bluuebunny/crossref_metadata_2025_split huggingface dataset:
2332 Large Hepatic Arteriovenous Fistula Leading to Non-Cirrhotic Portal Hypertension and Associated Complications
Authors: Le Thanh-Truc , Jencks Kara J. , Parupudi Sreeram 
Published: October 2019

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.
2335 Bladder Varices Causing Hematuria and a New Diagnosis of Cirrhosis
Authors: Calogero Cristina , Halegoua-DeMarzio Dina , Fenkel Jonathan 
Published: October 2019

INTRODUCTION:
A known consequence of portal hypertension is the development of varices, which are described as “ectopic” when located at unusual sites in the abdomen. Ectopic varices carry a mortality rate as high as 40% after initial hemorrhagic episode. We report a rare case of bladder varices as the presenting symptom for a new diagnosis of cirrhosis.

CASE DESCRIPTION/METHODS:
A 62yo female with a history of treated chronic hepatitis C (HCV) presented to an outside hospital with two days of gross hematuria. She was transferred to our center for further management. She had completed eight weeks of glecaprevir-pibrentasvir a few months prior to admission. On physical exam, she was an obese female without scleral icterus or jaundice, nor cutaneous stigmata of liver disease. Past imaging from six months prior to admission was notable for hepatomegaly only and not cirrhosis. Labs were significant for a hemoglobin of 7.6 and MELD-Na = 7. HCV RNA was undetectable. CT imaging of her abdomen/pelvis revealed cirrhosis with varices present along the right bladder wall in connection with the right internal iliac vein and the portal venous system. We performed a transvenous hepatic venogram and pressure study with biopsy. Liver pathology confirmed the presence of chronic hepatitis and cirrhosis and the hepatic venous pressure gradient was 13mm Hg. Urology placed the patient on continuous bladder irrigation and then performed a cystoscopy that revealed large, tortuous veins along the right lateral and posterior bladder, consistent with varices. She received an IV octreotide infusion with some improvement in hematuria, but not cessation. Multidisciplinary discussion resulted in our interventional radiology (IR) team performing a modified balloon-occluded retrograde transvenous obliteration, accessing the varix successfully via percutaneous transhepatic approach through the superior mesenteric vein and into the bladder varix. The hematuria resolved and the patient was safely discharged two days later.

DISCUSSION:
The bladder is an uncommon site for ectopic varices to form and hematuria is not often a symptom associated with cirrhosis or portal hypertension. This presentation was even more uncommon as the patient was not previously known to have cirrhosis. Cross-sectional imaging was key to the diagnosis in this case. Recognition of this rare event, combined with multidisciplinary management, helped this patient have a successful outcome.
2340 Almost Fatal: An Unusual Case of Focal Nodular Hyperplasia in Cirrhotic Liver Mimicking Cholangiocarcinoma
Authors: Ramachandran Preethi , Oluyadi Fatai , Boypati Lakshmi , Yadav Ruchi , Avezbakiyev Boris 
Published: October 2019

INTRODUCTION:
Focal nodular hyperplasia is a benign tumor of the liver, second most common after hemangioma. Most commonly, it is incidentally found in reproductive-aged women. It accounts for 8% of all primary liver tumors. Radiologically these regenerative nodules closely mimic intrahepatic cholangiocarcinoma and may be misdiagnosed leading to fatal outcomes. In most cases, the histopathological examination of a biopsy specimen establishes a definitive diagnosis. It is a rare entity for FNH to present in cirrhotic liver and as multiple nodular lesions (>5 in number).

