 mg/dL. Strongyloides IgG was positive. Stool was unremarkable. CEA, AFP, and CA 19-9 levels were normal. Abdominal US revealed a hepatic mass. MRI confirmed a 5 × 6 cm lesion in the inferior and anterior right lobe (Figure 1). The patient received ivermectin for strongyloidiasis. Repeat MRI at 4 weeks showed no change in lesion size. To prepare for lesion excision, the patient underwent CT imaging at 7 weeks. This showed a significant decrease in mass size to 3 × 2.5 cm (Figure 2). Surgery was deferred. MRI at 15 weeks showed continued resolution of the mass to a long axis of 2.5 cm. Marked lesion improvement with ivermectin alone was felt consistent with strongyloidiasis.

DISCUSSION:

Chronic strongyloidiasis affects 30 million people worldwide, with clinical significance mediated by equilibrium between human host and helminthic parasite. Most people are asymptomatic or have nonspecific gastrointestinal symptoms. Hepatic presentation is exceedingly rare and has been associated with immunocompromise and dissemination. To our knowledge, only one case of immunocompetent hepatic presentation has been reported.
1
In the presently described case, a large hepatic lesion in a healthy woman with marked eosinophilia was initially evaluated for a neoplastic process. Strongyloides consideration and testing was key to obtaining the diagnosis. Imaging also expanded our understanding of the range of possible radiographic findings in this disease. Physicians should be aware of chronic strongyloidiasis as they consider the differential for hepatic masses in immunocompetent patients.
2484 Intravenous Acetaminophen Associated With Hepatotoxicity in an Orthotopic Liver Transplant Patient
Authors: Khuc Thi , Selim Khaled
Published: October 2019

INTRODUCTION:

Intravenous acetaminophen (IV APAP) is a commonly used post-operative analgesic. In adults, IV APAP administered at therapeutic doses of less than 4 g over 24 hours is considered safe. Five to ten percent of APAP is metabolized into N-acetyl-p-benzoquinone imine (NAPQI), the toxic metabolite responsible for APAP hepatotoxicity. NAPQI is inactivated when conjugated to tripeptide glutathione.

CASE DESCRIPTION/METHODS:

A 69 year old male with a history of secondary biliary liver cirrhosis status post orthotopic liver transplantation in 2006 and newly diagnosed prostate adenocarcinoma was admitted to the Urology service for planned robotic-assisted prostatectomy. He underwent uncomplicated robotic-assisted prostatectomy, requiring no fluid resuscitation, vasopressor support or blood products. Post-operatively, vital signs remained normal with no episodes of hypotension. He was started on IV APAP 1000 mg every 6 hours for 36 hours, followed by PO APAP 650 mg every 6 hours for post-operative pain. On post-operative day 2, AST was 2751 IU/L, ALT 3447 IU/L, TBili 1.9 mg/dL and INR 1.5 (Figure 1). All orders for acetaminophen were stopped. Liver Doppler ultrasound was normal. A serum acetaminophen level drawn 10 hours after the last IV APAP dose was <15 ug/mL. Work up for infectious etiologies was negative. The patient was started on IV NAC therapy. Following completion of IV NAC therapy, AST was 986 IU/L, ALT 2340 IU/L, TBili 1.9 mg/dL, and INR 1.4. The patient was discharged home on post-operative day 4. Labs during his outpatient Hepatology follow-up appointment 4 days after discharge were AST 62 IU/L, ALT 426 IU/L, TBili 1.3 mg/dL, and INR 1.1.

DISCUSSION:

This is the first case of acute liver toxicity in a post-OLT patient who received therapeutic doses of IV APAP. There was no evidence of hypotension or APAP overdose in the electronic medical records. All other etiologies for acute liver toxicity were ruled out, including shock liver, infection and vascular compromise. It is possible APAP metabolism is altered in transplant livers compared to native livers. A study on 13 liver transplant patients concluded APAP metabolism was transiently altered in orthotopic livers following liver transplantation, resulting in enhanced NAPQI production. It is possible transplant livers carry less glutathione stores due to cold storage preservation and post-transplant reperfusion, rendering them more susceptible to hepatotoxicty from NAPQI concentrations that a native liver would tolerate.
2485 An Unusual Case of a Primary Mass in the Porta Hepatis
Authors: Sidhu Simran , Gonzalez Hector , Azhar Mishah , Steinberg Stephen
Published: October 2019

INTRODUCTION:

Burkitt's Lymphoma is an aggressive subtype of B-cell non- Hodgkin Lymphoma which can present in three forms: endemic, sporadic, and immunodeficiency- associated. It commonly presents in the pediatric population and occurs in less than 1% of adult non-Hodgkin lymphomas. It classically presents with abdominal symptoms with ascites and can commonly involve the distal ileum, stomach, cecum, and mesentery. We describe an unusual case of Burkitt's lymphoma presenting as a mass in the porta hepatis, associated with obstructive jaundice.

