bluuebunny/crossref_metadata_2025 huggingface dataset:
2399 West Nile Encephalitis in a Post-Liver Transplant Patient
Authors: Yang Grace H. , Apfel Tehilla , Shah Mehul , Krawitz Steven
Published: October 2019

INTRODUCTION:

West Nile Virus (WNV) is a neurotropic flavivirus that is transmitted from animals to humans by the bite of the Aedes, Anaphales, or Culex mosquito, with disease incidence peaking in the late summer and early fall. It is the leading cause of arboviral illness in the U.S. While many infected with WNV are asymptomatic, 25% of patients develop fever, and less than 1% of patients will develop neurologic symptoms. Age over 75-years old and immunosuppressed state are risk factors for developing neuroinvasive disease.

CASE DESCRIPTION/METHODS:

A 65-year old woman with a history of HCV cirrhosis complicated by HCC status post successful orthotopic liver transplantation (OLT) in 2016 compliant with tacrolimus and everolimus immunosuppression (ISP) presented with painless jaundice and acute transaminemia. Liver biopsy revealed severe acute cellular rejection (ACR), so steroids and thymoglobulin were started. Tacrolimus was increased and everolimus was changed to mycophenolate mofetil. Clinically, she appeared well and her LFTs responded to treatment. Suddenly, she developed fever to 103º associated with bilateral hand tremors. Infectious workup was obtained, and she was started on broad-spectrum antibiotics. Initially, symptoms were attributed to changes in ISP and treatment of ACR. However, she developed progressively worsening dyskinesia and encephalopathy eventually requiring intubation. Given initial negative infectious workup, lumbar puncture was obtained; see Table 1. HSV, VZV, and Lyme PCR as well as VDRL in the CSF were negative. Given clinical symptoms, positive WNV IgM in the CSF, and MRI Brain concerning for WNV neuroinvasive disease, patient was treated with IVIG for 5 days. WNV IgG became positive as well on repeat lumbar puncture 2 weeks later. Patient’s encephalopathy improved, and she was able to be extubated.

DISCUSSION:

There may be a delay in diagnosis and treatment of WNV encephalitis in post-liver transplant patients as neurologic symptoms may be attributed to ISP or as in this patient, to the treatment of acute cellular rejection. West Nile encephalitis must remain on the differential diagnosis when neurologic symptoms are present in a post-liver transplant patient, especially in endemic areas during the summer and fall months. A change in this patient’s ISP for the treatment of ACR may have caused the development of a more severe, neuroinvasive response to WNV infection and ISP management in such patients should be studied further.
2400 An Unusual Case of Spontaneous Reactivation of Hepatitis B in Adult T-Cell Lymphoma/Leukemia
Authors: Alexandre Cassidy , Batool Asra
Published: October 2019

INTRODUCTION:

Reactivation of hepatitis B (HBV) is typically triggered by immunosuppressive therapy of cancer, autoimmune disease, or organ transplantation. Here we report a rare case of spontaneous reactivation of hepatitis B in a patient newly diagnosed with adult T-cell lymphoma/leukemia without any exposure to immunosuppressive therapy of cancer.

CASE DESCRIPTION/METHODS:

A 34-year-old African American male was transferred from an outside institution for further management of probable T-cell lymphoma/leukemia as severe lymphocytosis was initially noted. Presenting symptoms included weakness and fatigue as well as bilateral conjunctival redness. His abdomen was mildly distended, and hepatomegaly was noted. Of interest, he was found to have elevated liver function tests (LFTs) and hepatitis panel showed acute hepatitis B with bloodwork significant for positive hepatitis B surface antigen, negative hepatitis B surface antibody, negative hepatitis B core IgM, positive hepatitis B core antibody and hepatitis B viral load of 16,954. Labs for hepatitis A and C were negative. He denied any known risk factors for hepatitis. Abdominal imaging showed peripancreatic concern of mass or swelling, mild hepatomegaly, splenomegaly and marked lymphadenopathy in the celiac, perigastric, pancreatic and aortocaval regions. Of note, he has not been on any immunosuppressive therapy or steroids prior to hospital admission. On hospitalization day #5, he was started on tenofovir and subsequently liver function tests continued to improve. He was also started on IV Solu-Medrol 1 g per day for 3 days with IV hydration and oral allopurinol. He continued to undergo diagnostic work-up as per hematology and Oncology prior to starting chemotherapy. On the day of discharge (day #17), remarkable improvement in his LFTs was noted. He was seen in GI clinic about 3 weeks later and his hepatitis B viral load was 760 and ALT was within normal limits.

DISCUSSION:

HBV reactivation is common following chemotherapy and is associated with a high mortality, however spontaneous HBV reactivation in the absence of cancer chemotherapy or transplantation is a rare clinical entity. Adult T-cell lymphoma/leukemia likely contributes to immune suppression or alteration in immune function causing spontaneous HBV reactivation. This case highlights the importance of screening all patients who are to undergo cancer chemotherapy, marked immunosuppressive treatments or solid organ or bone marrow transplantation for evidence of ongoing or previous hepatitis B.
2406 Cinnamon-Induced Acute Hepatitis
Authors: Odish Fadi , Karmo Dillon , Imam Zaid , Hanna Angy , Aneese Andrew
Published: October 2019

INTRODUCTION:

There is no denying the increasing interest in alternative medical practices in the United States, especially in regards to supplement use. According to a recent survey, 33.2% of adults in the US used complementary health approaches, and 17.7% reported using a supplement. As the use of supplements rises, it is imperative that physicians recognize the potential adverse effects. One example of this is the popular practice of using cinnamon supplementation to improve glycemic control in Diabetes. However, less attention has been given to a potential adverse effect of cinnamon use. Coumarin is a compound found in cinnamon and its metabolite are hepatotoxic. Our patient presented with hepatitis triggered by the use of a seemingly innocent supplement.