CASE DESCRIPTION/METHODS:
An obese 66-year-old woman with a past medical history of diabetes presented with recurrent episodes of hematemesis and melena. Physical examination revealed a soft abdomen with hepatomegaly. Laboratory examination revealed microcytic anemia with hemoglobin of 7.1 g/dL and MCV of 73 fL. Liver function tests were within normal limits. Esophagogastroduodenoscopy revealed bleeding medium sized gastric body ulcer which was clipped. Abdominal computed tomography (Fig. A) and Magnetic resonance imaging (Fig. B) revealed multiple ( >5) ill-defined slow enhancing liver lesions, the largest of which measured 5.7 × 7.8 × 7.4 cm mass. Hepatitis screen and tumor markers including CA 19-9, CEA, AFP were negative. Liver cirrhosis was thought to be secondary to nonalcoholic steatosis as her work up did not reveal any other cause. Magnetic resonance cholangiopancreatography (Fig. C) revealed cirrhotic liver and multifocal lesions including a 9 × 8.1 × 11.9 cm mass in the right lobe and additional multiple small lesions in the left lobe all showing a rim of enhancement in the arterial phase, centripetal enhancement in venous phase, delayed washout in late phase and with associated overlying capsular retraction. Biopsy of the liver lesions revealed focal nodular hyperplasia with underlying fibrosis (Fig. D, E). with no evidence of malignancy. Our case is a very rare mimic of multifocal cholangiocarcinoma due to similarities radiologically which without a biopsy could have led to a misdiagnosis and wrong management.

DISCUSSION:
FNH usually presents as a single lesion in a normal liver parenchymal tissue. When diagnosed and symptomatic, treatment modalities include surgical removal or in some cases, trans-arterial chemoembolization. Our patient is a very unique case of FNH presenting as multinodular lesions in a cirrhotic liver. Since she remains asymptomatic, she will be actively monitored with a regular ultrasound screening.
2343 Trimethoprim-Sulfamethoxazole Induced DRESS Requiring Liver Transplant
Authors: Iqbal Sara , Chow Stephen , Amankonah Thomas 
Published: October 2019

INTRODUCTION:
Trimethoprim-Sulfamethoxazole induced DRESS requiring Liver Transplant.

CASE DESCRIPTION/METHODS:
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome can occur in the setting of DILI and is indicative of an idiosyncratic reaction which is now increasingly recognized with antibiotic use. The pathophysiology of DRESS is incompletely understood, but thought to involve reactivation of herpes viruses, genetic deficiency of detoxifying enzymes and associations between human leukocyte antigen (HLA) and drug hypersensitivity, leading to overwhelming response by the immune system. DILI-associated DRESS is rarely refractory to medical treatment requiring liver transplantation. We report a case of a 21-year-old male who presented with acute liver injury preceded by two weeks of nausea and vomiting. Prior to onset of symptoms, he was started on trimethoprim-sulfamethoxazole (TMP-SMX) for penile discharge. On presentation, he had a fever of 100.5°F, right upper quadrant abdominal pain and an erythematous rash involving upper extremities and later spreading to his face, trunk and lower extremities. Review of his records showed normal aspartate aminotransferase (AST) and alanine aminotransferase (ALT) from a week prior to admission but repeat labs showed an AST of 1480, and ALT of 764. He was found to have an elevated prothrombin time (PT) and INR but no signs of encephalopathy. Complete blood count with differential showed peripheral eosinophilia. Workup for infectious, autoimmune, and metabolic etiologies returned negative and broad-spectrum antibiotics were started for recurring fevers. European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) score of 4-5 placed the patient as ‘Probable DRESS’ with the most likely culprit being TMP-SMX. Skin punch biopsy showed subacute spongiotic dermatitis with eosinophils consistent with DRESS. Due to worsened liver function a liver biopsy was obtained which showed lobular hepatitis with sub-massive hepatocyte necrosis and increased eosinophils. The patient developed encephalopathy and successfully underwent liver transplantation with resolution of his symptoms post-transplant.

DISCUSSION:
This case demonstrates the importance of early evaluation, diagnosis and management of acute liver in patients with indication of DRESS Syndrome. It also emphasizes the importance of early transfer of patients with significant liver involvement to a liver transplant center as it can improve clinical outcomes.
2354 Massive Hematochezia Due to Bleeding Cecal Varices in a 35-Year-Old
Authors: Yalamanchili Silpa , Long Amanda , Mikhail George , Mathur Malini 
Published: October 2019

INTRODUCTION:
Bleeding from cecal varices is a rare complication of portal hypertension. We present a case of bleeding cecal varices manifesting as massive hematochezia in a 35 year-old woman with alcoholic cirrhosis.

