bluuebunny/crossref_metadata_2025 huggingface dataset:
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.
2374 Rare Microbiologic Etiologies of Spontaneous Bacterial Peritonitis
Authors: Tripathi Nishant , Koirala Niki , Kudaravalli Praneeth , Akanbi Olalekan , Ali Saad Emhmed , Yarra Pradeep , Kato Hirotaka , Thakur Kshitij , Karri Kishore , Singh Tushi
Published: October 2019

Introduction:

Often regarded as a poor prognostic factor in ascites, spontaneous bacterial peritonitis (SBP) is usually caused by Gram negative rods (E. coli, K. pneumoniae) and Gram positive cocci (S. pneumoniae).This case report identifies 2 rare cases of SBP.

CASE DESCRIPTION/METHODS:

Case 1 A 59 yo M with h/o decompensated cirrhosis (ascites without SBP history, encephalopathy, and variceal bleed) due to HCV presented with an episode of fall requiring orthopedic procedures. Flapping tremor, mild icterus, anemia, thrombocytopenia, AKI, coagulopathy, hyperbilirubinemia, hypoalbuminemia, and MELD score of 18 were significant findings. Abdominal/pelvic CT revealed hydronephrosis, which was corrected with ureteral stent placement upon admission. While awaiting rehab placement, patient developed paralytic ileus, septic shock due to ceftriaxone susceptible E. coli bacteremia, upper GI bleed, hypernatremia, pancytopenia, worsening hepatic encephalopathy, and worsening ascites with PMN of 3062. Abdominal/pelvic CT revealed no bowel perforation or alternate peritonitis source. Initially covered with piperacillin/tazobactam (P/T) and vancomycin for 4 days, therapy was narrowed to ceftriaxone for the following 4 days. Unfortunately, ascitic culture revealed Eggerthella lenta on culture day 8 (susceptible to clindamycin, meropenem, amoxicillin/clavulanate); patient ultimately demised with multiorgan failure. Case 2 A 66 yo M with h/o HCV and alcoholic cirrhosis on ciprofloxacin SBP prophylaxis, HFrEF, PVD with non-healing ulcer, CKD, and COPD presented with abdominal distension for routine paracentesis. Patient requires 5 to 6 L of ascitic fluid drainage with albumin replacement every other week. Ascitic fluid revealed PMN of 384; ceftriaxone was administered for 5 days and patient was discharged on ciprofloxacin prophylaxis. Ascitic fluid drawn prior to discharge ultimately grew Paenibacillus barengoltzii. Patient was requested to return for repeat ascitic culture, which resulted negative.

DISCUSSION:

1. E lenta and P. barengoltzii SBPs have not been previously described in literature; thus, both represent the first cases of each. A rare anaerobic intra-abdominal pathogen, E. lenta is associated with high mortality with P/T monotherapy. Paenibacillus species have not been studied in depth but are likely susceptible to third-generation cephalosporins. Gastroenterologists and internists managing similar cases may fail to identify these etiologies and appropriate treatment resulting in unfavorable outcomes.
2378 Hepatic Actinomycosis Masquerading as Gastrointestinal Malignancy
Authors: Kang Jennifer , Tian Frances , Joseph Augustin , Russell Sarah , Jarrett Olamide , Chan Christine
Published: October 2019

INTRODUCTION:

Abdominal actinomycosis is a rare cause of liver cysts and rectal masses. It remains a diagnostic challenge.

CASE DESCRIPTION/METHODS:

A 60 year-old male (patient L) presented with a rectal mass found on colonoscopy. Biopsies were inconclusive; however, staging CT demonstrated liver lesions presumed to be metastases from rectal carcinoma. The patient began to have recurrent fevers, night sweats, and unintended weight loss, prompting an extensive infectious work-up notable only for a positive quantiferon. In the interim, patient L underwent laparoscopy and liver biopsy to further evaluate his presumed malignancy. Frozen sections revealed caseating granulomas with negative AFB and stains. The patient was diagnosed with GI tuberculosis (TB) based on constitutional symptoms, positive quantiferon, and frozen section findings, and was started on standard four-drug therapy. Final pathology resulted in visualization of sulfur granules, diagnostic of actinomycosis. TB treatment was discontinued and augmentin was started for a 6 month course.