CASE DESCRIPTION/METHODS:

A 51 year old male with a past medical history of hyperlipidemia presented with 10 days of right upper quadrant abdominal pain, jaundice and dark urine. He denies tobacco, alcohol, or illicit drug use. Home medications are aspirin and atorvastatin. Only new medication is cassia cinnamon supplements for the past month. Physical exam was significant for scleral icterus, jaundice, abdominal distention, and tenderness to palpation in the RUQ. Lab work revealed a CBC within normal limits, and elevated LFTs. Acute hepatitis panel, ANA, anti-smooth muscle antibody, anti-mitochondrial antibody, ferritin, HIV, HSV, EBV, and CMV were negative. An ultrasound of the abdomen was unremarkable. His atorvastatin and cinnamon supplements were held. An MRCP was obtained and was unremarkable. His symptoms and LFTs improved and he was discharged home after two days of hospitalization with instructions to hold the atorvastatin until his liver function tests normalized, and to discontinue the cassia cinnamon. The acute hepatitis was determined to be due to the cassia cinnamon supplementation with concomitant statin use.

DISCUSSION:

This case illustrates the importance of a thorough history when evaluating a patient. Special attention must be paid to over the-counter medications, herbs, and supplements. Often times patients will not volunteer this information when asked about what medications they take, so they must be specifically prompted. Given the increasing popularity of alternative and “natural” medical practices, it is also imperative that physicians stay up to date on the potential adverse effects. Emphasis needs to be placed on scientific investigation into potential risks and benefits of popular alternative medical practices.
2415 Seeing Is Believing: Conjunctival Suffusion in a Jaundiced Patient Diagnosed With Leptospirosis
Authors: Almoghrabi Anas , Nguyen Alexander , Patel Amit , Attar Bashar M.
Published: October 2019

INTRODUCTION:

The broad differential in the workup of infectious causes of abnormal liver enzymes can be narrowed by a good history and physical. Bilateral Conjunctival suffusion, characterized by non-purulent conjunctivitis, is present in 55% of patients with leptospirosis and is uncommon in other infectious diseases. We report a case of leptospirosis in a patient with conjunctival suffusion, abnormal liver enzymes, jaundice, and recurrent fevers.

CASE DESCRIPTION/METHODS:

A 44-year-old previously healthy Hispanic male presented with painless jaundice, subjective fevers, nonproductive cough, and a morbilliform rash for 14 days. He reported no recent travel, sick contacts, bad habits, or hepatotoxic herbal medications, and he worked in a slaughterhouse. He denied joint pain, abdominal pain, and pruritus. On exam, he had a temperature of 38.2°C and was tachycardic to 117 with normal blood pressure. He had bilateral non-purulent conjunctivitis with mild scleral icterus and a faint morbilliform rash over both his thighs. Laboratory data was remarkable for leukocytosis (14.9 WBC/mm3), elevated bilirubin (4 mg/dL total, 2.3 mg/dL direct), Alkaline phosphatase (517 U/L), AST (84 U/L) and ALT (162 U/L). Imaging studies revealed hepatic steatosis with no other findings. Infectious workup indicated that leptospira antibody to IgM were likely present based on DOT BLOT testing (ARUP labs). Serologic testing for other infectious causes were unremarkable. The patient was started on doxycycline 100 mg twice a day for one week for mild leptospirosis with improvement in his symptoms, labs, and clinical exam.

DISCUSSION:

Leptospirosis, although endemic in the tropics, is a rare cause of jaundice and abnormal liver enzymes in the United States. It is associated with environmental exposure such as animal urine, contaminated water or soil, or infected animal tissue. Gastrointestinal clinical features include abdominal pain, jaundice, elevated hepatic transaminases and serum bilirubin. An important but frequently overlooked clinical finding is bilateral conjunctival suffusion, which is rare in other infectious diseases. Diagnosis is made by serologic testing for IgM antibodies. Treatment involves supportive care with oral doxycycline in mild cases and IV antibiotics for hospitalized patients. An astute physician should have a high index of suspicion for leptospirosis if non-purulent conjunctivitis is found in the infectious workup of fever and abnormal liver enzymes as it may save time and prevent excessive testing.
2425 A Rare Case of Alloimmune Hepatitis Induced After Direct-Acting Antiviral Treatment in a Liver Transplant Patient
Authors: Choi Catherine , Botros Youssef , Shah Jamil M. , Jones Anja , Nasir Umair M. , Panchal Dayna , Pyrsopoulos Nikolaos T.
Published: October 2019

INTRODUCTION:

Cases of autoimmune hepatitis induced after Direct-acting antiviral (DAA) in patients undergoing treatment for hepatitis C virus (HCV) infection have been reported in the literature. This is a case report of alloimmune hepatitis in a post-liver transplant patient shortly after starting DAA treatment for recurrent hepatitis C in the graft.

