bluuebunny/crossref_metadata_2025 huggingface dataset:
2339 Hemobilia-Associated Acute Cholecystitis Post-Percutaneous Liver Biopsy: A Unexpected Sequelae of a Known Complication
Authors: Pakala Tina , Cohen Jason , Bunim Ari , Hussain Syed
Published: October 2019

INTRODUCTION:

Percutaneous liver biopsy is often a part of the diagnostic workup of unexplained abnormal liver enzymes. Known complications include hemobilia and unintentional perforation of an adjacent organ but rarely do these complications lead to sepsis.

CASE DESCRIPTION/METHODS:

We present a case of a 65-year old female being evaluated for abnormal liver enzymes (ALT 40 U/L, normal alkaline phosphatase) with a positive anti-mitochondrial (titer 1:1280) and anti-nuclear antibodies (titer 1:640). A percutaneous liver biopsy was performed under ultrasound guidance without any immediate complication. The biopsy revealed moderate-to-marked macrovesicula steatosis with focal centrilobular and periportal fibrosis (Stage 1 of 4). Five days later she presented to the emergency department with persistent RUQ pain, fevers and melena. Vitals notable for temperature of 39.3 °C. Physical examination revealed RUQ tenderness to palpation. The leukocyte count was 5.04 K/uL (predominantly neutrophils), AST 558 U/L, ALT 350 U/L, ALP 287 U/L, total bilirubin 1.9 mg/dL. CT abdomen demonstrated only cholelithiasis without other abnormalities. An abdominal ultrasound showed gallbladder sludge and wall thickening with a negative Murphy’s sign (Figure 1). Blood cultures were positive for Klebsiella pneumonia and IV antibiotics were started. Over the ensuing several days, the patient had worsening abdominal pain and developed melena. Imaging was remarkable for hyperdense material within the gallbladder. An endoscopic ultrasound was performed which revealed moderate amount of hyperechoic material in the gallbladder consistent with clotted blood. Given concern that an infected blood clot in gallbladder was a source of bacteremia, a laparoscopic cholecystectomy was performed. The surgical gallbladder specimen showed acute hemorrhagic acalculous cholecystitis. Postoperative course was uncomplicated and the patient was discharged home feeling back to normal.

DISCUSSION:

Hemobilia is a well-recognized complication of percutaneous liver biopsy, although this case is unusual because it appears to have led to acalculous cholecystitis and Klebsiella bacteremia. Blood, similar to sludge, can accumulate and mimic obstructive processes such as acute cholecystitis and acute pancreatitis. This case highlights the importance of early recognition of delayed complications of percutaneous liver biopsy.
2341 An Interesting Case of Drug-Induced Liver Injury Related to Khat
Authors: Waintraub Daniel J. , Zivari Kaveh , Fazio Richard M. , Weinberger Jonathan , Rahmani Rabin , Tsirlin Yuriy , Park James S.
Published: October 2019

INTRODUCTION:

Drug-induced liver injury (DILI) is the most common cause of acute liver failure in the United States (U.S). The United States Drug-Induced Liver Injury Network (DILIN) reported that approximately 15-20% of DILI cases per year could be attributed to herbal and dietary supplements. Khat is a stimulant derived from the leaves of the shrub Catha edulis, which is native to North Yemen and other East African countries. Chewing khat, a drug of abuse, is an established social habit in areas endemic to the shrub. Khat has been associated with clinically relevant acute and chronic liver injury. We present a case of drug induced liver injury related to Khat.

CASE DESCRIPTION/METHODS:

A 47 year old Yemeni male with no medical history presented with jaundice. He denied prior liver disease, alcohol use or prescription drugs. He reported routine use of Khat. Labwork is shown in Figure 1. MRCP revealed mild hepatosplenomegaly. Viral workup revealed immunity to hepatitis A. The patient presented 3 months later with jaundice and persistently elevated liver enzymes. Abdominal ultrasound was unrevealing. Extensive workup revealed a positive antinuclear antibody 1:320, in homogeneous pattern and an elevated serum immunoglobulin G and was otherwise unrevealing. Liver biopsy revealed mild portal mononuclear infiltration associated with focal interface hepatitis and widespread lobular hepatitis with plasma cell prominence in the infiltrates (Figures 1 and 2). Subsequently the patient was started on a course of Prednisone and Ursodiol with complete resolution of liver injury.

DISCUSSION:

Liver injury related to Khat typically occurs after years of use and may present acutely with nausea, fatigue, and jaundice or chronically with evidence of portal hypertension. Biochemical injury is typically hepatocellular and liver enzymes may be significantly elevated. Autoantibodies can mimic autoimmune hepatitis. Resolution of liver injury typically occurs with cessation of khat use. Patients may require corticosteroid therapy however the response is typically partial. Liver transplantation due to Khat related liver failure has been reported. Given immigration of individuals from countries endemic to Khat, an extensive history including use of cultural drugs such as Khat and other herbal remedies is critical in making a diagnosis of unexplained liver toxicity. Our case highlights the importance of a complete drug and supplement history and maintaining a high index of suspicion for DILI in the appropriate clinical context.
2342 Periodontal Flora Isolated From Pyogenic Liver Abscess in a Retired Dentist With Hepatic Metastasis of Colon Carcinoma
Authors: Chokshi Tanuj , Botti Evan , Arroyo-Mercado Fray , Likhtshteyn Michelle , Hossain Naseem , Huynh Chi Doan , Tello Daniela , McFarlane Isabel M.
Published: October 2019

INTRODUCTION:

The association of Streptococcus milleri group with pyogenic liver abscess is well recognized. Streptococcus gordonii, however, is a bacterium not of the milleri group and is included among the colonizers of the periodontal environment as it has a high affinity for molecules in the salivary pellicle on tooth surfaces. Here, we describe a case where Streptococcus gordonii was isolated from a pyogenic liver abscess in a retired dentist with hepatic metastasis of colon carcinoma. The clinical features, work up, management, and significance are described.