CASE DESCRIPTION/METHODS:

A 74 y/o male presented with a two-week history of jaundice and intermittent epigastric pain. Physical exam findings were scleral icterus, jaundiced skin, and soft nontender abdomen with normal bowel sounds. Labs were pertinent for alkaline phosphatase 1,264, AST 166, ALT 95, total bilirubin 18.8, and direct bilirubin of 15.1. Ultrasound and MRI/MRCP demonstrated a 10.3 × 6.8 × 7.2 mass infiltrating the porta hepatis, peripancreatic, and periduodenal tissues. There were no other masses or adenopathy. At ERCP there was a friable mass replacing the ampulla. Cholangiogram showed dilated intrahepatic ducts proximal to an obstruction at the level of the common bile duct and a metal expandable stent was placed. Biopsy of the ampullary mass revealed high grade, mature B-cell lymphoma. Endoscopic ultrasound revealed a heterogenous mass and fine needle aspiration yielded necrotic cells. Tumor cells were positive for CD20, PAX5, BCL6, CD10, CD20, and LEF1. Pathology was positive for Myc rearrangement and translocation t(8;14). Combination chemotherapy was then initiated.

DISCUSSION:

Primary tumors of the porta hepatitis are uncommon as it is more often the site of metastatic disease from breast, pancreas, gastric, and colon cancer. This case is particularly unusual in that the primary tumor is a Burkitt lymphoma, which did not arise from lymph nodes or from the pancreas and which presented with obstructive jaundice.
2486 Pick the Cause: Streptococcus Intermedius and Mitus/Oralis Hepatic Abscess Due to Occult Gastrointestinal Perforation
Authors: Martin Trent D. , Raymond Patricia L.
Published: October 2019

INTRODUCTION:

Abscess bacteria identification directs both evaluation and management. In our case, identification of Strep intermedius and mitus/oralis led to identification of an unsuspected perforation.

CASE DESCRIPTION/METHODS:

A 45 y/o female presented to the ED with 5 days of fevers to 103.5 F, headache, cramping RUQ abd pain, diarrhea. CT abd revealed a 6.8 cm hypodense septated collection in the liver. Abnormal labs: WBC 32K TBili 1.3 AP 210 ALT 45. History was negative for IVDA, FH and personal hx of IBD, blood in stool, or travel. IR placed a drainage catheter on HD#1 and sent 60 cc purulent material for culture, gram stain with GPC. Culture several days later revealed sensitive Strep intermedius and Strep mitis/oralis in abscess and one anaerobic blood culture. The patient was treated with vancomycin and zosyn. Echocardiogram HD#3 showed minimal mitral calcification and no vegetations. Despite appropriate antibiotics, the RUQ pain, fevers, and WBC persisted. Repeat CT Abd on HD#7 showed a second hepatic abscess (IR placed second drain) and new pelvic abscess measuring 3.5 × 6 cm. On HD#9 we pursued occult GI causes of the persistent infection. Colonoscopy revealed a 1 cm adenoma (snared/removed) with no colon cancer, perforation, or IBD. EGD revealed a toothpick embedded in the prepyloric stomach; this was removed by snare. The patient did not recall eating food with a toothpick or swallowing one. Patient was discharged to home on HD # 11 with a PICC line to complete Unasyn course. Drainage catheters were removed 29 days after initial presentation; repeat CT scan at 52 days revealed no residual hepatic or pelvic abscess collections.