CASE DESCRIPTION/METHODS:

A 56-year-old Egyptian woman with history of liver cirrhosis secondary to chronic HCV, genotype 4, complicated by hepatocellular carcinoma underwent orthotopic liver transplant. Two months later, an increase of the aminotransferases and the HCV viral load of 734,703 IU/mL were noted. Liver biopsy was suggestive of recurrent HCV infection. Treatment was started with sofosbuvir, velpatasvir, and voxilaprevir. Repeat HCV viral load was 537 IU/mL 2 weeks after starting DAA. At this point she presented with jaundice, nausea, and vomiting. Vital signs were normal. Scleral icterus and moderate abdominal tenderness were noted. AST 1,395 U/L, ALT 1,359 U/L, ALP 601 U/L, total bilirubin 12.2 mg/dL, direct bilirubin 9.6 mg/dL, and INR 1.5 were noted. Liver biopsy showed hepatitis with abundant plasma cells and interface activity, compatible with an alloimmune process. The patient was started on IV methylprednisolone and N-acetyl cysteine. Her symptoms resolved and liver enzymes normalized on subsequent follow up. She achieved sustained viral response.

DISCUSSION:

Alloimmune hepatitis is a rare cause for graft dysfunction that can occur among patients who have undergone liver transplantation for other reason than autoimmune hepatitis. The exact pathogenesis is not fully understood but believed to share similar mechanisms with classical autoimmune hepatitis. Calcineurin inhibitors may cause impairment of the regulatory T-cells function by reducing the production of IL-2, a cytokine important for the regulatory T-cells proliferation. The treatment of recurrent HCV with interferon can stimulate the activation of T-cells, which enhance the presentation and release of antigens. With DAA therapy's anti-HCV activity and its potential to stimulate host immunity, it has been suggested that rapid elimination of HCV might favor a dysregulation of immune surveillance, which precipitates alloimmune process. In summary, we report a rare case of alloimmune hepatitis in a liver transplant patient with recurrent HCV responding to DAAs and steroid. Thorough follow up of patients undergoing DAA therapy after liver transplantation for alloimmune hepatitis is warranted.
2427 A Case of Hepatitis B Virus-Related Membranous Nephropathy in an Adult With Low Hepatitis B Viral Load
Authors: Tan Chin Kimg , Tay Hui Boon , Kumar Rajneesh
Published: October 2019

INTRODUCTION:

Hepatitis B virus (HBV) infection is an important etiologic factor for secondary membranous nephropathy (MN), especially in regions with endemic HBV infection. Though an uncommon occurrence, it is crucial to recognize HBV-related MN as it may progress to renal failure in untreated adults. In patients with HBV infection, it is critical to exclude other secondary causes of MN. This case describes a case of HBV-related MN in a HBeAg-negative patient with low HBV viral load.

CASE DESCRIPTION/METHODS:

Mr C is a 64 year old gentleman who has underlying chronic HBV infection and Stage 3b chronic kidney disease. He presented with bilateral lower limb swelling. He was found to have hypoalbuminemia and nephrotic range proteinuria associated with microscopic hematuria. He was in “inactive chronic HBV carrier infection” phase with negative hepatitis B e antigen (HBeAg), positive anti-HBe and low HBV viral load 2.68 log IU/ml with positive antinuclear antibody and low complement C3. Other workup for secondary causes of membranous nephropathy was unyielding. Renal biopsy showed membranous nephropathy with focal proliferative glomerulonephritis. Immunofluorescence was not suggestive of lupus nephritis and staining for antiphospholipase A2 receptor was negative. He was diagnosed as HBV-related MN and he was treated with Tenofovir Alafenamide. Six months after HBV treatment, his proteinuria has improved with stable renal function. HBV viral load was suppressed to less than 1.3 log IU/ml but he still had high titer of HBsAg (7000 IU/ml) as expected.

DISCUSSION:

In HBV-related MN, HBV treatment is recommended as previous studies showed that HBV treatment and HBeAg clearance are associated with remission of MN. However, treatment endpoint remains unclear. The pathogenesis of HBV-related glomerular disease is due to the deposition of HBV antigens immune complexes in the glomerulus, especially to HBeAg. Therefore, MN can occur in patients with low HBV viral load. In HBeAg-positive patients, it may be reasonable to consider stopping antiviral following clearance of HBeAg. In HBeAg-negative patients, HBsAg immune complexes may contribute to MN. As in this case, it may be prudent to continue with HBV therapy as he has persistently high HBsAg titer. Future research is needed to find reliable HBV markers to guide management and define clinical end points for patients with HBV-related MN.
2432 Incidental Diagnosis of Merkel Cell Carcinoma in a Liver Transplant Patient Admitted for Diverticulitis
Authors: Prochot Jeffrey , Andersen Karl , Hoff Ryan T.
Published: October 2019

INTRODUCTION:

Merkel cell carcinoma (MCC) is a rare and highly aggressive neuroendocrine skin cancer. Most patients (80%) who develop MCC have been infected with a Merkel cell polyomavirus. Sun exposure, fair skin, and age are risk factors; however, it occurs most commonly in immunosuppressed patients, specifically organ transplant and HIV-infected patients. Though the exact mechanism is still unknown, it is thought thatimmunosuppression contributes to viral integration, mutagenesis, and carcinogenesis of the virus.

CASE DESCRIPTION/METHODS:

75-year-old white male with a history of liver transplantation for NASH cirrhosis, maintained on mycophenolate and tacrolimus, was admitted to the hospital for diverticulitis. Lab results showed pancytopenia. CT abdomen showed acute diverticulitis, and a nonspecific soft tissue density near the porta hepatis encasing the common and proper hepatic arteries (Figure 1). A focused physical exam showed a large axillary mass (Figures 2 and 3). His diverticulitis improved with a course of broad-spectrum antibiotics. The mass was biopsied and positive for MCC, suggesting the lesion near the porta hepatis represented metastasis. The axillary mass was excised and he received local radiation therapy. He was unable to undergo chemotherapy due to poor performance status. Within 10 months of the diagnosis, the patient died.