CASE DESCRIPTION/METHODS:

An 85-year-old male with colon carcinoma status post hemicolectomy complicated by metastasis to the liver status post chemotherapy and resection presented to the ED with intermittent abdominal discomfort for 2 weeks. Laboratory values were significant for WBC count of 16,000 with a predominance of granulocytes at 85%. CT of the abdomen and pelvis demonstrated a large, 7.6 cm by 5.3 cm, collection in the liver, likely an abscess. Infectious Diseases was consulted and recommended initiation of Meropenem and Daptomycin, in addition to drainage of the collection. The suspected abscess was drained by IR and Streptococcus gordonii sensitive to Linezolid was isolated on bacterial culture. The patient was transitioned to IV Linezolid and Metronidazole given susceptibilities. The patient's WBC count then began trending upwards. CT of the chest exhibited a 1.2 cm by 0.7 cm loculated pleural effusion on the right side. The pleural fluid collection was drained and a chest tube was placed. Studies of the drainage revealed an exudative process, although gram stain and culture were negative. Repeat CT imaging one week later revealed reduction in the size of the liver abscess. The WBC count normalized to less than 10,000 and the patient was discharged with a two-week course of oral Linezolid and Metronidazole with close outpatient follow-up with Infectious Diseases and Oncology.

DISCUSSION:

Pyogenic liver abscesses are generally associated with enteric gram-negative bacilli, Streptococcus milleri group, Streptococcus pyogenes, and Staphylococcus aureus. Hepatic abscesses secondary to periodontal flora have not been demonstrated in the literature without direct trauma to the GI tract involving foreign bodies contaminated with these organisms. We propose a possible hematogenous route of infection likely associated with possible risk factors related to the patient’s occupation as a dentist and history of dental manipulation.
2344 A Case of TACE: A Rare Complication of Transarterial Chemoembolization
Authors: Lorio Eric , Alameri Aws , Wathen Patricia
Published: October 2019

INTRODUCTION:

Transarterial chemoembolization (TACE) represents a proven therapy providing survival advantage in patients with large, unresectable hepatocellular carcinoma (HCC) not amenable to transplant or ablative therapies. Here we discuss a case of post-TACE liver abscess, a rare complication of this procedure.

CASE DESCRIPTION/METHODS:

A 65-year-old male with 10-year history of alcoholic cirrhosis (MELD-Na 12) presented for follow-up after a recent TACE procedure. Despite initial HCC isolation to segment 3, the patient had refused surgical intervention at the time of initial diagnosis. The mass rapidly enlarged, with new satellite lesions in segments 5 and 6. TACE of the left gastric artery supplying the HCC was performed 7 months after initial diagnosis, with subsequent ultrasound-guided HCC microwave ablation of a 7 cm segment 3 mass. One month after TACE, the patient presented to the emergency department with 2 weeks of weakness, fevers, and an episode of dizziness leading to a fall. Admission studies were notable for marked leukocytosis and blood cultures yielding Streptococcus lutetiensis. The patient was started on broad-spectrum antibiotics and scheduled for further imaging for evaluation of bacteremia source. CT abdomen revealed multifocal hepatic abscesses and a large 7 cm abscess communicating with distal stomach/proximal duodenum. On day 6 of hospitalization, IR placed a CT-guided hepatic abscess drain. Abscess culture was positive for E.coli and Enterococcus, and blood cultures from day after the procedure grew out extended spectrum beta-lactamase E.coli. He was started on a 3-week course of Ertapenem and discharged home with JP drain in place for follow-up in 3 weeks. JP drain was removed 10 days after discharge, and follow-up CT showed a significant decrease in the left hepatic lobe abscess with a small residual collection several centimeters above the drain. Ertapenem was discontinued after total of 5 weeks of antibiotic therapy.

DISCUSSION:

Liver abscess is an uncommon complication of TACE, occurring in about 2% of cases. Here our patient received the recommended therapy of percutaneous abscess drainage and parenteral antibiotics, resulting in a resolution of symptoms. Recognition of this complication is critical, as post-TACE liver abscess mortality has been estimated to be as high as 13.3%. Recent studies have even suggested prophylactic antibiotics when performing chemoembolization, with results showing promise, though this is not currently a routine or recommended practice.
2346 Primary Neuroendocrine Tumor of the Porta Hepatis in a Patient With Crohn’s Disease
Authors: Blau Lance N. , Parikh Rohan B. , Khalid Muhammad U. , Shina James F.
Published: October 2019

INTRODUCTION:

Well-differentiated neuroendocrine tumors (NETs) are rare entities usually of the GI tract and lung. A small subset of NETs have been identified in the biliary tree and liver, and large majorities of these are identified due to high concentrations of somatostatin receptors. We describe the rare case of a patient with Crohn’s disease and a primary porta hepatis NET.

CASE DESCRIPTION/METHODS:

An 80 year-old female with PMH of Crohn’s disease status post small bowel resection x3 and 30-pack year smoking history presented to clinic with a 5-year history of intermittent RUQ/mid-epigastric pain with known 2 cm liver mass. She denied any weight loss or changes in appetite or bowel habits. Abdominal US revealed a porta hepatis mass with a patent portal vein. Abdominal CT revealed mild diffuse infiltration of the liver and a 4.1 × 3.5 × 3.2 cm porta hepatis mass. The common bile duct measured 10 mm with thickening and irregularity of the antrum, pylorus, and proximal duodenum. There was moderate mesenteric lymphadenopathy. Biopsy of the porta hepatis mass revealed a low/intermediate grade NET of possible GI or pancreatic origin. Urine 5-HIAA was normal, and octreotide scan revealed abnormal activity in the area of the porta hepatis only. EGD revealed H. pylori (+) gastritis, and colonoscopy was normal. The patient underwent exploratory laparotomy revealing biliary invasion and positive metastatic lymph nodes. Open cholecystectomy was performed, and the patient underwent ERCP with common bile duct stent placement.