DISCUSSION:

Actinomyces colonizes the oropharynx and digestive and genital tracts. It infrequently causes disease. Abdominal infection accounts for 20% of actinomycoses; of these, only 15% report liver involvement. Infection is associated with compromise of the mucosal barrier, either due to instrumentation or appendicitis. Our patient had recently undergone colonoscopies to diagnose his rectal mass. As in Patient L, actinomycotic liver abscesses usually present with an indolent course; symptoms include insidious abdominal pain, fever, and weight loss. Although these symptoms may be associated with malignancy, fever even in cancer patients should be presumed infectious until proven otherwise. Imaging commonly shows loculated cysts in contrast to patient L’s simple cysts with no loculations. The signs, symptoms, and radiographic findings of abdominal actinomycosis are non-specific and indistinguishable from colorectal cancer, inflammatory bowel disease, and gastrointestinal TB, making diagnosis difficult. In one review, laparotomy was considered essential for diagnosis in 69% of cases; pre-operative diagnosis rate was less than 10%. Treatment generally involves 6-12 months of beta-lactams. In summary, GI actinomycosis poses a diagnostic challenge as it is a rare clinical entity with nonspecific symptoms and endoscopic and radiographic findings. Successful diagnosis often requires a collaborative, multi-disciplinary approach. Treatment is with prolonged antibiotics.
2383 Amiodarone-Induced Acute Liver Injury
Authors: Essrani Rajesh , Mehershahi Shehriyar , Essrani Rajesh , Bhura Sajeer , Mehmood Asif
Published: October 2019

INTRODUCTION:

Amiodarone is a lipophilic structure with a half-life of 25-100 days. Long term oral amiodarone is associated with photosensitivity, thyroid dysfunction, pulmonary, and hepatic toxicity. Hepatic toxicity varies from an asymptomatic and transient rise of serum aminotransferases that resolves after dose reduction or withdrawal, to severe liver disease. Acute hepatotoxicity is a rare but lethal complication of amiodarone use.

CASE DESCRIPTION/METHODS:

A 60-year-old male with a past medical history of hypertension and atrial fibrillation admitted for shortness of breath. He was taking Lopressor 25 mg twice a day and apixaban 5 mg twice a day at home. On exam, blood pressure was 130/70 mmHg, saturation 92% on room air, respiratory rate 13, and heart rate 145, which was irregular. ECG showed atrial fibrillation with a rapid ventricular rate. He was treated with diltiazem bolus and infusion but became hypotensive, so diltiazem infusion was discontinued. Amiodarone 150 mg bolus was given, followed by continuous amiodarone infusion of 900 mg over 24 hours. Blood work performed 24 hours later showed a sudden rise in aminotransferases (aspartate aminotransferase 3254 U/L, alanine aminotransferase 1643 U/L), and direct bilirubin (5.12 mg/dL). International normalized ratio, Gamma-GT, and alkaline phosphatase were normal. Extensive workup like abdominal ultrasonography, viral hepatitis serologies (hepatitis B and C, cytomegalovirus, Epstein-Barr, HIV and herpes zoster viruses) and autoimmune markers (antinuclear antibody, anti-smooth muscle antibody, anti-liver/kidney microsomal antibody type 1) were unremarkable. Amiodarone was discontinued about 50 hours after its beginning and his liver enzymes improved with normalization in 7 days. He was started on oral Lopressor and diltiazem, and his heart rate was well controlled.

DISCUSSION:

Acute intravenous amiodarone-induced hepatotoxicity is a rare but potentially lethal side effect so physicians should be aware of it and check for its occurrence by performing serial liver tests. The exact mechanism for intravenous amiodarone-induced acute liver failure is not well defined, but the hypotensive effect attributable to polysorbate 80 present in intravenous amiodarone is a compelling hypothesis. Treatment is mainly supportive. A recent study favored the use of N-acetylcysteine in acute liver injury beyond acetaminophen intoxication. Liver injury does not recur upon reintroduction of amiodarone by the oral route.
2384 Unusual Cause of Encephalopathy in an Immediate Post Transplant Patient
Authors: Humphrey Kyle , Henderson Phillip
Published: October 2019

INTRODUCTION:

Brain abscesses are a rare complication post liver transplantation. They can present as a wide array of symptoms that can include fever, headache, focal deficits, or altered mental status. Immunosuppression makes transplant patients more susceptible to a wide array of microorganisms. We present a case of Nocardia farsinica brain abscess in a patient 4 months after liver transplantation.