DISCUSSION:

While MCC is most commonly associated with the long-term immunosuppression following solid-organ transplantation, its occurrence is also rising in older adults whose immune function naturally declines with time. The median onset of MCC is greater than 65 years of age, and occurs most often in white patients. The most common site of origin is the head and neck region, but it can arise anywhere in the body. The diagnosis of MCC can be challenging due to the innocuous appearance of the primary tumor. This case demonstrates the importance of understanding the potential sequela of immunosuppressive medications in organ transplant patients and performing regular and thorough physical exams in order to quickly detect and treat diseases like MCC.
2438 An Unusual Cause of Jaundice: A Rare Diagnosis of Crigler-Najjar Type 2
Authors: Patel Krishen , Prabakaran Sabrina , Kapadia Nikesh
Published: October 2019

INTRODUCTION:

Crigler Najjar is an uncommon genetic disorder that results in elevated unconjugated bilirubin due to its impaired conjugation and excretion within the liver. Here we report a case of this unusual disorder in a relatively asymptomatic patient.

CASE DESCRIPTION/METHODS:

A 21-year-old woman with no significant past medical history presented with 1 year of intermittent jaundice, 20 pound unintentional weight loss, and decreased appetite. The patient reported recent travel to Cuba, but denied any known sick contacts. She also denied any pruritis, abdominal pain, nausea, or diarrhea. Her family history was notable for pancreatic adenocarcinoma. Social history was unremarkable. On physical exam, the patient was ill appearing with moderate scleral icterus and jaundice. Lab work demonstrated isolated unconjugated hyperbilirubinemia (Total Bilirubin: 7.5, conjugated: 0.4, unconjugated: 7.1). Right upper quadrant ultrasound and CT abdomen and pelvis were unrevealing as to the etiology of the patient's hyerbilirubinemia. Extensive testing for a variety of infectious, hereditary, hematological, and rheumatologic disorders were all negative as well. Further advanced genetic testing revealed a heterozygous UGT1A1 gene mutation, ultimately diagnosing the patient with Crigler-Najjar type 2 (CN2). Her symptoms resolved spontaneously without any intervention.

DISCUSSION:

CN2 is due to a partial deficiency of the enzyme uridine diphosphate-glucuronyl transferase (UGT1A1). Unlike the homozygous form of the disease where there is a total deficiency of the enzyme, usually resulting in death at an early age, the heterozygous form (type 2) is typically more benign in its course. While patients are relatively asymptomatic, they can develop hyperbilirubinemia in stressful situations, after surgery, or with certain medications. It is important to consider CN2 in patients with intermittent unconjugated hyperbilirubinemia. This is especially true in young women, such as this patient, as it can affect neonates during pregnancy. Unfortunately, due to the low prevalence of this, it is often a diagnostic challenge for clinicians.
2453 Inferior Vena Cava Syndrome in Hepatocellular Carcinoma With Tumor Extension Into the Right Atrium
Authors: Kobeszko Matthew , Anand Sheena , Berkelhammer Charles
Published: October 2019

INTRODUCTION:

Cardiac involvement in hepatocellular carcinoma is rare. This includes intra-atrial metastases, and tumor thrombus with extension of tumor into the inferior vena cava and the right atrium. We describe a patient presenting with inferior vena cava syndrome complicating hepatocellular carcinoma with extension of tumor into the inferior vena cava and the right atrium.

CASE DESCRIPTION/METHODS:

An 83 year old female with a history of hepatocellular carcinoma and underlying cirrhosis presented with bilateral leg edema to her thighs, and abdominal distension. She had a history of hepatitis C successfully treated with direct acting anti-viral therapy. Her hepatocellular carcinoma was treated with radiofrequency ablation. Several months later, she received transarterial chemo-embolization for recurrent hepatocellular carcinoma. She remained well for one year and was lost to follow-up, until she presented with bilateral leg edema, abdominal distension and shortness of breath. Physical exam showed no jugular venous distension. She had moderate abdominal distension with shifting dullness, hepatomegaly, and edema to both lower extremities extending to her thighs. Computed tomography of her abdomen with and without contrast revealed hepatomegaly with a large tumor mass in the left lobe of the liver extending into the inferior vena cava and right atrium. The thrombus in the inferior vena cava and right atrium showed contrast enhancement. Ascites was present. 2D echo showed tumor thrombus in her right atrium. The patient was diagnosed with inferior vena cava syndrome due to extension of hepatocellular carcinoma into her inferior vena cava and right atrium. She had some improvement with diuretics, and was discharged home to hospice care.

