bluuebunny/crossref_metadata_2025_split huggingface dataset:
2209 Abdominal Arterial Aneurysms in a Patient With Budd-Chiari Syndrome
Authors: Srivats Sudarshan , Niec Rachel , Wan David 
Published: October 2019

INTRODUCTION:
Budd-Chiari syndrome (BCS) is defined as hepatic venous outflow tract obstruction. Chronic BCS eventually leads to post-sinusoidal hypertension resulting in the symptoms of the disease. There have been case reports of BCS leading to intra-abdominal bleeding from ectopic varices, however, these are cases of venous bleeding. Here, we report a unique case of a Chinese woman with BCS presenting with bleeding from arterial aneurysms.

CASE DESCRIPTION/METHODS:
A 61-year-old woman with BCS requiring multiple prior porto-systemic shunting procedures was brought by EMS for coffee ground emesis. At the ED, she was stuporous and developed bloody emesis and hypotension. She was intubated and transferred to the ICU. Exam was remarkable for abdominal distention (AD) with a fluid wave. Labs showed leukocytosis; prolonged coags, elevated troponins, lactate, LDH and decreased fibrinogen and haptoglobin. FAST showed intra-abdominal free fluid. EGD showed grade II varices in the esophagus that were banded. However, the patient continued to deteriorate with increasing AD. CT-AP revealed a cirrhotic liver with occluded left and middle hepatic veins and extravasation of contrast near the greater curvature of the stomach into a LUQ hematoma (Figure 1). IR embolized the left gastric artery and identified aneurysms along a branch of the SMA (Figure 2) in addition to an aneurysm at the celiac artery origin (Figure 3). On the 2nd post-op day, the patient again developed hypotension with AD. Attempted embolization of the SMA branch was unsuccessful. On the 5th post-op day, the family agreed to withdrawal of life support and the patient was extubated and vasopressors were stopped. The patient passed away in a few hours.

DISCUSSION:
Chronic BCS presents with complications of cirrhosis; i.e., ascites and rupture of esophageal varices, like in our patient. On CTA, both the hepatic veins and part of the IVC were occluded. In fact, 31% of BCS patients have outflow occlusion at both these locations. However, in our patient, CTA revealed aneurysms in the celiac artery and distal portions of an SMA branch along with left gastric artery bleeding. Multiple arterial aneurysms have been associated with disorders such as PAN, Behcet's, Ehler-Danlos etc. By comparison, there have not been many reports of multiple idiopathic arterial aneurysms in adults. Several of these possible causes were unable to be worked up for due to the acute presentation, although our patient’s history was not typical for these diseases.
2210 Resolution of Hepatocellular Carcinoma in a Patient With Chronic Hepatitis B Treated With Entecavir Therapy
Authors: Suri Jaspreet , Shah Pir Ahmad , Choudhry Saad , Nasser Imad A. , Lau Daryl T. 
Published: October 2019

INTRODUCTION:
Hepatitis B virus (HBV) is a major cause of hepatocellular carcinoma (HCC) globally. Prolonged HBV DNA suppression with nucleos(t)ide analogues is known to regress hepatic fibrosis and reduce HCC risk. This is an unusual presentation of a patient with hepatitis B-associated HCC whose liver lesion resolved on antiviral therapy alone.

CASE DESCRIPTION/METHODS:
Patient is a 64-year-old Chinese male with HBeAg negative chronic hepatitis B with persistent normal serum aminotransferases and was antiviral treatment naïve. He had a positive family history of HCC and was first noted to have asymptomatic elevation of alpha-fetoprotein (AFP) to 648.9 ng/ml 6 months prior to his referral to Hepatology. During his initial Liver Center visit, the AFP increased to 1,900 ng/ml with elevated AFP-L3 at 18.4%. His hepatitis B remained inactive with normal ALT and minimal detectable HBV DNA at 1.6 log IU/ml (Table 1). He was started on entecavir 0.5 mg daily in view of his age and suspicious HCC complication. Initial MRI of the abdomen revealed lesions in segment 4 (2 cm) and segment 7 (2.1 cm) of the liver with arterial enhancement, venous washout and pseudocapsule. Targeted liver biopsy of segment 7 lesion and adjacent liver was performed within 2 weeks on entecavir therapy. The targeted liver specimen revealed a necrotic focus with loss of reticulin with strong immunoreactivity for cytokeratin Hep Par1 and Glypican-3; the findings were highly suspicious for HCC. The adjacent liver had only mild inflammation (grade 0-1) and minimal fibrosis (stage 0-1). Six weeks later, AFP decreased to 19 ng/dl but AFP-L3 remained elevated at 17%. On repeat CT scan, the segment 7 lesion decreased to 1.2 cm and no longer met OPTN HCC criteria. Patient remained clinically stable with optimal HBV DNA suppression. By 7-month follow-up, both AFP and AFP-L3 normalized and the liver lesions remained stable in size (Table 1). He continued to have regular monitoring and the segment 7 liver lesion was no longer visualized on abdominal MRI after 2 years.