CASE DESCRIPTION/METHODS:

A 76-year-old male with known cirrhosis from fatty liver disease, and recent liver transplant presenting with acute encephalopathy. Four months earlier, the patient received a liver transplant secondary to Nash cirrhosis. His post-operative course showed no infectious complications. He did have issues with mobility and was placed in a rehab facility. He was on tacrolimus as well as azathioprine for immunosuppression. He had hyperkalemia at rehab and his trimethoprim–sulfamethoxazole was held. The patient's family brought him to the hospital for changes in mental status. The patient met sepsis criteria on admission. CT of the head showed multifocal hypoattenuation lesions. MRI with contrast was obtained which showed multiple peripherally enhancing lesions. On peripheral history he had fallen at rehab and developed a hematoma of his thigh. The fluid collection on his thigh which was aspirated. Culture results were concerning for Nocardia. Patient was presumed to have cerebral nocardiosis and was started on imipenem and trimethoprim–sulfamethoxazole. The culture confirmed Nocardia farcinica. During his hospital stay, patient's mental status gradually improved. He was discharged on imipenem and Bactrim. Repeat MRI one year later showed complete resolution of lesions.

DISCUSSION:

Nocardia intracranial abscesses represent a rare complication post liver transplantation; although, infection is a common cause of mortality following liver transplantation. Opportunistic infection prophylaxis is vital and his lapse was likely cause of this infection. Patients that present with fever require expansive evaluation for the common and uncommon infectious causes. Any transplant patient with new or changing headache or altered mental status require imaging of the CNS as well as a lumbar puncture. Imaging, physical exam, cultures and serologies can then further delineate the cause. Brain abscesses have high morbidity and mortality; therefore, prompt diagnosis and treatment is vital.
2389 Nivolumab-Induced Hepatitis: A Rare Side Effect of Programmed Cell Death-1 (PD-1) Inhibitor
Authors: Kang Gurjeet , Ghanekar Dilip , Orpiano Christopher L. , Quinn Michael , Staffetti Joseph , Venu Lakshminarasimhan
Published: October 2019

INTRODUCTION:

Nivolumab is a human immunoglobulin G4 (IgG4) monoclonal antibody that blocks the function of programmed cell death (PD-1) activity by binding to the PD-1 receptor. This prevents suppression of T-cell activity, which can lead to T-cells targeting cancer cells. Unfortunately, these medications induce various types of immune related adverse events including colitis, pneumonitis, endocrinopathies and hepatitis. We report rare case of of nivolumab-induced hepatitis.

CASE DESCRIPTION/METHODS:

An 86-year-old female with history of metastatic melanoma and was started on nivolumab 3 mg/kg every 3 weeks. Patient noted to have abnormal liver chemistries, nausea and yellowing of skin after 6 months of nivolumab. On presentation she was found to have a total bilirubin of 7.79 mg/dL, INR 1.3, AST 885 IU/L and ALT 999 IU/L. Physical examination was significant for mild scleral icterus only. An ultrasound of liver was notable prominent left lobe of liver but no focal hepatic masses and s/p cholecystectomy with no bile duct dilation. Infectious etiologies were unrevealing for viral hepatitis and acute EBV. Her autoimmune serologies were negative for anti-smooth muscle antibody and anti-mitochondrial antibody. Work up was negative for herbal supplements, illicit substance or other medications. The most likely culprit for her hepatitis was due to nivolumab. Patient was started on prednisone at 1 mg/kg daily for a total of 100 mg prednisone daily and her nivolumab dose was held. On discharge her total bilirubin was 2.45, INR 1.2, AST 62, ALT 249 and Alk Phos 252. Patient continued prednisone taper over 30-day period and by end of the month her liver chemistries had normalized and patient was asymptomatic.