DISCUSSION:

Well-differentiated NETs are particularly rare. In a National Cancer Institute series, age-adjusted incidence for non-pancreatic primaries was 4.7 per 100,000. Most primary NETs are located in the GI tract (55%) and bronchopulmonary system (30%) and tend to metastasize to the liver. Patients may experience pain, jaundice, early satiety, flushing, or diarrhea. Our patient’s only complaint was pain. Despite an extensive workup to stage the cancer and identify a potential primary, no additional culprit lesion was identified. Immunohistochemical staining and/or molecular classifier assays of the specimen can be helpful to identify the primary site but are not definitive. We believe this is a primary hepatic lesion which is very rare. Metastasis to the biliary tree and hilar lymph nodes portends a poorer prognosis. Our patient was started on Somatuline. Somatostatin analogs are highly effective in controlling symptoms associated with advanced GI NETs.
2347 Acute Liver Failure: Drug-Induced Liver Injury and a Perfect Thyroid Storm
Authors: Harris Patrick , Shoreibah Mohamed
Published: October 2019

INTRODUCTION:

Acute liver failure (ALF) is a rare but serious condition defined by severe acute liver injury with coagulopathy (INR ≥ 1.5) and encephalopathy without preexisting cirrhosis. ALF carries a high morbidity and mortality. Liver transplantation (LT) is the only definitive treatment for patients that fail other supportive therapies.

CASE DESCRIPTION/METHODS:

A 28 year-old female with hyperthyroidism due to Grave disease was transferred to our institution for acute liver failure (ALF) and transplant evaluation. One month prior, she developed jaundice and pruritus. She had been treated with propylthiouracil (PTU) for 1 year but that was stopped 1 week prior to transfer due to elevated liver enzymes. On evaluation she was found to have elevated AST/ALT (2800/1900), hyperbilirubinemia (28.6) and an elevated INR (2.85). The patient progressively worsened and developed encephalopathy thus fulfilling the criteria for ALF. On admission, her MELD-Na was 33 and she had grade III hepatic encephalopathy. Her thyroid studies were found to be TSH <0.010, free T3 17.9 and free T4 >5.6. Her Burch-Wartofsky Point Scale was >45, which is highly suggestive of thyroid storm. She was subsequently started on plasmapheresis, methimazole, hydrocortisone and cholestyramine for thyroid dysfunction. Patient continued to clinically decompensate requiring an emergent thyroidectomy followed by LT. Post-operatively patient had an acute change in her neurological status with loss of reflexes. CT head showed diffuse global cerebral edema with brainstem compression and herniation. Medical care was withdrawn and patient expired.

DISCUSSION:

This is a case of ALF from PTU drug induced liver injury complicated by thyroid storm. PTU is the second most common non-acetaminophen related cause of DILI that requires liver transplantation. PTU-related ALF can occur at any point of treatment and the onset is usually sudden with rapid progression. While extremely rare, thyroid storm is also a reported cause of ALF. Excess thyroid hormone may cause hepatocyte dysfunction. This case poses a diagnostic dilemma given the coexistence of thyroid storm and PTU therapy. It is possible that the initial hepatic injury due to PTU-related DILI made the liver more susceptible to fulminant hepatic failure from thyroid storm. Despite the rapid listing and transplantation within 48 hours of arrival, the patient did not survive. This case extends the spectrum of the presentation of ALF in the setting of thyroid dysfunction.
2348 A Rare Case of Biktarvy-Induced HBV Flare
Authors: Gujjula Srilaxmi , Barakat Mohamed , Misra Jaideep , Aloreidi Khalil , Berkowitz Leonard , Reddy Madhavi
Published: October 2019

INTRODUCTION:

Exacerbation of chronic hepatitis B virus (HBV) is defined as an acute rise in alanine aminotransferase (ALT) more than five times the upper limit of normal. Flare-ups of chronic HBV and human immunodeficiency virus (HIV) coinfection receiving antiretroviral therapy is seldom due to discontinuation or initiation of emtricitabine treatment. Here, we present a rare case of reinitiation of Biktarvy causing HBV flare.

CASE DESCRIPTION/METHODS:

A 53-year-old male presented to the emergency department from infectious disease clinic for worsening transaminitis, nausea and right upper quadrant (RUQ) pain for two days. Patient did not report any alcohol, supplement or acetaminophen use. Past medical history is significant for HIV and chronic HBV. He had been non-adherent to Biktarvy (bictegravir, emtricitabine, tenofovir alafenamide) for months and was restarted on the medication three weeks prior to presentation. His vitals were within normal limits. Physical examination revealed RUQ tenderness. Complete blood count showed platelet count of 137K. Coagulation study, basic metabolic panel, lipase were within normal limits. HBV Viral load: 7,126,237, HIV Viral load: undetectable, aspartate aminotransferase (AST): 599, ALT: >680. Hepatitis panel showed immunity to Hepatitis A, Hepatitis C: negative, HBV surface antigen: positive, HBV core IgM: positive and no seroconversion of Hep Be antigen to antibody. All viral serologies, HIV, HBV status and transaminases listed in Tables 1 and 2 from former and current admission. Autoimmune and hereditary conditions work up done during current admission were negative (Table 3). RUQ ultrasound was unremarkable. Patient was seen in clinic two weeks later and reported adherence with Biktarvy. Liver enzymes continued to trend down and his symptoms of nausea and RUQ pain subsided.