DISCUSSION:
Nucleos(t)ide analogue therapy is known to suppress HBV replication but has not been reported to have direct effect on HCC. This HCC case is unique as the liver lesion regressed and resolved on entecavir therapy alone. While the molecular mechanism leading to the HCC resolution is currently unknown, this case highlights the beneficial role of prolonged HBV DNA suppression with antiviral therapy.
2212 Hemophagocytic Lymphohistiocytosis Disguised as Acute Liver Injury
Authors: Hasan Badar , Yim Yunjoo , Castaneda Daniel , Rashid Mamoon Ur , Al Khalloufi Kawtar 
Published: October 2019

INTRODUCTION:
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disorder with unregulated immune activation and inflammation. Primary HLH occurs in infants or young children with or without family history whereas secondary HLH manifests in older children or adults without a family history or gene mutation. Concurrent infection, malignancy or rheumatological disorder usually trigger an uncontrolled immune response in the older age group. Our aim is to raise is awareness among physicians to recognize the early signs and symptoms of this life-threatening condition.

CASE DESCRIPTION/METHODS:
A 33-year-old female with a history of multiple sclerosis and rheumatoid arthritis presents with elevated LFTs, cholestatic jaundice and intermittent high-grade fever. Imaging studies showed no significant abnormality in the hepatobiliary system. Viral and autoimmune serologies were negative. Lab works revealed low fibrinogen and elevated ferritin and IL-2 receptor levels. Bone marrow biopsy was unremarkable; however, liver biopsy showed features suggestive of HLH. Steroid and etoposide therapy was started but treatment was complicated by pancytopenia and hemodynamic instability despite multiple transfusions and filgrastim administration. With worsening clinical pictures, emapalumab (Interferon gamma blocker) was started. However, the patient developed multiorgan failure with overwhelming sepsis and died within days of emapalumab administration.

DISCUSSION:
HLH has a wide spectrum of clinical manifestation often masquerading as sepsis or SIRS. Some studies have found that cholestasis and high AST levels are associated with poor outcomes in adult HLH patients. The goal of therapy is to suppress hyper inflammation with agents such as corticosteroids, calcineurin inhibitors, and immunoglobulins. When a patient presents with prolonged fever and liver dysfunction of unknown etiology, HLH should be considered in the differential diagnosis as early recognition and treatment can prevent mortality, which can be high up to 75 % in the adult age group.
2223 Zieve’s Syndrome: Case Report, Review of the Literature and Electronic Patient Database
Authors: Twohig Patrick , Desai Aakash , Sandhu Sonia , Sandhu Dalbir S. 
Published: October 2019

INTRODUCTION:
Zieve’s syndrome (ZS) is rare condition of alcoholic hepatitis, hyperlipidemia and hemolytic anemia1. Clinicians need to be aware of ZS for several reasons: First, hemolysis increases unconjugated bilirubin levels, leading to false elevation of the Maddrey’s discriminant function (MDF), which may lead to the unnecessary prescription of steroids.2 Second, the anemia that occurs from hemolysis may be misinterpreted as a gastrointestinal bleed, leading to extra endoscopic procedures. Finally, since patients can present with fever and abdominal pain, diagnosis may be construed for other causes such as sepsis or acute cholecystitis.2-5

CASE DESCRIPTION/METHODS:
A 39-year-old female with long-standing alcohol abuse had three successive admissions resulting in refractory ZS. We performed a review of the literature to assess other case reports of patients with ZS. A total of 38 patients were included, 8 in women and 30 in men. Patients’ age ranged from 28 to 65 years old (mean 45.6 years). Average total bilirubin was 9.12. Average hemoglobin was 9.15. Average total cholesterol was 486. Average LDH was 243. Most patients did not receive prednisolone and fully recovered with strict alcohol cessation. A retrospective analysis using the IBM Explorys database of over 63 million de-identified, unique patients in the United States revealed that of 17,990 patients with alcoholic hepatitis, only 80 had ZS. The odds of a patient with alcoholic hepatitis also having hemolytic anemia is 7. ZS is rare, only seen in 0.4% of patient’s with alcoholic hepatitis.