DISCUSSION:

Streptococcus intermedius is one of the members of the S. milleri group, which are normal flora in the mouth and gastrointestinal tract. S. milleri may cause liver, pelvis, and subphrenic abscess, cholangitis, peritonitis, and appendicitis. Presence of S. millerigroup bacteremia should direct attention to exclusion of occult abdominal perforation or infection, distant focal suppurative infection, and infective endocarditis. Streptococcus mitus/oralis is a common oral bacteria in the Strep viridans group; this may cause a variety of infections including cerebral, epidural, and liver abscesses and endocarditis. Upon the identification of Strep intermedius as the main abscess pathogen in our patient, prompt search for occult abdominal perforation and removal of the foreign body from the perforation tract resulted in resolution of both symptoms and infection.
2487 Primary Biliary Cholangitis With Autoimmune Hepatitis Overlap in Twin Males: A Unique Case of Related Autoimmune Diseases
Authors: Sanchez Antonio J. , Zhao Weidan
Published: October 2019

INTRODUCTION:

Primary biliary cholangitis (PBC) and autoimmune hepatitis (AIH) are immune-mediated liver diseases with female predominance. Combined features of both, termed AIH-PBC overlap syndrome, occurs in rare instances. We present a unique case of AIH-PBC overlap syndrome in twin males.

CASE DESCRIPTION/METHODS:

Twin #1 was a 36-year-old man when he presented in 2012 with asymptomatic liver enzyme elevation. No new medications, herbals, alcohol or illicit drug use. No known family history of liver disease. Labs showed total bilirubin (TB) 0.5, alkaline phosphatase (ALP) 478, aspartate aminotransferase (AST) 81, alanine aminotransferase (ALT) 111, gamma-glutamyl transferase (GGT) 625, positive anti-mitochondrial antibody (AMA) >1:640, and hypergammaglobulinemia (2128 mg/dL). Liver biopsy showed moderate plasma rich cell infiltrate with bile duct damage and mild fibrosis, consistent with AIH-PBC overlap. Patient was treated with steroids, azathioprine (AZA), and ursodeoxycholic acid (UDCA), later switched to obeticholic acid due to refractory PBC. He subsequently experienced marked biochemical improvement without disease progression. Twin #2 presented in 2017 with elevated liver enzymes and pruritus. Labs showed TB 1.1, ALP 755, AST 81, ALT 116, GGT 838, positive ANA >1:2560, positive AMA >100, and positive ASMA >100. Liver biopsy showed moderate bile duct epithelial injury with lymphoplasmacytic infiltrate, consistent with AIH-PBC overlap, and stage II/IV hepatic fibrosis. He was treated with steroids, AZA, and UDCA. He also experienced marked biochemical improvement without disease progression.

DISCUSSION:

PBC, a rare autoimmune disorder with female preponderance, can lead to cirrhosis and liver failure. PBC-AIH overlap syndrome, described in 4.8-9.2% patients with PBC, is more likely to have rapid disease progression and hepatic decompensation. Combination of steroids, AZA and UDCA has been variably effective and can delay disease progression. To the best of our knowledge, this is the first case of AIH-PBC overlap syndrome reported in twin males and highlights the importance of inherited factors in immune mediated liver disorders. Although it is known that PBC and AIH are associated with human leukocyte antigen genes on chromosome 6, further study of the association between these disorders is limited by the paucity of patients. Early diagnosis of this condition is essential to prevent liver related complications with medical management.
2494 Rare Case of Concomitant Acute Hepatic Failure and Renal Failure Induced by Intravenous Amiodarone
Authors: Mohamed Mujtaba , Al-Hillan Alsadiq , Hossain Mohammad
Published: October 2019

INTRODUCTION:

Hepatotoxicity caused by chronic oral amiodarone is well documented with around 15–20%. However, acute liver failure due to intravenous Amiodarone is rare clinical presentation with 3% incidence rate. Incidence of concomitant renal failure is even more rare. There is no full explanation for the underlying mechanism.

CASE DESCRIPTION/METHODS:

67-year-old male with PMH of CAD s/p coronary artery bypass graft, CKD, presented with chest pain for one week. In the ED, he was found to have atrial flutter. Due to unresponsiveness to IV beta blocker and Diltiazem, patient was loaded with IV Amiodarone and continued IV Amiodarone drip. 16 hours later patient developed acute hepatic failure with AST 4250 (10-42 IU/L), ALT 2422 (10-60 IU/L), INR 2.28 (2-3 conventional anticoagulation), acute renal failure with creatinine of 3.2 mg/dl (0.44-1.0 mg/dl), BUN of 44 mg/d (5-25 mg/dl). Patient was intubated due to acute hepatic encephalopathy. IV Amiodarone was stopped immediately. All workup for other causes of acute hepatic failure came back negative. He was started on intravenous N-Acetyl cysteine and required hemodialysis for acute renal failure. LFTs peaked 72 hours after discontinuation of Amiodarone. Kidney functions improved 5 days after discontinuation of amiodarone and hemodialysis stopped.