DISCUSSION:

Emtricitabine is one of the components of HIV regimen of Biktarvy. Treatment flares due to emtricitabine arise only in 5-10% of patients and are usually asymptomatic. Our patient had symptomatic hepatotoxicity after restarting Biktarvy over the span of three weeks. Hepatotoxicity could be due to the variations of viral replication activating immune responses. Even though the flare ups of chronic HBV can be due to initiation of emtricitabine, it is recommended to continue the agent and monitor for down trending pattern of aminotransferases which was depicted in our case.
2350 An Unlikely Presentation of Diffuse Large B-Cell Lymphoma Manifesting as Hepatic Failure
Authors: Pandey Nishi , Truscello David , Parikh Sharan , El Genaidi Hisham
Published: October 2019

INTRODUCTION:

Primary Hepatic Lymphoma (PHL) is a very rare subtype of Diffuse Large B-Cell Lymphoma (DLBCL), accounting for less than 1% of all cases. In rare instances, a patient can present with fulminant hepatic failure. The prognosis is grim with an average survival of 10.7 days from admission. The mainstay of diagnosis is liver biopsy, which must be performed as early as possible to prevent progression to acute liver failure.

CASE DESCRIPTION/METHODS:

A 55-year-old male with no pertinent medical history presented with a two week history of abdominal pain, fever, lethargy and mild jaundice. He was a former smoker and drinker who denied any alcohol use for the past 15 years. Upon presentation, the patient’s labs showed acute liver injury, anemia, thrombocytopenia and acute kidney injury. The patient’s model for end-stage liver disease (MELD) was 29. He was subsequently admitted to the medical intensive care unit (ICU) where emergent liver transplant evaluation was initiated. A full work up for the etiology of his liver failure was initially unrevealing so a liver biopsy was performed, which came back positive for DLBCL. The patient was then started on a chemotherapeutic regimen consisting of cyclophosphamide, vincristine, etoposide and high dose steroids. The patient’s condition further deteriorated during his ICU stay, leading to the need for mechanical ventilation, vasopressor therapy for circulatory shock, continuous renal replacement therapy for worsening renal failure and transfusion of blood products for worsening coagulopathy. Despite all of these aggressive measures, the patient’s clinical status only continued to worsen. The family decided to institute comfort measures and the patient died on ICU day 12.

DISCUSSION:

DLBCL predominantly affects Caucasian men over the age of 50. Some viruses, specifically EBV, HBV and HCV, have shown correlation with Non-Hodgkin Lymphoma (NHL); our patient had serologies indicative of past exposure to EBV and HBV. The only way to definitively diagnose DLBCL is via liver biopsy with immunohistochemical staining that reveals CD20, CD45 and BCL-6. The prevailing treatment modality is the use of Dose Adjusted Etoposide, Prednisone, Oncovin, Cyclophosphamide, Hydorxydauorubicin and Rituximab (DA-EPOCH R) Therapy. Due to its poor prognosis, early diagnosis and initiation of treatment for PHL is crucial. For this reason, it must be considered early in the differential when evaluating a patient with acute liver failure.
2351 NAC of All Trades: Use of N-acetylcysteine in Cocaine-Induced Acute Liver Failure
Authors: Bill Katherine , Chaar Abdelkader , Barawi Mohammed
Published: October 2019

INTRODUCTION:

Among patients with acute liver failure (ALF), drug-induced liver injury (DILI) presents a challenging clinical syndrome with limited therapeutic options. N-acetylcysteine (NAC) is a mainstay of treatment in acetaminophen-induced ALF, acting as an antioxidant, a vasodilator and a glutathione precursor. However, it has been used with success in other toxic ingestions causing ALF, and its beneficial effects do not seem to be unique to acetaminophen-induced hepatotoxicity. We present a case of ALF after acute cocaine ingestion with rapid recovery after administration of IV NAC.

CASE DESCRIPTION/METHODS:

A 58 year-old-male with a past medical history of chronic hepatitis C without cirrhosis and polysubstance abuse presented with acute psychosis. On arrival he was tachycardic but hemodynamically stable, with psychomotor agitation and fear delusions. He endorsed recent heavy cocaine ingestion. Initial labs demonstrated acute kidney injury, rhabdomyolysis and mild transaminitis; AST 59, ALT 56. Toxicology workup was positive for cocaine with undetectable acetaminophen, ethanol and salicylate levels. The following day however, he developed fulminant hepatic failure, AST >7000, ALT 2,568 (Figure 1) with progressive coagulopathy, INR 2.14, albumin 2.9, platelets 120. Of note, despite chronic hepatitis C, there was no evidence of cirrhosis and preserved liver synthetic function at presentation, INR 1.10, albumin 4.7, platelets 254. Our patient was not a candidate for liver transplant (LT), and decision was made to initiate IV NAC therapy, 140 mg/kg bolus followed by 70 mg/kg daily. Transaminases rapidly normalized after only 3 days of therapy, and he was discharged in stable condition.

DISCUSSION:

ALF is a rare but challenging syndrome, for which LT is the only curative treatment available. NAC is well established as an antidote in acetaminophen ALF, yet its mechanisms are applicable to other forms of DILI as well. Cocaine hepatotoxicity has been reported in humans, and is well studied in animal models. Studies in mice suggest toxic metabolites play a critical role, much like acetaminophen ALF. These species deplete glutathione, decrease hepatic blood flow, generate reactive oxygen species and cause lipid peroxidation of membranes. We present a case of ALF secondary to cocaine ingestion in the setting of underlying chronic hepatitis C, with rapid recovery after fluid resuscitation and IV NAC. NAC therapy has minimal adverse effects, and may be lifesaving in patients with ALF who are not candidates for LT.
2353 Tuberculosis: Another Great Masquerader of the GI Tract
Authors: Gampa Anuhya , Shah Ayush N. , Shah Dhiren
Published: October 2019

INTRODUCTION:

This case underlines the role tuberculosis has in non-pulmonary settings. Elevated liver enzymes in any setting of tuberculosis should raise concern for TB hepatitis until proven otherwise.