DISCUSSION:
Our patient was 39, younger than the average age of 45.6 years. She was also female, a gender seen in a minority of previously reported cases. She had a total bilirubin 3 times above average, and a hemoglobin 33% lower than average. Despite 12 units of packed red blood cells, prednisolone, and pursuit of inpatient/outpatient alcohol rehabilitation, she ultimately succumbed to her liver injury 6 weeks after initially presenting to the hospital. This is the first reported case of death from ZS. Clinicians should maintain a high index of suspicion for ZS in patients with alcoholic hepatitis and transfusion refractory anemia. Evaluating for hemolysis and hyperlipidemia are recommended. Alcohol abstinence is essential. There is no role for steroids. Early diagnosis is essential. Our goal is that increased case reporting will raise clinician awareness.
2224 A Rare Complication of Post-TIPS Related Acute Cholecystitis
Authors: Chiejina Maria , Samant Hrishikesh , Morris James D. 
Published: October 2019

INTRODUCTION:
Amongst the well documented TIPS-related biliary complications, there has been no reported case of acute cholecystitis. Multiple multicenter RCT comparing TIPS to large volume paracentesis reported better control of ascites, lower diuretic doses or cessation of diuretics in approximately 75% of the patients. Biliary related complications include biliary venous fistulas, biloma or biliary occlusion secondary to biliary compression. This may be the first reported case of Post TIPS acute cholecystitis.

CASE DESCRIPTION/METHODS:
67-year-old white male with end-stage NASH cirrhosis was hospitalized 6 months ago for diuretic-resistant ascites. Prior to admission, he has required weekly large volume paracentesis. While hospitalized, he underwent large-volume paracentesis and was treated for acute urinary tract infection. TIPS was placed two weeks post discharged and ascites improved. Three days later while undergoing further evaluation, a liver ultrasound showed gall bladder wall thickening and gall stones, suggestive of acute cholecystitis with cholelithiasis. He subsequently developed severe pancytopenia and worsening liver function test. A percutaneous cholecystostomy tube was placed for gall bladder decompression along with a 14-day course of IV antibiotics. He later developed Choledocholithiasis and required ERCP and cholangioscopy. Multiple stones were extracted from the bile duct and complete fibrosis of the cystic duct was noted. The patient required multiple hospitalizations since then, frequent paracentesis and treatment for SBP. He recently underwent OLT with cholecystectomy which was technically challenging.

DISCUSSION:
TIPS insertion has been established as an effective second-line treatment for refractory ascites. There are 3 documented category of TIPS complications; technical, portosystemic and unique shunt related but no known reported case of acute cholecystitis. Hepatic encephalopathy occurs in approximately 30% of patients, early thrombosis or delayed shunt stenosis occurred in 22- 50% of patients prior to the development of polytetrafluoroethylene-covered stents. The technical intricacy of this intervention poses a potential risk, and morbidity rates as high as 20%. A broad understanding of the uncommon and unique complications can potentially decrease post-op-related complications especially in a patient undergoing OLT It is necessary for clinicians who perform this procedure to be aware of the clinical spectrum of TIPS-related complications and their management.
2225 Hydrazine Sulfate: A Case Report of Drug-Induced Acute Hepatorenal Toxicity
Authors: Weigle Rachael L. , Carver Charles K. , Mank Gerald , Campanile Teresa , Burnette Tonya 
Published: October 2019

INTRODUCTION:
Hydrazine sulfate (HS) is a synthetically manufactured ammonia derivative marketed in the US as a dietary supplement, though many patients still use it for its previously proposed anticancer properties. HS inhibits phophoenol pyruvate carboxykinase thereby blocking gluconeogenesis. In investigations since the 1980s, it has been proven ineffective with respect to its ability to improve cachexia and limit rate of tumor growth as previously postulated. Also, multiple animal trials have shown an increase in the incidence of new lung, liver, and breast tumors with use of HS. It has been listed by the IARC Working Group as a possible carcinogen to humans. Below we discuss a case of acute hepatic and renal failure from HS use after daily ingestion for 24 days.