DISCUSSION:

Hepatocellular carcinoma with tumor extension into the inferior vena cava and atrium is rare. This can present with inferior vena cava syndrome, superior vena cava syndrome, or both. Aggressive treatments have been performed in this situation, including surgical resection of the atrial tumor thrombus while under cardio-pulmonary bypass, with or without resection or ablation of the hepatocellular carcinoma. Given the understandably poor prognosis and age of the patient, hospice care was chosen. Tumor extension into the inferior vena cava and right atrium should be considered in patients with advanced hepatocellular carcinoma presenting with inferior vena cava syndrome.
2462 Rapid Development of Bleeding Esophageal Varices After Placement of Continuous Flow Left Ventricular Assist Device
Authors: Majmudar Kaushal , Northcutt Michael , Gordon Robert , Fimmel Claus
Published: October 2019

INTRODUCTION:

Upper gastrointestinal bleeding – typically due to arterio-venous malformations - is common in patients with continuous-flow LVAD, and is due to acquired von Willebrand disease and systemic anticoagulation. We present a patient with compensated cirrhosis in whom large esophageal varices developed within a few weeks of LVAD placement, resulting in hemorrhage and eventual fatal outcome.

CASE DESCRIPTION/METHODS:

A 62-year old male with a history of non-ischemic cardiomyopathy presented with recurrent pulmonary edema requiring intra-aortic balloon pump (IABP) to maintain stability. Workup for placement of a LVAD was initiated. An abdominal CT scan unexpectedly revealed cirrhotic liver changes. The patient had no physical exam stigmata of advanced liver disease. The INR was 1.1, platelets 170,000, albumin 3.7, transaminases and bilirubins were normal. EGD revealed no varices. A liver biopsy revealed a portal-systemic gradient of 20 mmHg and histology confirmed cirrhosis but revealed no specific etiology. The patient's MELD score of 11 and Child-Pugh class A were consistent with well-compensated cirrhosis, and the patient underwent uneventful placement of a LVAD. Seventeen days after placement, the patient sustained an acute upper GI bleeding episode. Emergent EGD revealed large, actively bleeding esophageal varices, which were successfully ligated. Over the following days, the patient developed ascites, hepatic encephalopathy and renal failure. Twelve days later, the patient developed recurrent bleeding. EGD revealed active bleeding in the esophagus and a large clot. No specific bleeding source could be identified and endoscopic attempts at hemostasis were unsuccessful. Placement of a transjugular intrahepatic portosystemic shunt was considered, but the patient's MELD score of 40 was prohibitively high. The patient continued to bleed and expired on the following morning.

DISCUSSION:

We hypothesize that the fluid shifts and increased hepatic arterial inflow following LVAD placement caused rapid increases in esophageal variceal pressures and bleeding. The patient's low cardiac output prior to LVAD placement likely masked the extent of his portal hypertension. In hindsight, the markedly elevated pre-LVAD portal-systemic gradient was the clearest predictor of post-LVAD variceal complications in our patient. Our experience suggests that even well-compensated cirrhotic patients with significant portal hypertension are at risk for variceal bleeding after LVAD placement.
2465 A-DRESS-ing the Big Picture: Liver Injury as Evidence of DRESS Syndrome
Authors: Moon Jin A. , Wayde Melinda , Musleh Mustafa
Published: October 2019

INTRODUCTION:

Drug reaction with eosinophilia and systemic symptoms syndrome is a rare and potentially life-threatening form of drug hypersensitivity which can be difficult to diagnose due to its long latency period and nonspecific symptoms. We present a case of a woman diagnosed with DRESS after a gastroenterology consult for elevated LFTs.

CASE DESCRIPTION/METHODS:

A 60-year-old female with a complex past medical history including polysubstance abuse presented to the hospital with fever, diffuse body rash, shortness of breath and altered mental state. Four weeks prior to her admission, the patient was hospitalized for alcohol withdrawal. An EEG at that time showed status epilepticus and she was placed on phenytoin and levetiracetam, then discharged. On the current admission, the patient was afebrile, tachycardic, tachypneic, hypotensive. The patient was on a ventilator, diffusely tender to palpation of her abdomen, and had a diffuse brawny rash. Her labs showed an elevated leukocyte count of 27.7 K/mm3 with 30% eosinophils and elevated ALP of 206 IU/L. CXR showed left basilar consolidation, consistent of pneumonia. She was started on antibiotics, but was suspected to have an adverse drug reaction. Few days later her LFTs worsened. ALP went up to 1315 IU/L, AST 294 U/L, ALT 260 U/L, but bilirubin was normal. Further workup to rule out other causes of liver injury, including an acute hepatitis panel, ANA, AMA, and ASMA were all negative. RUQ ultrasound and CT scan of the abdomen were unrevealing. ALP levels continued to increase to 1673 IU/L and WBC peaked at 60 K/mm3 with 50% eosinophilia. DRESS was suspected secondary to anticonvulsants started from prior hospitalizations. Phenytoin and levetiracetam were discontinued and changed to zonisamide. She was closely monitored in the hospital for several weeks. Her pulmonary function, leukocytosis, eosinophilia, and LFTs improved and she was discharged to a nursing facility where she fully recovered.

DISCUSSION:

DRESS syndrome is a severe form of drug hypersensitivity that involves the skin and often other visceral organs. Although our patient had many typical findings of DRESS, including fever, rash, abnormal LFTs, leukocytosis, and eosinophilia, diagnosis was challenging due to the patient's multiple comorbidities and prior hospital admissions initially overlooked. As treatment hinges on cessation of the culprit agent, it is essential to get a complete history and evaluate the patient as a whole to avoid missing this potentially life-threatening condition.
2474 Identification of Large Liver Mass of Unknown Origin
Authors: Gochanour Eric , Rank Joseph , Kowdley Kris
Published: October 2019

INTRODUCTION:

Liver lesions of unknown etiology are commonly encountered at tertiary referral centers. Differential diagnoses include both benign and malignant neoplasms. Most often, lesions can be identified based solely on characteristic imaging findings; however, in rare cases, biopsy should be considered for definitive diagnosis. Here we describe the diagnostic work up of a large liver mass secondary to metastatic uveal melanoma.