DISCUSSION:

Nivolumab-induced hepatoxicity is rare and accounts for 3-6% of all immune-related adverse effects. The median time of onset was 3.3 months (range: 6 days to 9 months). In addition to systemic corticosteroid, alternative immunosuppressive therapies may be employed if symptoms continue after 3-5 days. Etiologies of liver dysfunction such as viral infection, obstruction and other forms of autoimmune hepatitis must be excluded. In such an event, oral mycophenolate mofetil 500 mg every 12 hours can be used for treatment. Our patient showed rapid improvement in liver chemistries and had minimal physical side effects. Healthcare provides managing patients with liver injury should be able to recognize and treat prior to development of fulminant hepatitis.
2390 Trenbolone-Induced Liver Injury
Authors: Kang Gurjeet , Venu Lakshminarasimhan , Orpiano Christopher L. , Quinn Michael , Staffetti Joseph , Ghanekar Dilip
Published: October 2019

INTRODUCTION:

Anabolic-androgenic steroid abuse has been steadily increasing in the general population. According to a 2014 study, approximately 1 in 50 students in the 12th grade reported using anabolic steroids. Trenbelone acetate is a synthetic anabolic androgen which is an agonist of the androgen receptor and most often used in cattle.

CASE DESCRIPTION/METHODS:

A 23 year old male with no significant past medical history presented with complaints of jaundice and generalized weakness which began 10 days prior to presentation. Patient endorsed weekly IM trenbelone 1000 mg weekly over the last 8 weeks. Physical examination was remarkable for icterus and appropriate mentation. Initial investigations reflected a total bilirubin of 13.44 mg/dL, AST 159 IU/L, ALT 425 IU/L, PT 11.0, INR 1.0, and Alk Phos of 142. Serologies were negative for viral hepatitis, anti-mitochondrial antibody, anti-smooth muscle antibody and ANA of 1:40. A liver ultrasound reported normal liver size without biliary obstruction. Patient's liver chemistries improved over the next three days and he was discharged with total bilirubin of 12.54 mg/dL, AST 135 IU/L, ALT 320 IU/L, PT 11.0, INR 1.0 and Alk Phos of 121. Patient advised to abstain from any future use of trenbelone. On repeat labs in 10 days, his total bilirubin of 29.50 mg/dL, AST 78, ALT 123, PT 20.1, INR 1.85 and Alk Phos 242. Physical examination noted worsening jaundice overall along with icterus but mentation remained appropriate. Patient denied any new medications and no further use of trenbelone since discharge. Patient's liver chemistries remained stable and he underwent MRI abdomen which was unremarkable. Patient was discharged home on ursodiol 300 mg BID. Patient's liver chemistry improved in one week and returned to baseline in one month.

DISCUSSION:

Anabolic steroids have been linked to four different forms of liver injury including an acute cholestatic syndrome such as in our patient, elevation in serum enzymes, chronic vascular injury and hepatic tumors including adenomas and hepatocellular carcinoma. Mechanism of cholestatic syndrome is not well defined in this type of injury. Urosdiol has been used in the past for treatment however efficacy has not been shown in a study. Healthcare provides managing patients with elevated liver chemistries should able to recognize anabolic induced liver injury given increasing use and ease of availability of such performance enhancing drugs.
2392 Not so Safe for All: A Case of Statin-Induced Autoimmune Hepatitis and Primary Biliary Cholangitis Overlap
Authors: Hang Thuy-Van , Taborda Cesar , Barrett Amanda , Xue Yue , Vora Ravi
Published: October 2019

INTRODUCTION:

Hydroxymethylglutaryl coenzyme A reductase inhibitors, or statins, are widely used and typically well tolerated. They are rarely associated with severe hepatotoxicity and several randomized trials have demonstrated no significant difference in incidence of persistently elevated liver function tests (LFTs) between statins and placebo. We present an interesting case of drug induced liver injury from atorvastatin resulting in autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) overlap.