CASE DESCRIPTION/METHODS:
Patient is a 74-year-old-female with history of metastatic large cell neuroendocrine rectal tumor status post chemoradiation and resection of colon with ileostomy placement and vaginectomy in 2013. In February 2019, CT chest imaging showed multiple bilateral pulmonary nodules, the largest measuring 2.3 cm, with extensive lymphadenopathy and bilateral pleural effusions. In April 2019, patient presented to the hospital with acute encephalopathy with associated transaminitis, hyperbilirubinemia, coagulopathy, and acute renal failure. Subsequent testing included negative hemolysis workup, hepatic viral and autoimmune panel, and MRCP abdomen. Patient had not followed up with oncology aboutpulmonary nodules, and instead took HS 60mg TID for 4 weeks prior to presentation. The patient’s encephalopathy improved after several days of supportive care. She was followed in clinic by gastroenterology with eventual resolution of hepatorenal toxicity after multiple weeks with cessation of HS.

DISCUSSION:
HS has been proven to be ineffective as any form of cancer treatment, though patients continue take it due to false hopes of cure and its ability to induce a state of euphoria with neurotropic effects. Unfortunately, many patients develop fulminant hepatorenal toxicity, severe gastrointestinal hemorrhage, hepatic necrosis, and autolysis of kidneys instead. The diagnosis of HS toxicity is often overlooked, and if diagnosed in a timely manner, extensive and costly workups could be avoided. Cancer patients with poor prognosis or limited chemotherapeutic options remain at risk for falling prey to seemingly benign naturalistic remedies. This population must be better educated on the dangers of hydrazine sulfate.
2227 Herpes Simplex Virus - 1 Related Acute Fulminant Liver Failure Successfully Treated With Medical Therapy
Authors: Kataria Archish , Speeg Kermit V. , Gonzalez Lisandro 
Published: October 2019

INTRODUCTION:
Herpes simplex virus (HSV 1&2) is an uncommon cause of asymptomatic hepatitis and acute liver failure. Fatal disease often occurs in immunocompromised adults but is a rare event in immunocompetent patients. Early suspicion of infection is imperative as late diagnosis and treatment carries poor prognosis and high mortality. We describe a case of acute hepatic failure from HSV-1 virus in an immunocompetent adult successfully treated with medical therapy without need for liver transplantation.

CASE DESCRIPTION/METHODS:
A 55-year-old diabetic male with recent hospitalization for necrotizing fasciitis of the groin (discharged on amoxicillin/clavulanate 5 days ago) presented to the hospital with fever, vomiting, abdominal pain and was found to have severe elevation in liver enzymes. Patient denied prior history of liver disease. Physical exam was notable for fever: 102 degree F, tachycardia, multiple small circular lesions on the oral mucosa and tender hepatomegaly. Liver tests at baseline were normal and on admission showed: AST 1317 U/L (normal:< 32 U/L), ALT 966 U/L (normal:< 36 U/L), total bilirubin 0.4mg/dL (normal:0.2-1.2 mg/dL), alkaline phosphatase 86 U/L (normal:45-117 U/L) and INR 1.7 (normal:0.8-1.2). Urine drug screen was negative of acetaminophen. Imaging excluded hepatic venous thrombosis. Laboratory tests for viral and autoimmune hepatitis, wilson’s disease were negative. Patient was empirically started on IV acyclovir given concern for herpes hepatitis based on oral exam. Diagnosis was confirmed with a positive blood and oral swab HSV DNA PCR and a liver biopsy (Figures 1 and 2) showing herpes simplex viral inclusions. Due to progressive worsening of liver tests and patient’s condition while on treatment, intravenous immunoglobulin (IVIG) therapy was added eventually leading to clinical and laboratory signs of improvement (Table 1). He was discharged on an oral course of valganciclovir and continues to do well on follow up visit.

DISCUSSION:
Fulminant liver failure from herpes virus has been infrequently reported in immunocompetent adults and usually represents disseminated infection. It is a potentially treatable condition that requires high degree of diagnostic suspicion as presenting symptoms can be non-specific. Early empiric treatment with acyclovir while awaiting HSV PCR or liver biopsy has been shown to improve outcomes and decrease need for liver transplantation. We herein describe a novel approach to treat HSV hepatic failure with combination therapy of acyclovir and IVIG.
2228 A Rare Report of Pantoprazole-Induced Acute Hepatocellular and Cholestatic Hepatitis
Authors: Kataria Archish , Hubbard Hopethe , Page Robert 
Published: October 2019

INTRODUCTION:
Pantoprazole is a widely used proton pump inhibitor that is used to treat variety of acid related disorders. It is well tolerated with excellent safety profile. Mild transient elevations in serum aminotransferase can be seen with pantoprazole use that resolves without dose modification. Pantoprazole induced clinically apparent liver injury on the other hand is exceedingly rare.