CASE DESCRIPTION/METHODS:

65-year-old male with no personal or family history of liver disease who was referred for a large liver mass discovered during work up of progressive abdominal pain and weight loss. Patient reported moderate alcohol use (2-4 alcoholic drinks per night) but had no other risk factors for the development of liver disease. Physical exam revealed a large, tender mass crossing the abdominal midline. Labs unremarkable including normal tumor markers. Imaging revealed 14.7 cm mass taking up majority of the left hepatic lobe (Figure 1). Vibration controlled transient elastography showed a low stiffness score indicating very low likelihood for advanced fibrosis/cirrhosis. Case was discussed at multidisciplinary liver tumor board. Mass fulfilled all requirements of LI-RADS 5 lesion although LI-RADS designation did not apply as patient was not at elevated risk for hepatocellular carcinoma (HCC). No evidence of metastasis on initial imaging. Patient subsequently referred for possible left hepatectomy. Preoperative studies showed evidence of extensive metastases. Therefore, mass was deemed non-resectable. Patient consequently referred to Medical Oncology. Liver biopsy was performed which was consistent with metastatic malignant melanoma (Figure 2). Further staging studies showed hypermetabolic activity involving L orbital globe, bilateral lungs and liver indicating continued disease progression (Figure 3). Patient started on nivolumab and ipilimumab for rare metastatic uveal melanoma with extensive hepatic as well as lung metastases. Unfortunately, tumor remained very aggressive and patient died within 3 months of initial imaging findings.

DISCUSSION:

This case highlights the importance of good history taking and maintaining a broad differential during evaluation of a liver lesion of unknown origin. While this lesion met all diagnostic criteria for HCC, it was ultimately identified as metastatic melanoma. This underlines the sustained role of liver biopsy in indeterminate liver lesions in patients without liver disease.
2475 Polycystic Liver Disease Causing Inferior Vena Cava Compression and Venous Thromboembolism
Authors: Strader Mary , Richter Seth , Sunil Narayan
Published: October 2019

INTRODUCTION:

Polycystic Liver Disease (PLD) is a rare disorder that can be diagnosed in patients with greater than 10 fluid filled-cysts identified on imaging. PLD is associated with two genetic disorders autosomal dominant polycystic liver disease and autosomal dominant polycystic kidney disease. Since PLD is uncommon, it is difficult to study both the natural history of the disease and treatment modalities. We present a case of a massive hepatic cyst in a 95-year-old patient with polycystic liver disease that led to inferior vena cava compression and venous thromboembolism.

CASE DESCRIPTION/METHODS:

A 95-year-old male with past medical history of colon cancer status-post resection twenty-five years ago, with no subsequent recurrence and hemorrhagic hepatic cyst, two months prior, presented for evaluation of abdominal distension, lower extremity edema and shortness-of-breath. Physical exam was remarkable for a firm distended abdomen with hepatomegaly and bilateral lower extremity pitting edema. Laboratory evaluation showed a hemoglobin of 8.6 mg/dL, with a baseline hemoglobin of 8.0 mg/dL on prior admission. An abdominal CT scan showed numerous hepatic cysts with an enlarging right hepatic lobe cyst, measuring 25 × 25 × 30 cm, with interval resolution of blood products within the cyst, and an incidentally noted pulmonary embolism in the right lower lobe. The size of the cyst led to compression of the inferior vena cava and displacement of the right kidney. Ultrasound revealed that it was multiseptated in nature. Image-guided drainage and catheter placement was performed, revealing 5.7 liters of clear brown fluid. Cytologic evaluation revealed no evidence of malignancy and aerobic and anaerobic cultures showed no growth. A duplex ultrasound of the lower extremities exhibited acute thrombus of the left deep femoral vein and sluggish flow throughout all vessels. Given the patient's prior hemorrhage, anticoagulation was contraindicated and an inferior vena cava filter was placed. At the time of filter placement, the cyst had decreased in size and sclerotherapy was performed. The patient felt substantial improvement in his dyspnea and abdominal distension and was discharged home with plans for sclerotherapy as an outpatient.

DISCUSSION:

PLD is a rare disorder which makes the natural history difficult to define. Our case further clarifies the association of PLD with external compression of the inferior vena cava. It adds data to the literature by illustrating the complication of venous thromboembolism.
2479 Autoimmune Hepatitis Triggered by Hepatitis A: An Interesting Case and Review of Pathophysiology
Authors: Gala Khushboo , Bierman Thomas , Luckett Robert T. , Barve Ashutosh
Published: October 2019

INTRODUCTION:

Autoimmune hepatitis (AIH) is an immune-mediated disorder of the liver, whose pathophysiology involves triggering of T-cell mediated events by environmental agents in the liver in a host genetically predisposed to this disease. One trigger described in literature has been the hepatitis A virus (HAV). Hence, occasionally cases which clinically appear to be that of relapsing HAV are in fact concealing underlying autoimmune hepatitis that has been provoked from the initial episode of HAV. We describe one such case.

CASE DESCRIPTION/METHODS:

A 45 year old Caucasian male with past medical history of chronic hepatitis C and acute hepatitis two months prior to admission was admitted for multifocal pneumonia. During his initial evaluation, the patient was found to have generalized jaundice, and laboratory evaluation revealed elevated transaminases with a hepatocellular pattern. He was initially thought to have reactivation of the hepatitis A, however non-improvement with conservative measures led to further serological testing, which revealed positive anti-smooth muscle antibody. Autoimmune hepatitis was confirmed by biopsy, and patient was started or oral steroids. The patient's clinical status and liver function improved significantly, and he was discharged with outpatient follow-up.

