bluuebunny/crossref_metadata_2025_split huggingface dataset:
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.
2501 A Rare Case of Sofosbuvir/Velpatasvir/Voxilaprevir Failure in a Patient With Hepatitis C Virus Infection and Previously Undiagnosed Hepatocellular Carcinoma
Authors: Agostini Tandy , Vangimalla Shiva , Lalos Alexander 
Published: October 2019

INTRODUCTION:
The advent of direct acting antiviral (DAA) agents has revolutionized the treatment for chronic hepatitis C virus (HCV) infection with greater than 95% success rate. In 2017, a combination of sofosbuvir, velpatasvir, and voxilaprevir (sof-vel-vox) was approved after clinical trials demonstrated a 97-100% sustained virological response (SVR) rate in patients who had failed a previous NS5A inhibitor regimen. We present a rare case of treatment failure with sof-vel-vox in a non-cirrhotic patient with previously undiagnosed hepatocellular carcinoma (HCC).

CASE DESCRIPTION/METHODS:
The patient is a 68-year-old African-American male with a history of HCV-G1b infection who had previously failed treatment with a 12-week regimen of sofosbuvir/ledipasvir. He was subsequently retreated with 12 weeks of sof-vel-vox. He had an end of treatment response but had a viral relapse at 3 months post-treatment. He denied hepatitis exposure in the interim and repeat genotyping was G1b. He was compliant with his medications. He was referred to Hepatology for salvage treatment options. Prior labs and images did not show evidence of cirrhosis and he did not have a history of hepatic decompensations. Combined HIV Ab/Ag and Hepatitis B Core Ab were negative. A Fibrosure test revealed F3 fibrosis but unfortunately, he did not undergo HCC surveillance. At our initial evaluation a contrast MRI was ordered and revealed a 4.6 cm right lobe enhancing lesion with delayed washout consistent with HCC. Resection was not possible due to the location of the lesion, so he underwent locoregional therapy and is currently listed for liver transplantation with MELD exception.

DISCUSSION:
Treatment failure with sof-vel-vox is a rare event. Underlying HCC is known to be associated with lower SVR rates in patients treated with DAAs. Some have surmised a protection from eradication due to immunologic changes in cancer patients. We hypothesize that the presence of HCC was the most likely factor for this treatment failure. With sof-vel-vox as the last available option in the treatment paradigm, failure of this regimen in previous NS5A treated patients leads to a situation where there is not an available proven treatment regimen for salvage therapy. As such, we suggest that patients being considered for sof-vel-vox have “up to date” advanced imaging of the liver even in the absence of documented cirrhosis. If treatment failure does occur, one should consider the possibility of an underlying diagnosis of HCC.
2510 Bacteremia With Liver Abscess and Endophthalmitis: A Rare Case of Invasive Klebsiella Pneumoniae Syndrome
Authors: Tageldin Omar , Parsa Nour , Copare Jennifer , Mansoor Muhammad Sohail , Alexandre Cassidy , Richter Seth 
Published: October 2019

INTRODUCTION:
Pyogenic liver abscesses are often polymicrobial with Escherichia coli being the most common isolated organism. Klebsiella pneumoniae is a rare cause of pyogenic liver abscesses and often affects patients with immunodeficient states. Here we discuss a rare case of primary Klebsiella pneumoniae liver abscess with subsequent metastatic infection leading to vision loss.

CASE DESCRIPTION/METHODS:
A 68-year-old African American woman presented with complaints of intermittent fever, chills, right upper quadrant abdominal pain and blurry vision of the left eye. Her past medical history was notable for recent laparoscopic hernia repair, cataract surgery, and hypertension. The abdominal exam showed moderate tenderness of the right upper quadrant; ophthalmic exam of the left eye revealed afferent pupillary defect and decreased visual acuity. Labs were notable for leukocytosis, total bilirubin of 2.7, AST of 161, ALT of 135 and an alkaline phosphatase of 110. CT abdominal imaging revealed an abscess within the dome of the right liver measuring 5.2 × 6.3 × 4.1 cm. She was admitted to the intensive care unit and started on broad-spectrum empiric antibiotic therapy. Blood culture revealed gram-negative rods. B-scan ultrasonography of the left eye showed vitritis with loculation confirming the diagnosis of endophthalmitis. Intravitreal antibiotics were promptly administrated. She underwent CT guided sampling and drainage of the hepatic abscess with fluid culture growing the same organism identified in the blood cultures: mucoid Klebsiella pneumoniae complex. The patient was diagnosed with invasive Klebsiella bacteremia, liver abscess, and endophthalmitis and developed permanent vision loss in the left eye despite prompt intravenous and intravitreal antibiotic therapy.