DISCUSSION:

Acute hepatic failure due to intravenous amiodarone is extremely rare with incidence of 3%. The underlying mechanism is still not fully understood. However, a case–control retrospective study argues that hepatotoxicity due to intravenous amiodarone cannot be differentiated from ischemic hepatitis from concomitant hypotension. Intravenous amiodarone is formulated with polysorbate 80 as a solubilizer, which has been shown to induce hypotension. In this case, patient had a stable blood pressure when amiodarone was started. Intravenous (IV) amiodarone toxicity can raise AST/ALT up to 100–200-fold (AST of 21000 and ALT of 9020 in our case) within a day of infusion which then reverses quickly after discontinuation of amiodarone. In this case, liver function tests increased after 26 hours of starting intravenous amiodarone and peaked after 72 hours of stopping the infusion. All cases of acute liver failure due amiodarone didn't survive except for one case. The most common cause of death is encephalopathy. Administration of N-Acetylcysteine was administered is associated with good outcome. Incidence of concomitant renal failure is extremely rare. It is caused by concomitant acute tubular necrosis.
2499 Idiopathic Eosinophilic Syndrome With Hepatic Involvement
Authors: Ali Ahmad , Dailey Francis , Hammoud Ghassan M. , Al-Juboori Alhareth , Rao Deepthi S. , Tahan Veysel , Tuncer Togla
Published: October 2019

INTRODUCTION:

Hepatic involvement in hypereosinophilic syndrome is rare.

CASE DESCRIPTION/METHODS:

A 66-year-old gentleman was referred for evaluation of persistent eosinophilia, cough, wheezing, and abnormal liver chemistries. No abdominal pain, fever, weight loss, and bowel habit changes. No traveling outside the U.S. On exam his vital signs were within normal range. Eyes, scleral icterus; Lungs; wheezing the bases; Abdomen, soft, nontender, nondistended, negative for, shifting dullness, no organomegaly, no distended veins on the abdominal wall; Extremities, no edema; Skin, no icterus no bruising. His initial laboratory findings were as follows, WBC 15.3 × 109/L, Hgb of 15.3 g/dL, platelets of 438 × 109/L, absolute neutrophil count of 6.7 × 109/L, absolute lymphocyte of 2.7 × 109/L, absolute eosinophil count of 4.5 × 109/L, alkaline phosphatase of 234 U/L, AST of 51 U/L, ALT of 94 U/L, total bilirubin of 0.4 mg/dL, albumin of 4.8 g/dL. With respect to the liver related laboratory findings: negative viral hepatitis panel, ANA of 1:160, total IgG of 1010 mg/dL, IgG1 of 659 mg/dL, IgG2 of 258 mg/dL, IgG3 of 12.9 mg/dL and IgG4 of 28 mg/dL; F-actin IgG antibody 23.3 units; negative anti-smooth muscle antibodies; soluble liver antibody <20 units; negative AMA, alpha-1 antitrypsin level of 167 mg/dL; and ceruloplasmin of 27 mg/dL. Comprehensive studies for stool ova and parasites was unremarkable. Peripheral blood smear showed eosinophilia. Bone marrow biopsy, with flow cytometric analysis revealing hypereosinophilia showing aberrant expression of CD 56. Abdominal MRI revealed a 5.8 cm hemangioma in the left lobe; no hepatic steatosis. Myeloproliferative neoplasms (MPN) and platelet-derived growth factor receptor α (PDGFRA A/B) workup was negative. Liver biopsy showed many eosinophils within portal areas with foci of sinusoidal involvement compatible with idiopathic eosinophilic syndrome; chronic hepatitis (grade 2/4), with portal and periportal fibrosis (stage II/IV); no steatosis. After starting prednisone 60 mg oral daily, tapered over 4 weeks, his cough, wheezing, hepatic enzymes' elevation and eosinophilia have resolved.

DISCUSSION:

Hypereosinophilic syndrome with hepatic involvement is rare, and should be in the differential diagnosis especially when hepatic enzymes' elevation is associated with eosinophilia.
2502 Rare Hepatic Adenomatosis Diagnosed by Endoscopic Ultrasound-Guided Fine-Needle Biopsy
Authors: Banerjee Avantika , Green William C. , Soape Matthew P.
Published: October 2019

INTRODUCTION:

While hepatic adenomas are uncommon (1:100,000), hepatic adenomatosis (HA), defined as >10 adenomas, is even more rare (1-2.4:1,000,000) and poorly understood. HA poses a significant risk of hemorrhage and progression to hepatocellular carcinoma. Diagnosis by percutaneous fine needle biopsy (FNB) poses potential risk of infection and bleeding, and can be made more safely with endoscopic ultrasound FNB (EUS-FNB). Data on safety and efficacy of EUS-FNB in diagnosis of HA is limited, however it has high efficacy in the evaluation of gastrointestinal tumors and solid pancreatic lesions. This rare case of HA highlights the scarcity and risk factors of the condition while presenting a minimally invasive approach for diagnosis.