CASE DESCRIPTION/METHODS:

A 70 year old woman with a history of stroke with residual dysarthria presented with generalized weakness and decreased appetite for one week, found to have AST of 1347, ALT 807, ALP 230, bilirubin 10.2, INR 1.47, and ferritin 221,358. She did not take any herbals or over-the-counter medications, but was on atorvastatin. Four months before presentation, she had LFTs in the 400-500s, which was attributed to atorvastatin 80mg daily. This was stopped and her LFTs normalized. One month before admission, she was started on atorvastatin 40mg daily, which was discontinued on admission. Hepatitis A, B, and C and HIV were negative, and her right upper quadrant ultrasound was unremarkable. Her workup was significant for positive ANA, dsDNA Ab, anti-centromere Ab, anti-Smith Ab, RNP Ab, F-actin Ab, and smooth muscle Ab, as well as IgG of 5,599. She underwent liver biopsy, which demonstrated panlobular active hepatitis and portal inflammation with ductitis. TPMT was normal, so she was started on azathioprine and prednisone taper for AIH/ PBC overlap. Her labs dramatically improved and on discharge, AST was 375, ALT 641, ALP 184, bilirubin 3.2, and ferritin 2784. She has continued to do well and LFTs and ferritin normalized one month after discharge.

DISCUSSION:

While statins generally have infrequent significant side effects, they can cause hepatocellular injury that does not improve with dose reduction. There have been case reports of statins causing AIH, but in this patient’s unusual case, atorvastatin caused an autoimmune hepatitis/ primary biliary cholangitis overlap. It is important to recognize that in patients who develop LFT elevations more than three times ULN, it may not be safe to restart statins, even at a lower dose. In addition, statin-induced AIH can cause a hyperinflammatory response with significant elevations in multiple acute phase reactants, as well as lead to numerous auto-antibodies such as in the case described.
2393 A Unique Case of Liver Disease in Pregnancy
Authors: Rana Prachi , Han Hyosun
Published: October 2019

INTRODUCTION:

Pregnancy-related liver disease is seen in up to 3% of all pregnancies. Pregnancy-related etiologies include hyperemesis gravidarum, pre-eclampsia, HELLP, intrahepatic cholestasis of pregnancy, and acute fatty liver of pregnancy (AFLP). Given the significant morbidity and mortality associated with these conditions, there is a need for early recognition. Here we present a unique case of pregnancy-related liver disease in an asymptomatic patient. Despite a thorough evaluation for liver and non-liver related causes, her lack of symptomatology and associated laboratory findings made establishing a definitive diagnosis difficult.

CASE DESCRIPTION/METHODS:

30 year-old woman G1P0 at 29 weeks gestation with dichorionic triamniotic triplets presented with generalized weakness. Her past medical and surgical histories were otherwise non-contributory and her only medication included prenatal vitamins. The physical exam was unremarkable except for a gravid uterus. Throughout her hospital course, she remained normotensive. Admission labs were significant for an abnormal liver profile: AST 200 U/L, ALT 349 U/L, ALP 121 U/L, and total bilirubin 0.5 mg/dL, compared to a previous normal baseline. Complete blood count and renal function were normal. Abdominal ultrasound (US) showed increased liver echogenicity consistent with fatty liver disease. Additional work-up revealed a normal coagulation profile, fibrinogen, lactate dehydrogenase, and haptoglobin. Urinalysis was negative for proteinuria. Serologies for viral and autoimmune hepatitis were negative. Total bile acids were mildly elevated at 9.3 umol/L (normal < 6.8umol/L) and 24-hour urinary copper was normal. Though she remained asymptomatic, liver enzymes continued to uptrend. On day 16 she underwent urgent C-section due to rising liver enzymes and evidence of fetal distress. Liver enzymes continued to rise until POD1 before rapidly declining.

DISCUSSION:

This was a unique case of pregnancy-related liver disease of unknown etiology, but most consistent with an early manifestation of AFLP. We suspected AFLP based on the steatosis noted on US, however, since we could not check her children for LCHAD deficiency a definitive diagnosis could not be made. Surveillance US showing resolution of steatosis would have helped confirm the diagnosis however the patient was lost to follow up. This was an interesting presentation of AFLP as most cases are discovered in women presenting with acute liver failure; this case was unique in that it was discovered unexpectedly.
2401 A Case of Hepatitis B Reactivation
Authors: Haddadin Sadam H. , Martinez Beatriz , Ravendhran Natarajan
Published: October 2019

INTRODUCTION:

It is estimated that more than two billion people around the world have been infected by hepatitis B virus (HBV). Several immunosuppressive agents have been associated with HBV reactivation (HBVr). We present a case of HBVr in a patient with ulcerative colitis (UC) treated with tofacitinib.