CASE DESCRIPTION/METHODS:

A 48 year old man a with PMH of Crohn’s colitis s/p right hemicolectomy, latent tuberculosis (TB) and asthma presented for elevated liver enzymes on routine testing. He denied abdominal pain, jaundice, acholic stools or dark urine. He was taking Adalimumab, Isoniazid and Pyridoxine but ran out of medications. He emigrated from Mongolia a few years ago and endorsed heavy alcohol use (bottle of liquor 2-3 times per week) and daily herb use. His exam was notable for fever to 102.9 F, tachypnea 25 breaths/min with hypoxia SpO2 8o% and mild hepatomegaly. Labs were notable for AST 92 IU/L, ALT 157 IU/L, total bilirubin 0.9 mg/dL, ALP 476 IU/L, protein 6.5 g/dL, albumin 3.0 g/dL, INR 1.1. Viral hepatitis panel was negative and interferon gamma release assay was positive for TB. MRCP revealed an enlarged liver but otherwise normal biliary tree and gallbladder. CXR and CT chest showed innumerable pulmonary nodules and lymphadenopathy concerning for pulmonary TB, which was confirmed on bronchoscopy. Liver enzymes continued to stay elevated, so a liver biopsy obtained showed cholestatic hepatitis with no granulomas. PCR testing was added on which was positive for M. Tuberculosis DNA, consistent with TB hepatitis. The patient was started on anti-tubercolosis therapy with improvement in liver enzymes.

DISCUSSION:

Elevated liver enzyme testing in the setting any manifestation of tuberculosis should raise concern for TB hepatitis. The presentation is highly variable from an acute to chronic process, and the most common lab abnormality is ALP elevation. Biopsy findings with caseating granulomas is only 68% sensitive while PCR testing is 85% sensitive. Despite the multiple risk factors for acute hepatitis and nonspecific biopsy findings, if the suspicion for TB hepatitis is still high, PCR testing can be added.
2356 Severe Allopurinol-Induced Liver Injury
Authors: Guzik Paul , Kim Christopher , Kumbum Kavitha
Published: October 2019

INTRODUCTION:

The severity of drug-induced liver injury (DILI), a diagnosis of exclusion, can vary greatly from mild to life-threatening. Allopurinol is a commonly used medication that rarely causes serious hepatotoxicity. Objective data, such as aspartate aminotransferase (AST), alanine aminotransferase (ALT), alkaline phosphatase (ALP), gamma-glutamyl transferase (GGT), and total serum bilirubin, have been used to grade the severity of liver injury. We present a case of idiosyncratic allopurinol-induced cholestatic injury that meets diagnostic criteria for severe and life-threatening.

CASE DESCRIPTION/METHODS:

A 70-year-old African American woman with gouty arthritis, hypertension, and dyslipidemia presented to the Emergency Room with two weeks of abdominal pain. The patient was started on allopurinol for acute gout flare one month prior to presentation. In the interim, she developed malaise, anorexia (3 kg weight loss), and pruritus. She had no personal or family history of liver disease. Physical examination was notable for scleral icterus and mild, diffuse abdominal tenderness. Complete blood count was remarkable for absolute eosinophilia of 3.0 thou/cu mm, confirmed on peripheral blood smear. Liver function tests revealed a cholestatic pattern, with AST 259 u/L (normal 5-34), ALT 411 u/L (10-60), ALP 817 u/L (4-121), GGT 766 u/L (9-36), total bilirubin 15.7 mg/dL (0.2-1.2) and direct bilirubin 10.6 mg/dL (0.1-0.5). Synthetic liver function was normal. Toxicology screens, ceruloplasmin level, hepatitis serologies, and autoimmune workup were unremarkable. Imaging studies did not reveal acute pathology. Liver biopsy showed mild/moderate expansion of mixed inflammatory infiltrate with increased number of eosinophils. After discontinuation of allopurinol, absolute eosinophil count and liver function tests trended down with clinical resolution of symptoms.

DISCUSSION:

Among DILI literature, few reports exist of severe, life-threatening cholestatic liver injury after allopurinol initiation. This patient had 8x the upper limit of normal AST, 7x ALT, 7x ALP, 21x GGT, and 21x direct bilirubin. According to multiple DILI severity grading tools, this case is classified between severe (grade 3) and life-threatening (grade 4). Beyond discontinuation of the medication, there are limited treatment options available for idiosyncratic DILI other than liver transplantation. Therefore, prompt discontinuation of the offending medication along with close monitoring for progression to fulminant liver failure is crucial.
2360 Progression of Post-Islet Cell Transplantation Hepatic Steatosis to Cirrhosis: A Rare Complication of a Common Finding
Authors: Condon Sally , Smart Laura , McClain Craig
Published: October 2019

INTRODUCTION:

Non-alcoholic fatty liver disease (NAFLD) is a growing cause of liver disease in the USA and throughout the rest of the world and is predicted to soon become the leading indication for liver transplantation. NAFLD exists on a spectrum from hepatic steatosis to non-alcoholic steatohepatitis (NASH) to cirrhosis. It is closely related to metabolic syndrome and insulin resistance. A lesser known cause of hepatic steatosis is islet cell allotransplantation and autotransplantation, which are experimental treatments for Type 1 Diabetes Mellitus and FDA- approved treatment for patients with chronic pancreatitis who have undergone pancreatectomy, respectively. In this case, we present the first case of post-islet cell transplantation hepatic steatosis progressing to cirrhosis.