DISCUSSION:

It is hypothesized that environmental agents trigger T-cell–mediated events directed at liver antigens in a host genetically predisposed to AIH, leading to a progressive necro-inflammatory and fibrotic process in the liver. Studies have shown hepatotropic viruses including HAV to be associated with AIH. Defects in suppressor-inducer T lymphocytes specifically controlling immune responses to the asialoglycoprotein receptor may be the cause of susceptibility. It has also been shown that humoral immune responses to liver cell surface antigens are frequently provoked by hepatitis A and B viruses. Newer research is also exploring molecular mimicry between the infectious particle and the liver constituent as the mechanism, although specific data has not been discovered on HAV. In the setting of the recent outbreaks of HAV, such pathology may become more commonplace. It is important for a clinician to distinguish between relapses of HAV (documented to be between 3-20% of patients in the first 6 months) and onset of AIH, as treatment modalities and prognosis are vastly different. A high level of awareness of this pathology allows for its prompt recognition and the potential for successful treatment.
2489 A Rare Case of Isolated Symptomatic Hepatic Sarcoidosis
Authors: Rashid Shazia , Khan Maria , Mavuram Meher S. , Arab Bassem , Hafiz Nazar , Samant Hrishikesh , Morris James D.
Published: October 2019

INTRODUCTION:

Sarcoidosis is an inflammatory granulomatous disease that has multi- organ involvement with varied course of disease expression and progression. Liver involvement is seen in only a hand full of cases, especially with florid symptoms. Here, we present a rare case of symptomatic isolated hepatic sarcoidosis mimicking malignancy, that was promptly diagnosed.

CASE DESCRIPTION/METHODS:

A 60- year old African American female, who is otherwise healthy, presented to our tertiary care center with a month-long history of worsening epigastric pain, nausea, vomiting, malaise, weight loss and night sweats. Exam was remarkable for hepatomegaly and labs significant for elevated ALP and GGT, mild transaminitis and elevated CA 19-9 levels. CT scan revealed hypodensities and perfusion defects throughout the liver, hepatosplenomegaly and hilar lymphadenopathy, which had been confirmed by MRI and Triple-phase CT of the liver. With the suspicion for malignancy, liver biopsy was pursued and showed epithelioid-cell granulomas with focal necrosis and multiple asteroid bodies, pathognomonic for sarcoidosis. Subsequently, ACE levels were found to be elevated. Patient was then started on prednisone and immunosuppressants with relief of symptoms. A repeat CT scan 6-months later, showed resolution of liver lesions and improvement in liver chemistries.

DISCUSSION:

Sarcoidosis is a systemic granulomatous disease of unknown etiology. Per ACCESS study, liver involvement of sarcoidosis was described in only 11.5% of 736 patients, more commonly seen in African American population than Caucasian, with only 10% of them were reported to have transaminitis. Hepatic involvement appears to be clinically silent with most patients presenting with constitutional symptoms, even then liver lesions on CT are described in only 5% of the patients. Steroid therapy and immunomodulators are used to help with symptom control, however their effect on the natural progression of the disease is not established. Our patient presentation with significant symptoms and imaging findings, prompted us to think beyond the obvious. Sarcoidosis is a diagnosis of exclusion; hence it is essential for clinicians to have a high degree of suspicion for hepatic involvement.
2493 Large Perisplenic Hematoma and Spontaneous Hemoperitoneum as the Initial Presentation of Undiagnosed Burkitt's Lymphoma
Authors: Ilyas Fariha , Shrestha Prabhat , Lopez Christan , Qavi Ahmed H. , John Geevarghese
Published: October 2019

INTRODUCTION:

Spontaneous hemoperitoneum (SH) is a rare but life-threatening condition of non-traumatic etiology. It usually arises from spleen, hepatic, gynecologic and vascular sources.

CASE DESCRIPTION/METHODS:

A 61-year-old male with no known medical history presented with acute left sided, non-radiating abdominal pain. On examination, he was hemodynamically stable and had a soft, non-tender abdomen with splenomegaly. His laboratory workup was significant for pancytopenia. CT of abdomen showed a 9 cm perisplenic hematoma with moderate ascites. Patient was admitted to ICU for closer monitoring and a follow-up contrast enhanced CT of abdomen confirmed a perisplenic hematoma and revealed splenomegaly measuring 20 cm with the presence of hemoperitoneum. Sclerotic lesions in multiple thoracolumbar vertebrae, sacrum, and right iliac bone, suspicious for metastasis were also found. Follow-up chest CT revealed mildly enlarged mediastinal lymph nodes. His bone scan was negative. Further malignancy workup was remarkable for a low haptoglobin and high LDH levels. Flow cytometry was positive for CD4, CD5, CD10 and CD19 consistent with B cell lymphoma. Chromosomal analysis confirmed a translocation t(8;14) and FISH analysis showed fusion of MYC and IGH genes in 15.5% of cells, consistent with BL. The patient was managed conservatively.