CASE DESCRIPTION/METHODS:
A 35yo woman with chronic alcoholism presented with hematochezia, hypotension, and syncope. She endorsed a 15-year history of heavy alcohol intake, with no known liver disease or prior GI bleeding. Vital signs included HR 112 bpm and BP 83/48 mmHg. Labs indicated hemoglobin 6.6 g/dL, platelet count 58 K/uL, BUN 12 mg/dL, INR 1.5. Upper endoscopy showed no esophageal varices or blood. Colonoscopy showed red blood in the entire colon causing impaired visualization of underlying mucosa. The patient had continued hematochezia and hypotension requiring massive transfusion. CT angiography revealed active bleeding in the cecum and proximal ascending colon with abnormal surrounding vascularity, consistent with bleeding cecal varices (Figure 1). Arteriography demonstrated rectal, splenic, gastric, and small esophageal varices. The patient underwent transhepatic intravenous portosystemic shunt (TIPS) with an associated portosystemic gradient decrease from 24mmHg to 14mmHg. She had no further bleeding and was started on Nadolol for further reduction in portosystemic pressure. The patient was discharged in stable condition.

DISCUSSION:
Bleeding colonic varices are an uncommon manifestation of portal hypertension, with an incidence of 0.07%. Specifically, bleeding cecal varices are far more rare, and have been reported in less than 20 cases in the literature. This report describes a case of bleeding cecal varices in a 35 year-old with alcoholic cirrhosis; there is only one other report of bleeding cecal varices in a younger patient. Hemorrhage from these lesions is life threatening and requires robust knowledge of diagnostic and therapeutic options. Diagnosis is often attempted with colonoscopy, however can be difficult due to visual obscuration by bleeding. Mesenteric angiography is most effective in diagnosis. Endoscopic and radiologic interventions include cyanoacrylate injection, endoscopic variceal ligation, coil embolization, balloon occluded retrograde transverse obliteration (BRTO), and TIPS. In our case, the decision to pursue TIPS was made due to complicated superior mesenteric artery anatomy precluding coil embolization. Aggressive resuscitation and collaboration between gastroenterology, surgery, and radiology made for a successful outcome for this young patient.
2359 Acute Liver Failure in the Setting of HSV-Induced Hemophagocytic Lymphohistiocytosis
Authors: Amer Kamal , Block Peter , Hong Jennifer , Tofani Christina 
Published: October 2019

INTRODUCTION:
Acute liver failure (ALF) is characterized by acute (< 26 weeks) liver injury, encephalopathy, and impaired synthetic function (INR ≥ 1.5) in a patient without a preexisting liver disease carries a poor prognosis1. Common etiologies of ALF include acetaminophen toxicity, sepsis, and toxin exposure2. Here, we present a rare case of ALF secondary to HSV-induced Hemophagocytic lymphohistiocytosis (HLH).

CASE DESCRIPTION/METHODS:
A 57-year old male with a history of hypertension presented with a week history of cough and fever. Review of systems also included fatigue, decreased appetite, and decreased interactiveness. Labs were remarkable for anemia, thrombocytopenia (plt 15,000), tranaminitis (AST/ALT 6943/1551), ferritinemia (>105,000 ng/mL) and hypofibrinogenemia (134 mg/dL). Workup for causes of ALF was unrevealing, including acetaminophen, viral hepatitis, Wilson Disease, and autoimmune hepatitis. Blood and urine cultures, respiratory viral PCR, Adenovirus PCR, HIV Ab/Ag, Lyme IgM/IgG, ANA and RF were negative. HSV and EBV antibodies were collected early in admission. A CT Chest was unremarkable and a CT abdomen showed diffuse hepatic hypo-attenuation. His course was complicated by hypothermia, hypotension, and unresponsiveness prompting intubation and admission into the ICU. He was started on broad spectrum antibiotics as well as high dose steroids. His clinical status continued to deteriorate after-which his family requested comfort care measures. Postmortem autopsy demonstrated hepatomegaly with HSV hemorrhagic and necrotic hepatitis resulting in confluent non-zonal hepatic necrosis, as well as splenomegaly. Postmortem his HSV IgM (type 1 and 2) was not detected. His serum EBV IgM and IgG elevated with PCR showing 2246 copies/mL.