CASE DESCRIPTION/METHODS:

The patient is a 27 year old female with a history of chronic pancreatitis secondary to cystic fibrosis, status-post total pancreatectomy and islet cell autotransplantation, complicated by development of non-alcoholic steatohepatitis. Her NASH was treated with probiotics and vitamin E supplementation. Eight years after her islet cell transplant, she was hospitalized for nausea, vomiting, and abdominal pain. A CT scan showed that she had developed severe, diffuse hepatic steatosis. Her ALP and AST were mildly elevated. ALT and total bilirubin were normal. She had facial and pedal edema, but no other stigmata of cirrhosis. She underwent a liver biopsy which showed bridging fibrosis and a small regenerative nodule with steatohepatitis. It was with these findings that she was diagnosed with cirrhosis.

DISCUSSION:

Hepatic steatosis has been reported with a prevalence of 20-40% of patients following islet cell transplantation. These reports have not been associated with any long term liver or graft survival outcomes. The usual onset of histological findings in one study was approximately 3 years after transplant, and some of these cases resolved over time. These reported cases did not have any changes in total bilirubin, ALP, AST, or ALT. Hepatic steatosis from islet transplantation is thought to develop due to the paracrine effect of localized hyperinsulinemia and fatty acid infiltration from the islet cells, which are inserted into the portal vein and taken to the liver during transplant. To our knowledge, this is the only reported case in which post-islet cell transplantation hepatic steatosis progressed to NASH cirrhosis along with abnormal liver biomarkers.
2362 A Case of BRAF V600E Intrahepatic Cholangiocarcinoma
Authors: Ahmed Zarir , Doane Elizabeth , Antao Nirav , Chhaparia Anuj
Published: October 2019

INTRODUCTION:

Intrahepatic cholangiocarcinoma is an uncommon hepatic malignancy and accounts for 3% of all gastrointestinal malignancies. Patients with this disease have a poor prognosis, with an average 5-year survival rate of 5 to 10%. Herein we describe a case of BRAF-related cholangiocarcinoma.

CASE DESCRIPTION/METHODS:

A 26 year-old female presented with right-upper quadrant pain and worsening nausea, vomiting for four months. On exam the abdomen was soft, non-tender and non-distended. A right upper quadrant ultra sound revealed a 7 cm mass in the inferior right hepatic lobe and ensuing magnetic resonance imaging of the liver should an 8 cm lesion with numerous satellite lesions. She underwent laparoscopic wedge excision of the liver mass which revealed poorly differentiated adenocarcinoma with features concerning for cholangiocarcinoma (See Images). PET revealed also portal hepatic lymph node metastasis, and metastatic lytic lesions at the clavicle and scapula. CEA and AFP were elevated. Comprehensive genetic analysis with next generation sequencing genomic panel was performed on her initial biopsy and confirmed BRAF V600E mutation. She was initiated on a phase II trial with trametinib and dabrafenib at a referral center. Despite this, repeat CT imaging revealed she had progression of metastatic cholangiocarcinoma. She was switched to FOLFIRI and vemurafineb. However, the patient developed worsening abdominal distension, hyperbilirubinemia and pancytopenia. At that point, the family elected for hospice care whereupon after a few weeks, the patient expired at home.

DISCUSSION:

Currently the only curative therapy is surgical resection or transplant. However there has been research in molecular targeted therapies to treat BRAF cholangiocarcinoma with optimal response. BRAF V600 mutation results in activation of down-stream signaling though MAPK pathway and can contribute to malignancies such as melanoma, hairy cell leukemia, or non-small cell lung cancer. The frequency of BRAF V600E mutation has been reported to be 0-22% in intrahepatic cholangiocarcinoma. Inhibitors of BRAF V600 kinase such as vemurafenib and dabrafenib can be used to target these aberrations. Although this patient failed treatment with molecular targeting, precision medicine may change the trajectory in management of intrahepatic cholangiocarcinoma. The implementation of these therapies will require multi-team and institutional collaborations and will positively impact these patients.
2371 Non-Cirrhotic Portal Hypertension Presenting With Variceal Bleeding in a Patient With Breast Cancer on Capecitabine
Authors: Luft Suzannah , Calderwood Audrey H.
Published: October 2019

INTRODUCTION:

We describe a case of variceal bleeding in a cancer patient with improving liver metastases without radiographic evidence of cirrhosis.

CASE DESCRIPTION/METHODS:

A 66 year-old woman with stage IV bilateral breast cancer presented to the hospital with hematemesis. Platelet count was 98,000/mcL and INR 1.2 with normal albumin and liver enzymes. Upper endoscopy showed large varices with stigmata of recent bleeding and portal hypertensive gastropathy. Varices were banded without further bleeding. CT abdomen/pelvis with contrast showed left hepatic lobe atrophy, normal spleen, normal liver contour and no portal venous thrombosis (PVT). She was diagnosed with breast cancer 26 years prior and underwent lumpectomy and radiation. Eight years ago, she had recurrence, underwent mastectomy and then was diagnosed with contralateral breast cancer followed by mastectomy. Six years ago, she had recurrence requiring axillary dissection with chemotherapy and radiation. Four years ago, imaging showed new metastases with heavy left liver tumor burden with left portal vein cut off, ascites and bony metastases. Liver lesion biopsy showed breast cancer. She started doxorubicin with resolution of ascites and marked left lobe atrophy by imaging. For two years, she was remained on doxorubicin without evidence of disease. Three months prior to admission, PET CT showed two small right hepatic lobe metastasis, and she was started on capecitabine.

