bluuebunny/crossref_metadata_2025_split huggingface dataset:
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.