DISCUSSION:
This was a case of ALF due to suspected HSV-induced HLH. In review of the literature, only one other case study has reported ALF in the setting of HSV-induced HLH3. The diagnosis of HLH clinically requires 5/8 criteria [Table 1]4. Our patient met five of eight criteria for HLH. Notably, both EBV and HSV have been strongly implicated as potential triggers for HLH5. Although primary liver disease and HLH can present in a similar fashion, HLH tends to incorporate more extensive organ involvement, cytopenias, extremely high ferritin, and neurologic findings. Our case a rare precipitating cause of ALF, highlighting the importance of considering HSV-induced HLH early in the course of newly diagnosed ALF given their rapid deterioration.
2361 A Rare Presentation of Acute Hepatitis in the Setting of Wilson’s Disease
Authors: Al-Salameh Nizar , West Sara , Walker John T. , Smith Robert 
Published: October 2019

INTRODUCTION:
Wilson’s disease is a genetic disorder that leads to impairment in the transportation of cellular copper. The worldwide incidence of Wilson’s disease is estimated at one per 30,000 live births. Autoimmune hepatitis (AIH) has a varying degree of prevalence, anywhere from 0.9-25 cases per 100,000 globally. We describe a rare case of a patient presenting with acute hepatitis in the setting of known Wilson’s disease with nonadherence to therapy who was diagnosed with concomitant autoimmune hepatitis.

CASE DESCRIPTION/METHODS:
A 19-year-old woman with a history of biopsy-proven Wilson’s disease diagnosed at age 17, non-adherent to trientine chelation therapy, presented to her primary care physician with nausea and abdominal pain. Physical exam was benign and workup revealed an elevated aspartate aminotransferase (AST) and alanine aminotransferase (ALT) of 1500. She was referred for hospital admission. Acute viral hepatitis was ruled out and trientine was resumed as it was felt the presentation may be due to untreated Wilson’s Disease. Evaluation for drug-induced liver injury, human immunodeficiency virus, and thrombosis was negative. Despite therapy with trientine, transaminases continued to rise. Autoimmune serologies revealed a positive anti-smooth muscle antibody. A liver biopsy showed copper granules in hepatocytes consistent with Wilson’s disease, in addition to moderately active hepatitis with frequent plasma cells supporting the diagnosis of AIH. Prednisone was initiated for treatment of AIH and her hepatitis rapidly improved. One week follow up showed further improvement in her liver chemistries. She was transitioned to azathioprine for AIH. Trientine was continued for treatment of Wilson’s disease.

DISCUSSION:
Given the multiple causes of acute hepatitis and targeted therapies available, determining the etiology remains the cornerstone of management. This is an unusual case of biopsy-proven Wilson’s disease who presented with acute hepatitis. She was found to have serology and biopsy consistent with both Wilson’s disease and AIH. She responded rapidly to steroid therapy for AIH followed by treatment with an immunomodulator. This combination of a rare inherited disease coupled with an uncommon autoimmune phenomenon highlights the need for diagnostic precision in the patient with acute hepatitis, even when pre-existing liver disease is known. Ultimately liver biopsy findings directed specific therapy.
2366 Acute Liver Failure Caused by Hemophagocytic Lymphohistiocytosis Secondary to Aggressive Natural Killer Cell Leukemia and Epstein-Barr Virus Reactivation
Authors: Khalaf Suha Abu , Yousef Mohamad , Albarrak Abdulmajeed , Kuwajima Vanessa , Ibdah Jamal A. , Tahan Veysel 
Published: October 2019

INTRODUCTION:
Acute liver injury is a common presentation of many systemic illnesses, including hematologic malignancies. The present case was an unusual presentation of acute liver failure (ALF) caused by hemophagocytic lymphohistiocytosis (HLH) secondary to aggressive natural killer cell leukemia(ANKL) associated with Epstein-Barr virus(EBV) infection reactivation.