CASE DESCRIPTION/METHODS:

A 43-year-old female presented with nausea, vomiting, diarrhea, vague epigastric pain, and unremarkable past medical history with 20-year history of oral contraceptive pill (OCP) use. Laboratory results were significant only for elevated liver function tests and quickly resolving leukocytosis and symptoms. She was diagnosed with uncomplicated gastroenteritis but an abdominal CT incidentally showed multiple hepatic lesions scattered throughout the liver. MRI confirmed diffuse hepatic steatosis and numerous liver lesions (maximum diameter 3.51 cm) (Figure 2), consistent with hepatic adenomatosis vs. metastatic cancer. EUS-FNB was performed with pathology revealing inflammatory type hepatocellular adenoma (HA-I) (Figure 3).

DISCUSSION:

Although little evidence correlates the development of HA with the use of OCP's, there is a notably higher prevalence in females, consistent with the known association of adenoma and exogenous estrogen. Our patient's female gender and 20-year OCP use are the only currently understood risk factors for her HA. They are structurally composed of an extensive sinusoidal network and have minimal connective tissue covering, expanding the risk of intralesional hemorrhage and hemoperitoneum. Continuing to diagnose this condition through minimally invasive EUS-FNB can further illuminate risk factors for progression as well as aid in the understanding of HA's pathology and risk for malignant transformation and hemorrhage.
2505 Pregnancy as a Confounding Factor in Autoimmune Hepatitis
Authors: Padial Doble Ian C. , Cruz-Cruz Jorge , Antunez Ivan , Pastrana Rafael
Published: October 2019

INTRODUCTION:

Autoimmune hepatitis (AIH) has a variety of clinical phenotypes. AIH presenting with acute liver failure (AFL) and jaundice is generally uncommon, however, other possible liver etiologies may confuse and delay diagnosis. Prompt recognition of AIH as possible cause of ALF is crucial in order to prevent morbidity and mortality associated with ALF. We present an atypical presentation that may help future clinicians in the early recognition and diagnosis of this condition despite possible confounders.

CASE DESCRIPTION/METHODS:

This is the case of a 39-year-old female (G2P1A0) with no history of systemic illness with intrauterine pregnancy of 32 5/7 weeks gestational age that was consulted to our service due to elevated transaminase and bilirubin levels. Patient had originally arrived at OB/GYN Emergency Department with complaints of nausea, vomits, jaundice and dark urine; no acholia, fever, or change in mental status noted. Additionally, patient reported recent use of oral antibiotics for UTI. On arrival patient presented hyperbilirubinemia (11.86) with direct predominance, elevated AST/ALT (251/134) and coagulopathy (INR 1.76). Patient with sudden clinical deterioration including altered mental status. Differential diagnoses included Acute Fatty Liver of Pregnancy (AFLP), Drug Induced Liver Injury, and preeclampsia with severe features. Due to suspected AFLP she was provided with IV steroid for fetal lung maturation and emergent C-Section preformed. MRCP reported no intra or extra hepatic bile duct dilation and liver US with Doppler noted with patent vasculature. Labs remarkable for negative ANA, ASMA, AMA, Anti DS DNA among other work up. After delivery patient had slow improvement of AST/ALT and bilirubin until 5 days later when AST/ALT and bilirubin levels began to increase steadily. Patient transferred to liver transplant center and empiric steroids started. Liver biopsy preformed and findings compatible with AIH but not diagnostic. After starting steroids patient began with slow improvement and was discharged on oral steroids. At post-hospitalization clinic visit, AST/ALT and bilirubin continued to improve and patient started on Azathioprine.

DISCUSSION:

It is suspected that initial brief improvement of liver function was secondary to steroids provided for fetal lung maturation, which lead us preemptively classify the ALF secondary to AFLP instead of AIH. This case is a prime example of pregnancy as a possible confounding factor that may delay the recognition and diagnosis of AIH.