CASE DESCRIPTION/METHODS:
A 47-year-old woman with no prior liver disease was referred to the hospital for acute hepatitis. She started pantoprazole 2 months prior to admission for gastritis. Patient had nausea, fatigue, abdominal pain, jaundice and dark urine 2 weeks after starting pantoprazole and stopped it a week prior to hospital admission. She denied alcohol use. Her current medications were levothyroxine, enalapril/hydrochlorothiazide and aspirin and no use of herbal supplements. Physical exam was notable for conjunctival icterus. Baseline liver enzymes were normal. Labs on admission were: AST 681 U/L (< 32 U/L), ALT 2100 U/L (< 36 U/L), total bilirubin 12.3 mg/dL (0.2-1.2 mg/dL), direct bilirubin 8.3 mg/dL (< 0.5 mg/dL), alkaline phosphatase 96 U/L (45-117 U/L) and INR 1.9 (0.8-1.2). Urine drug screen was negative for acetaminophen toxicity. Imaging excluded biliary obstruction and hepatic venous thrombosis. Laboratory investigations for viral and autoimmune hepatitis, wilson’s disease and hemochromatosis were negative. Liver biopsy (Figure 1) reported portal areas with edema and a minimal amount of inflammation composed of mixed lymphocytes, occasional eosinophils and neutrophils. The lobular parenchyma showed cholestasis with lymphocytic and neutrophilic inflammation. Patient noted improvement of symptoms with supportive care (Table 1). Two months after stopping pantoprazole, liver enzymes were normal.

DISCUSSION:
DILI (drug-induced liver injury) is a significant cause of acute hepatitis. To our knowledge, only 7 case reports of pantoprazole induced liver injury have been published. The timing of starting pantoprazole prior to acute hepatitis, negative serologies, and normalized liver enzymes after stopping suggests pantoprazole as the cause of DILI. Based on its pharmacokinetics, timeline and pattern of insult, an idiosyncratic reaction has been suggested as the mechanism for hepatotoxicity. We have described a rare case of acute mixed (hepatocellular and cholestatic) liver injury from pantoprazole use suggesting its consideration in the differential diagnosis of DILI.
2236 Metastatic Melanoma: A Rare Case of Liver Involvement
Authors: Marusca Georgiana , Selim Ranya 
Published: October 2019

INTRODUCTION:
Literature shows that cutaneous melanoma can metastasize hematogenously or via the lymphatic system. The reported median time course for distant metastases to develop for a tumor recurrence is 25 months. The anatomical location of the primary melanoma is considered the most important factor for the location of metastasis: 30% of patients with melanoma on the trunk or upper extremities develop direct distant metastases.

CASE DESCRIPTION/METHODS:
We present the case of a 68-year-old man with a history of diverticulosis, remote alcohol abuse and recently diagnosed melanoma of the scalp, who presented to the emergency room with complaints of sudden generalized abdominal discomfort, malaise, decreased appetite, fatigue and dark urine for one week. Eight months prior, the patient underwent a wide excision of a malignant melanoma on his left flank. In the emergency room, the patient was hemodynamically stable. Physical exam was notable for distended abdomen with dullness to percussion at the peripheries. Labs showed elevated liver function tests with ALT 98 IU/L, AST 144 IU/L, total bilirubin of 4.3 mg/dL, direct bilirubin of 2.8 mg/dL and INR of 1.46. Abdominal US showed right portal vein thrombosis. An abdominal MRI demonstrated hepatosplenomegaly with multiple lesions in the spleen and liver, small amount of perihepatic and perisplenic fluid with small right-sided pleural effusion as well as a 17 mm subcutaneous nodule in the left chest concerning for metastatic disease. A subsequent liver biopsy was consistent with metastatic melanoma. His hospital course was complicated by acute renal failure in the setting of hyperuricemia secondary to tumor lysis requiring dialysis. Unfortunately, he was unable to tolerate dialysis due to hypotension. Due to his overall clinical status, a family meeting was arranged and the decision was made to pursue hospice care. A few hours after enrolling in hospice, the patient expired.

DISCUSSION:
Cutaneous melanoma metastasizes to the lungs, lymph nodes and soft tissue, with only 10-20% of cases being liver metastasis. Liver metastasis, an important prognostic factor, occur more quickly after the primary lesion is detected. The median survival for patients with stage IV metastatic melanoma has been reported to be one year. Hepatic resection is considered the only potentially curative treatment for patients with liver metastases, with only 2-7% of patients being surgical candidates. Patients who are not surgical candidates can undergo systemic and/or regional therapies.