CASE DESCRIPTION/METHODS:
A 24-year-old female presented with fever and altered mental status. Her physical examination revealed massive hepatosplenomegaly. Complete blood count showed pancytopenia with WBC 460/µL, microcytic anemia Hgb 7.8g/dL with MCV 79, thrombocytes 45,000/µL, AST 170IU/L, ALT 162IU/L, INR 1.5, albumin 1.3g/dl and total bilirubin 11.08mg/dL, triglycerides 1164mg/dL, ferritin 1792ng/ml. CT abdomen showed hepatosplenomegaly with hepatic steatosis. Her liver biopsy showed hepatic necrosis with moderate steatohepatitis and perisinusoidal fibrosis with normal liver copper level. She was initially treated with steroids and N-acetylcysteine. Flow cytometry and bone marrow biopsy revealed CD56+ and CD3+ positive cells with high serum soluble IL-2 receptor consistent with ANKL and HLH. EBV markers were found positive for EBV IgG antibody and EBV nuclear antigen with high EBV PCR and negative for EBV IgM antibody, which is consistent with EBV reactivation. A modified SMILE chemotherapy regimen with rituximab was initiated. Unfortunately, her clinical status continued to decline progressively with worsening acute encephalopathy, acute hypoxic respiratory failure and Pseudomonas septic shock which required vasopressors and IV antibiotics. The patient died on day-30 of hospitalization due to her hematologic malignancy, septic shock, and ALF.

DISCUSSION:
HLH is a consequence of abnormal immune activation which could be secondary to infections, malignancies, or autoimmune diseases; leading to life-threatening multi-organ inflammatory response, especially in the liver. Natural killer (NK) cells are types of lymphocytes that play an important role as part of innate immune system in fighting tumors and viral cells; NK cells are abundant in the liver. It was shown that NK cells might be involved in the intense immune response causing severe liver damage. ANKL is a rare malignant lymphoproliferative disorder of mature NK cells closely associated with EBV. EBV reactivation can occur in this disease setting. Careful evaluation of patients with ALF and abnormal hematologic profile is essential for early detection and therapy of the primary culprit.
2372 Atypical Infectious Mononucleosis From EBV in a Splenectomized Sickle Cell Disease Patient
Authors: Pillai Priyamvada , Mehta Amit , Wan David 
Published: October 2019

INTRODUCTION:
Infectious mononucleosis (IM) is a clinical entity usually presenting with fever, pharyngitis and lymphadenopathy. However, atypical features of IM in Sickle cell disease (SCD) patients post splenectomy have been rarely reported. Here, we present a case of an atypical IM from Epstein Barr Virus (EBV) in a SCD patient post splenectomy with concurrent sickle cell hepatopathy (SCH).

CASE DESCRIPTION/METHODS:
This is a 22-year-old young man with history of SCD post splenectomy, who presented with severe dull pain in his right knee and persistent fevers up to 38.3oC along with watery diarrhea for 2-3 days. The patient denied any sore throat, sick contacts or recent blood transfusions. Besides a history of transient hemochromatosis from blood transfusions, the patient denied any history of liver disease or immunosuppression. Clinical examination was significant for non-tender hepatomegaly and a small palpable lymph node in the left posterior cervical chain. Initial workup was significant for lymphocytosis, mild transaminitis with raised AST, ALT, ALP, total bilirubin and direct bilirubin. Blood and urine cultures, parasitic screen, hepatitis and autoimmune hepatic panel were all unremarkable. Imaging studies were only significant for hepatomegaly on Abdominal Ultrasound. Despite initial management with broad spectrum antibiotics, the patient continued to have high fevers and rising liver transaminitis. Subsequent investigations revealed a positive EBV IgM and a viral load of 6119 along with 19% atypical lymphocytes. The patient was diagnosed with atypical EBV infection with mild SCH and managed supportively with IV fluids, hydroxyurea and analgesics.

DISCUSSION:
IM often presents as a clinical triad of fever, pharyngitis and lymphadenopathy with EBV being the most common pathogen implicated. EBV has often been associated with a mild transaminitis in healthy adults. A similar pattern is also seen in SCD patients with SCH resulting in tender hepatomegaly secondary to vaso-occlusive hepatic ischemia. Hence, it can be difficult to distinguish an atypical presentation of EBV-IM from SCH. In our patient, although his liver transaminitis partially resolved post exchange transfusion, he continued to have rising fever and atypical lymphocytosis along with high EBV viral load indicating an active atypical EBV-IM with a concurrent mild SCH. Therefore, any mild transaminitis with cholestasis in SCD patients post splenectomy, should prompt an evaluation for atypical EBV-IM.