DISCUSSION:

Non-cirrhotic portal hypertension in the setting of liver metastases occurs for various reasons. One etiology is PVT, which imaging ruled out. Pseudocirrhosis describes a radiographic appearance of cirrhosis without histopathologic cirrhosis due to capsular retraction from metastases shrinkage after therapy, nodular regenerative hyperplasia (NRH) due to chemotherapy and fibrotic changes around metastases. While this is reported with capecitabine, our patient did not appear cirrhotic on imaging. Portal hypertension due to micronodular metastases is reported, but this is unlikely with improved liver lesions on imaging. Hepatic steatosis with elevated transaminases is reported with capecitabine. Several chemotherapies cause NRH, including doxorubicin. We suspect underlying NRH from previous doxorubicin and left hepatic lobe atrophy from shrinkage of metastases combined with new capecitabine caused an increased in liver stiffness. This case demonstrates the complex effects on the liver from both metastases and cancer treatments in a patient without known liver disease.
2379 Biopsy Confirmed Infiltrating Breast Cancer in the Liver Not Captured by MRI Abdomen
Authors: Stewart Sarah , Reddy Shravani , Kesar Varun , Sahebjam Farhad , Grider Douglas J.
Published: October 2019

INTRODUCTION:

Early detection of liver metastases (mets) has the potential to improve patient outcomes, and those with breast cancer are at increased risk for liver mets. Literature has shown that computed tomography (CT) or magnetic resonance imaging (MRI) techniques detect most liver mets with a high level of accuracy; however, small mets or non-mass mets in sinusoidal spaces, are less likely to be recognized on imaging studies. Of the imaging studies available, MRI is toted to be more sensitive than CT or positron emission tomography in detecting small lesions. Here we present a case of metastatic breast cancer not detected by MRI.

CASE DESCRIPTION/METHODS:

A 50-year-old female with a history of cirrhosis, invasive lobular carcinoma of the left breast (T3N3aM1) following unilateral mastectomy and axillary dissection, and mets to uterus status post total abdominal hysterectomy with bilateral salpingo-oophorectomy presented with complaints of confusion and abdominal distension. She was admitted for acute on chronic decompensated liver failure with exam significant for jaundice, asterixis, distended abdomen, and pitting edema to bilateral extremities. MELD score on admission was 23 with Child-Pugh Class C, and labs were notable for hyperbilirubinemia (15.9) and elevated alkaline phosphatase (950). Abdominal ultrasound demonstrated a nodular heterogenous liver consistent with cirrhosis. Ascitic fluid was felt to be secondary to portal hypertension (SAAG 2.4g/dL, ascitic protein 0.5 g/dL) and negative for spontaneous bacterial peritonitis. The patient was negative for hepatitis A, B, C, or D, antimitochondrial antibodies, and acetaminophen levels were unremarkable. MRI of the abdomen indicated hypertrophy of the left lobe of the liver and an irregular hepatic capsule contour, but no distinct reticular enhancement pattern or focal abnormalities. Transjugular liver biopsy was, however, positive for a large burden of metastatic lobular breast carcinoma, mostly confined to sinusoidal spaces, in a background of end-stage liver disease and acute cholestasis.

DISCUSSION:

The presence of liver mets that were undetectable by MRI, the imaging technique of choice metastatic lesions, demonstrates this case’s uniqueness. The size and/or location of the metastasis is directly related to the ability of anatomic imaging to detect metastatic lesions. This patient’s presentation and lack of radiographic evidence of metastasis demonstrates the importance of tissue pathology studies in patients with suspected metastatic processes.
2380 Liver Cirrhosis of Unknown Etiology With Iron Overload in a Patient Heterozygous for Hereditary Hemochromatosis and Alpha-1 Antitrypsin
Authors: Shastri Pinang , Ghazaleh Sami , Khader Yasmin , Moussa Zeinab , Nehme Christian , Awad Mohammed , Nawras Ali
Published: October 2019

INTRODUCTION:

Abnormal liver function tests are frequently detected in routine laboratory work. The differential diagnosis is broad and further evaluation is required to identify and treat the underlying cause. We present a patient with abnormal liver function tests who was found to have a unique genetic makeup.

CASE DESCRIPTION/METHODS:

A 68-year-old female patient presented with jaundice and dark urine for 3 months. Past medical history was significant for paroxysmal atrial fibrillation and nephrolithiasis. Past surgical history and family history were insignificant. Home medications included aspirin and atenolol. She used alcohol socially and denied IV drug use. Review of systems was unremarkable. On physical exam, the patient was diffusely jaundiced and had scleral icterus. Vital signs were within normal limits. Abdominal exam showed a soft, non-tender, and non-distended abdomen without organomegaly. Skin exam was significant for multiple spider angiomas. She had elevated AST of 518 U/L, ALT of 246 U/L, alkaline phosphatase of 185 U/L, total bilirubin of 9.5 mg/dL, and direct bilirubin of 4.5 mg/dL. Workup was negative for viral hepatitis, Wilson’s disease, autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. She had elevated Ferritin of 3547 ng/mL and iron saturation of 83%. MRI showed features of intrinsic liver disease without a focal mass. Next, upper endoscopy revealed Grade 1 esophageal varices. Subsequent liver biopsy revealed cirrhosis and increased iron stores. The patient underwent genetic testing for hemochromatosis and alpha-1 antitrypsin deficiency. She had a heterozygous C282Y mutation of the HFE gene. In addition, she had a heterozygous PI*MS mutation of SERPINA1 - the gene that codes for alpha-1 antitrypsin. The patient experienced a slow improvement in her abnormal labs over a period of a few months.