DISCUSSION:
Klebsiella pneumoniae invasive liver abscess syndrome is a rare condition in the United States making timely diagnosis and treatment a challenge per se. Known risk factors for invasive liver abscess syndrome include diabetes mellitus, hepatobiliary disease, alcoholism, chronic renal failure, and cancer. While our patient did not have any of these specific comorbidities and had no recent international travel, her age, and recent surgical interventions likely predisposed her to an immunocompromised state. Invasive liver abscess syndrome can be fatal with poor prognosis if treatment is delayed. Timely abscess drainage and administration of intravenous antibiotics is crucial and should be implemented early on in the clinical course.
2516 Use of Ustekinumab in Pediatric Crohn's Disease: A Report of Three Cases
Authors: Sarles Harry E. , Whitney Annette E. , Van Anglen Lucinda J. , Kuten Samantha A. , Hardin Thomas C. 
Published: October 2019

INTRODUCTION:
Treatment options for pediatric IBD are limited. Use of non-FDA approved agents to manage difficult pediatric IBD patients (pts) may be considered. Ustekinumab (UST) is a humanized IgG1κ monoclonal antibody designed to block interleukin-12 and interleukin-23, suppressing cytokine activation and inflammatory response. While UST is approved for the treatment of moderate-to-severe Crohn's disease (CD) in adults, there are little safety and efficacy data in pediatric CD pts. We describe our experience with UST in pediatric CD.

CASE DESCRIPTION/METHODS:
Pt 1 is an 18 y/o female, diagnosed (dx) with CD in 2015. She was stable on infliximab (IFX) and methotrexate (MTX) but presented in Nov 2016 with worsening scalp psoriasis thought attributable to IFX. IFX was D/Ced and UST initiated in Dec 2016 (260 IV mg, then 90 mg q8wks) with concomitant MTX 15 mg weekly. By Feb 2017, CD was quiescent and psoriasis improving. She presented in Nov 2017 with abdominal cramping and loose stools after running out of MTX. CRP was 8.8 mg/L on UST alone; psoriasis had resolved. MTX was restarted at this time. Pt continued to do well until early Feb 2019 when she had a CD flare associated with a delay in UST dose. She responded to a prednisone taper and restarted therapy. UST serum level in Mar 2019 was 2.5 mg/L and the dose was increased to 90 mg q4wks. The patient continues to do well. Length of therapy (LOT) to date is 2.5 yrs. Pt 2 is an 18 y/o male, dx with CD in 2017. He has intermittently unstable CD, treated with various combinations of adalimumab (ADA), MTX, and prednisone in the past. In Aug 2018 ADA was D/Ced, and UST initiated (390 IV mg, then 90 mg IV q8wks). His CD responded well to UST with a reduction in CRP from 18 to 1.6 mg/L. In late Dec 2018 he reported frequent bloody stools but was otherwise stable. Colonoscopy and upper GI endoscopy in early Jan 2019 were unremarkable; hematochezia resolved with the addition of mesalamine. LOT to date is 10 mos. Pt 3 is a 15 y/o female, dx with CD in 2013. She was stable on ADA until Aug 2018 when she presented with increased symptoms. ADA was D/Ced and UST initiated in Sep 2018 (390 mg IV, then 90 mg IV q8wks). Significant improvement was noted (CRP 1.3 mg/L) in Dec 2018. LOT to date is 10 mos.

DISCUSSION:
Clinical improvement in CD was observed in these pts following conversion to UST, with no significant safety concerns observed. Further studies are warranted to determine the place in therapy for UST in unstable pediatric CD pts.