DISCUSSION:

The exact mechanism of non-traumatic splenic hematoma leading to hemoperitoneum is unclear. One theory is an increased intra-splenic pressure due to endothelial hyperplasia and vascular occlusion due to a hypercoaguble state leading to spontaneous bleeding from leaking capillaries. Diagnosis is unsuspected and mainly made by CT as seen in our patient whose only findings included abdominal pain and pancytopenia. There have been very few reported cases in literature of SH and even fewer in patients with diagnosed BL. All such cases involved a ruptured spleen. Our patient is unique and to the best of our knowledge, the first ever reported case of SH presenting as an initial feature of a previously undiagnosed BL. Furthermore, his hemoperitoneum was secondary to a large perisplenic hematoma with an intact but enlarged spleen. Therefore, it is important for clinicians to keep lymphoma as a differential in patients presenting with acute abdominal pain and pancytopenia in order to effectively diagnose and treat an underlying malignancy and prevent future complications.
2495 Spontaneous Fungal Peritonitis Secondary to  Candida  glabrata in a Patient With Cardiac Cirrhosis
Authors: Yang Tsujung , Pachpande Vrushali , Ghimire Subash , Shah Harshil , Walsh James
Published: October 2019

INTRODUCTION:

Spontaneous fungal peritonitis (SFP) is an uncommon, but serious complication of liver cirrhosis. In one study, the one-month mortality rate was 73.3% as compared to 28% in SBP alone. The mortality remains high despite appropriate antifungal therapy partly due to delayed diagnosis.

CASE DESCRIPTION/METHODS:

We present a case of a 67 years-old male patient who presented with acute confusion and worsening abdominal distension for unknown duration. He has history of heart failure with reduced ejection fraction secondary to ischemic cardiomyopathy and liver cirrhosis presumed secondary to heart failure. He was found to have acute hepatic encephalopathy, anasarca, acute kidney injury, and septic shock. Abdominal exam showed tender, distended abdomen with positive fluid thrill. Spontaneous bacterial peritonitis (SBP) was suspected and ascitic fluid analysis was sent. It reported nucleated cells 3766/uL, 80% neutrophils, SAAG 1.7, glucose <20 mg/dl, and total protein 3.2 g/dl. Computed tomography of abdomen and pelvis without contrast showed massive ascites, but there was no evidence of bowel perforation (Figure 1). He was treated with Vancomycin and Zosyn for SBP and lactulose for hepatic encephalopathy. On day 3, gram stain of ascitic fluid reported budding yeast, and he was started on anidulafungin. The final culture reported Candida glabrata. Bacterial and fungal blood cultures were negative. Despite treatment, his encephalopathy persisted, and he had rapid accumulating ascitic fluid. Ascitic fluid culture was repeated which reported the same. A repeat CT abdomen showed perihepatic hematoma and free intraperitoneal air (Figure 2). He underwent exploratory laparotomy that revealed duodenal perforation. Despite surgical repair and antifungal, septic shock progressed, and patient died from multi-organ failure. Candida glabrata was sensitive to anidulafungin on retrospective review of sensitivity report.

DISCUSSION:

Although initial ascitic fluid analysis suggested secondary peritonitis, CT scan was negative for perforation or loculated infection. Our case highlights the high mortality rate of Candida peritonitis despite appropriate treatment. It is likely secondary to delayed antifungal and undiagnosed bowel perforations.
2498 ECMO: A Bridge to Liver Transplant
Authors: Kumar Anila , Gollapudi Lakshmi Asritha , Nishida Seigo , Bodin Roxana
Published: October 2019

INTRODUCTION:

ECMO has been rarely used in the perioperative setting during liver transplantation (LT) and there is scarce data on its use. Many patients requiring LT have cardiopulmonary compromise due to complications of liver disease or other co-morbidities. We present a use of VA-ECMO as cardiopulmonary support during LT in a patient with pulmonary hypertension.

CASE DESCRIPTION/METHODS:

A 57-year-old man with a medical history of ETOH cirrhosis complicated by recurrent ascites, portal hypertension, and hypothyroidism was admitted for management of fluid overload despite compliance with low-salt diet and diuretics. He had multiple prior hospitalizations for fluid overload which were managed with intravenous diuretics. He was listed for liver and kidney transplant in New York. He was eventually started on hemodialysis for end stage kidney failure secondary to hepatorenal syndrome. He also had severe pulmonary hypertension (PH). He had an offer for liver transplant and went to the operating room (OR). However, a Swan-Ganz catheter was placed and showed mean PA pressure of 47 mm Hg with high cardiac output despite the use of vasodilators. After careful evaluation, his transplantation was aborted. Few weeks later, he was taken back to the OR for liver transplant and was placed on VA-ECMO electively. His cardiopulmonary status was carefully monitored and remained stable with VA-ECMO support during the procedure even with severe PH. His transplantation was successful, after which his hemodynamics improved significantly and the ECMO was removed in the OR. Subsequently, he underwent kidney transplant and his PH improved postoperatively.

DISCUSSION:

Severe cardiac and pulmonary dysfunction is a contraindication for LT. Hepatopulmonary syndrome is a complication of liver failure and can cause pulmonary hypertension, which was the case with our patient. Intraoperative use of ECMO, both VA and VV, have been used sparingly. However, over the recent years, the use of ECMO peri-operatively has had wider use although poor outcomes are still reported. Bleeding risk is extremely high due to the coagulopathic nature of liver disease as well as the anti-coagulation necessity during ECMO so it is imperative to limit the duration of ECMO. In conclusion, VV or VA- ECMO can be a useful therapeutic option in patients undergoing transplantation with significant cardiac or pulmonary compromise especially in New York where the waiting period for transplant is long.