DISCUSSION:

Hereditary hemochromatosis and alpha-1 antitrypsin deficiency are inherited conditions that result in liver damage and eventually liver cirrhosis. Our patient was heterogenous for the HFE gene and developed iron overload on liver biopsy. She was also heterozygous for PI*MS, a less common mutation for SERPINA1 that causes isolated lung disease. Therefore, we can conclude that our patient’s condition was primarily related to iron overload as a result of a mutated HFE gene.
2381 Abnormal Aminotransferases, Think Beyond the Liver
Authors: Ghazaleh Sami , Khader Yasmin , Ghanim Marcel , Shastri Pinang , Alhmoud Tarik
Published: October 2019

INTRODUCTION:

Liver aminotransferases are frequently utilized to evaluate liver and biliary tract diseases. Elevated aminotransferases are non-specific and further testing is usually necessary to identify the underlying cause. We present a puzzling case of abnormal aminotransferases.

CASE DESCRIPTION/METHODS:

A 28-year-old male patient was referred to hepatology clinic for evaluation of abnormal laboratory findings. Routine blood tests revealed elevated AST of 67 U/L and ALT of 77 U/L. Alkaline phosphatase was 36 U/L and total bilirubin, total protein, and albumin were within normal limits. Review of systems was unremarkable. Past medical and surgical histories were insignificant. His only home medication was diphenhydramine. Family history was negative for liver disease or malignancies. He reported drinking 5-6 drinks per week and denied the use of tobacco or illicit drugs. On physical exam, body mass index (BMI) was 28. Abdomen was soft, non-tender, and slightly distended. He had normal bowel sounds and no organomegaly. Abdominal ultrasound showed a normal liver and gallbladder. Hepatitis A, hepatitis B, and hepatitis C serology were unremarkable. Serum ceruloplasmin and alpha-1 antitrypsin levels were normal. Antinuclear antibody (ANA) and antimitochondrial antibodies were negative. Anti-smooth muscle antibody (ASMA) was borderline positive with a titer of 1:20. Ferritin level was 100 and transferrin saturation (TSAT) was elevated at 59%. Subsequently, a liver biopsy was performed and revealed normal liver architecture with intact hepatic lobules. Additional history revealed that the patient gets leg cramps while walking and could not run as fast as the other kids in school. Upper musculoskeletal exam was unremarkable. However, lower musculoskeletal exam revealed a 4/5 weakness in addition to bulky calf muscles. Laboratory workup showed elevated CK of 1002 U/L and aldolase of 11.2 U/L. The patient was referred to rheumatology and neurology for further evaluation of myopathy.

DISCUSSION:

The evaluation of elevated aminotransferases can be challenging especially in asymptomatic patients. In our patient, extensive liver work-up came back normal. Although he had elevated BMI, there was no evidence of non-alcoholic steatohepatitis (NASH). Muscle disorders/myopathies may present with elevated aminotransferases. A good history and physical exam can provide important clues to reach the correct diagnosis.
2382 Successful Hepatitis C Virus Antibody-Positive Donor Liver Transplantation in Acute Sickle Cell Intrahepatic Cholestasis: A Case Report
Authors: Alkhayyat Motasem , Saleh Mohannad Abou , Sanghi Vedha , Singh Tavankit , Rouphael Carol , Simons-Linares Roberto , Lindenmeyer Christina C. , Romero-Marrero Carlos , Carey William
Published: October 2019

INTRODUCTION:

Sickle cell hepatopathy (SCH) is the umbrella term referring to liver dysfunction in sickle cell disease (SCD) patients. We report a case of acute intrahepatic cholestasis (ASCIH) with underlying cirrhosis managed with hepatitis C virus (HCV) antibody-positive donor liver transplantation (LT).

CASE DESCRIPTION/METHODS:

A 29-year-old African American male with known medical history of SCD (Hb SS) and cirrhosis presented to the medical intensive liver unit with vaso-occlusive pain crises and jaundice. Patient was afebrile with benign exam. Labs were consistent with obstructive jaundice (total serum bilirubin of 57 mg/dL, direct serum bilirubin of 30 mg/dL), and elevated liver enzymes (alkaline phosphatase 306 U/L, aspartate transaminase 227 U/L and alanine transaminase 54 U/L). He had significant leukocytosis 38.6 k/uL, low hemoglobin 6.3 g/dL and thrombocytopenia 39 k/uL. Sodium level was 149 mg/dl and INR was 2.3. Computed tomography scan showed changes consistent with cirrhosis but no evidence of ductal dilatation. He developed encephalopathy after 24 hours of presentation, acute kidney injury (serum creatinine 3.57 mg/dl) and respiratory failure requiring endotracheal intubation. He was diagnosed with acute liver failure secondary to ASCIH after a non-revealing extensive workup for acute liver failure. Exchange transfusion was started without improvement in hepatic function or mental status. His MELD-Na (Model for End-Stage Liver Disease-Sodium) score was 43 and thus patient was listed for urgent LT. He received an HCV positive donor liver after 9 days of hospitalization. Explanted liver biopsy showed widespread sinusoidal dilatation with innumerable clusters of sickled red blood cells, scattered pigmented histiocytes, and cholestasis. Following transplant, exchange transfusion was resumed and immunosuppression treatment started with mycophenolate, tacrolimus and steroid taper. Glecaprevir/Pibrentasvir started for high HCV RNA titer. He was discharged after 23 days with total bilirubin of 4.2 mg/dL and normalization of his liver enzymes. He was doing well during his 4 months follow up.

DISCUSSION:

We present a rare case of acute sickle cell intrahepatic cholestasis treated with successful LT. This represents the 1st case of a positive HCV donor LT and the 20th LT case in SCD. ASCIH is the most severe, often fatal, form of SCH. Our goal with this case is to increase the awareness of this important condition and emphasize the role of liver transplantation as a potential treatment.