CASE DESCRIPTION/METHODS:

A 57-year-old female with UC on tofacitinib presented to the emergency department with flu-like symptoms for 2 months after being switched from azathioprine to tofacitinib. She was HBV surface antigen (HBsAg) negative, anti-HBV core antibody (anti-HBcAb) and anti-HBV surface antibody (anti-HBsAb) positive and had normal liver enzymes before initiation of tofacitinib. On initial evaluation she was febrile, hypotensive, and had lower extremity edema. Laboratory data was significant for total bilirubin 2.5 mg/dL, AST 1601 U/L, ALT 1013 U/L, positive HBsAg, anti-HBsAb, total anti-HBcAb and HBeAg, HBV DNA 10,354,246 IU/m; she had negative HBeAb, IgM antibody to HBcAg, hepatitis A, C and E antibodies. She was diagnosed with HBVr and was started on tenofovir disoproxil fumarate. Over the next two days her enzymes continued to rise and peaked at day 5. At day 7 liver enzymes started to trend down. She was later discharged on tenofovir. At the 2 month follow up liver enzymes had normalized and HBV DNA was down to 30 IU/m.

DISCUSSION:

Tofacitinib is a kinase inhibitor extensively studied in patients with rheumatoid arthritis and has been shown to be generally safe in multiple clinical trials. In patients with UC the data is limited. Most clinical trials excluded patients with chronic HBV infection, therefore the risk of HBVr among tofacitinib-treated patients remains unknown. HBVr is defined as an increase in HBV DNA of more than 100-fold in patients with previously detected HBV DNA, detection of HBV DNA in patients that previously had undetectable titers or when reverse seroconversion or development of detectable HBsAg occurs. In this case, the reactivation of HBV was most likely induced by tofacitinib and its suppression of various kinase signaling pathways essential for controlling immune activation and proliferation the cells responsible of preventing HBV replication. In conclusion, in patients with resolved HBV infection who are HBsAg negative and anti-HBcAb positive, periodic HBV DNA monitoring should be considered as HBVr is possible and the exact risk of reactivation remains unknown.
2404 Hepatic Double Trouble—A Case of Combined Hepatocellular Carcinoma and Cholangiocarcinoma
Authors: Ponna Pramod , Saxena Rovin , Kulkarni Mayuri , Diaczok Benjamin , Krishnamoorthy Geetha
Published: October 2019

INTRODUCTION:

Combined Hepatocellular carcinoma and Cholangiocarcinoma (cHCC-CC) accounts for <1% of all liver malignancies and is difficult to diagnose pre-operatively. We present a case of cHCC-CC diagnosed by imaging, tumor markers and biopsy.

CASE DESCRIPTION/METHODS:

A 61-year-old male with advanced cirrhosis due to hepatitis C and alcohol, was admitted for hepatic encephalopathy. His alpha-fetoprotein (AFP) was elevated at 17, 771 ng/mL. MRI abdomen revealed a 2 cm mass that showed early enhancement and rapid wash-out consistent with HCC in the right lobe and 1 lesion in the left lobe that showed hyperenhancement in post contrast images which was not consistent with HCC. CA 19-9 level was elevated at 54.3 U/mL. Biopsy of the left lobe lesion showed Cholangiocarcinoma. Metastatic work up (CT chest, MRI brain, bone scan) was negative. Patient and his family chose comfort care due to the poor functional status and need for hepatectomy.

DISCUSSION:

There are 3 types of cHCC-CC: In type A, HCC and CC are seen at different sites in the liver, as in ours. Type B consists of juxtaposed HCC and CC that mingle with ongoing growth and type C has both within the same tumor. Imaging shows HCC features of arterial enhancement and washout and CC features of irregular surface and late enhancement. The presence of both elevated AFP and CA 19-9 should lead to the suspicion of cHCC-CC. Hepatectomy is the only curative treatment for cHCC-CC and transplant is contraindicated. Survival after resection for cHCC-CC is worse than HCC.