bluuebunny/crossref_metadata_2025 huggingface dataset:
2455 Nivolumab-Induced Immune Cholestatic Hepatitis in a Liver Transplant Recipient
Authors: Anugwom Chimaobi , Wongjarupong Nicha , Leventhal Thomas M.
Published: October 2019

INTRODUCTION:

Cancer treatment has taken giant strides in recent years with the advent of immunotherapy including check-point inhibitors. We present a case of cholestatic hepatitis following treatment of recurrent HCC with Nivolumab.

CASE DESCRIPTION/METHODS:

A 62-year-old male with a history of alcoholic cirrhosis and hepatocellular carcinoma (HCC), was seen 5 years after liver transplantation. Approximately one year after transplant, he developed metastatic HCC in his lung and anterior abdominal wall with progression of tumor burden after several chemotherapy regimens. Two months prior to admission, he began palliative Nivolumab. He developed elevated serum alkaline phosphatase (ALP), aspartate transaminase (AST), alanine transaminase (ALT), and total bilirubin; so, he was hospitalized. Admission labs showed ALP of 813, ALT of 1265, AST of 696 and bilirubin of 8.3. Investigations were negative for viral or autoimmune hepatitis. Acute cellular rejection was unlikely given time from transplant and stable tacrolimus trough levels. Given the concern for Nivolumab-induced liver injury, high dose intravenous steroids at 2 mg/kg daily was started with temporary improvement in his liver tests. A trans-jugular liver biopsy confirmed the diagnosis of immune-induced liver injury due to Nivolumab. By hospital day 7, his liver tests continued to rise precipitously (Figure 1). Ultimately, he developed massive hematemesis and hemodynamic instability, necessitating intubation and intensive care unit (ICU) admission. An emergent upper endoscopy showed severe esophagitis with denudation of the esophageal mucosa (Figure 2) and gastric blood and clot precluding completion of the endoscopy. Steroids were increased to 4 mg/kg, for worsening hepatitis. However, his family decided to pursue comfort cares considering his rapid decline and metastatic cancer. He expired within minutes of implementing comfort care measures.

DISCUSSION:

We believe that Nivolumab caused a cholestatic liver injury in our patient. Nivolumab-induced liver injury has been previously reported but with paucity of data in transplants recipients or cholestatic pattern of injury. The difference in clinical, biochemical and histopathologic features may suggest subtle differences in strategies for management of Nivolumab-induced liver injury. Further prospective studies are needed to understand the pathophysiology and management of this type of liver injury as well as the benefits of PD-1 inhibitors in liver transplant recipients.
2456 Fibrosing Cholestatic Hepatitis Following Transplantation of a Hepatits C Virus Positive Donor Liver
Authors: Abdulhamid Ahmed , Jafri Syed-Mohammed
Published: October 2019

INTRODUCTION:

Fibrosing cholestatic hepatitis (FSC) is a rare disorder, typically seen in immunosuppressed patients with a history of chronic hepatitis B and C infections. We present a case of a 67-year-old recipient of a hepatitis C virus positive donor liver with subsequent development of FSC.

CASE DESCRIPTION/METHODS:

We present a case of a 67 year-old gentleman, with a past medical history of HCV cirrhosis and multifocal hepatocellular carcinoma (HCC), who underwent orthotopic liver transplantation (OLT). Prior to transplantation, the patient was treated for HCV with Vosevi/ribavirin with sustained viral response. His course was complicated by multifocal HCC, for which he received treatments with DEB-TACE and Y90. He subsequently underwent OLT with a HCV positive (RNA quantitative positive) donor liver. The patient's post-operative course was complicated by marked elevation in AST and ALT (1610 and 647 respectively) and HCV RNA quantitative (1.5 million), concerning for fibrosing cholestatic hepatitis. A liver biopsy was performed and showed both mild acute cellular rejection (ACR) and cholestasis. The patient was treated with Epclusa for HCV and immunosuppression (prednisone, tacrolimus) for ACR with improvement in his liver enzymes.

DISCUSSION:

Fibrosing cholestatic hepatitis (FSC) is a rare disorder, typically seen in immunosuppressed patients with a history of chronic hepatitis B and C infections. It is particularly seen in patients following transplantation, namely recipients of donor liver and kidneys. Patients with FSC present with a number of symptoms including malaise, fatigue, jaundice, abdominal pain and progressive liver failure, which may occur over a period of weeks to months. The pathophysiology is related to direct cytotoxic effect of viral antigens on hepatocytes in the setting of immunosuppression. High viral titers and elevated liver enzymes in an immunosuppressed patient should raise concern for FSC. The diagnosis is established with a liver biopsy, which typically reveals hepatocyte ballooning, bridging fibrosis, pericellular and sinusoidal fibrosis as well as intracellular and canalicular cholestasis. This ultimately leads to acute liver failure and may result in rapid graft loss. Given the high rates of mortality associated with FSC, prompt diagnosis and treatment with direct-acting antiviral regimens may be effective in treatment, even in severe cases.
2464 A Case of Acute Icteric Hepatitis Caused by Epstein Barr Virus in an Immunocompetent Adult
Authors: Sieloff Martin , Tariq Tooba , Nguyen Minh
Published: October 2019

INTRODUCTION:

Epstein Barr Virus (EBV) is a viral infection that can often remain asymptomatic but is also a major cause of infectious mononucleosis. Patients often present with sore throat, fever, lymphadenopathy and can develop splenomegaly. EBV-associated hepatitis is uncommon and cases with clinical jaundice are rare.

CASE DESCRIPTION/METHODS:

A 23-year old male without significant past medical history presented with one week of flu-like symptoms and acute onset of a generalized pruritic rash, right upper quadrant (RUQ) abdominal pain and dizziness. He denied any recent alcohol consumption, IV drug use or new medication initiation. On arrival to the emergency department the patient was afebrile with a blood pressure 130/84. Physical exam revealed scleral icterus and cutaneous jaundice, a diffuse macular rash was across his face, chest and arms and he had tender hepatomegaly. He was alert and oriented without confusion. Laboratory examination revealed a leukocytosis of 11.8, a total bilirubin of 15.8, direct bilirubin of 13.3, an INR of 1.2, ammonia of 26, an alkaline phosphatase of 499, and an ALT of 615 with AST of 405 signifying acute hepatitis. A urine drug screen was negative. A RUQ ultrasound with doppler showed no evidence of Budd-Chiari or portal vein thrombus; fatty infiltration of the liver was seen with no evidence of cholelithiasis or common bile duct obstruction. Hepatitis A, B and C viral screen was negative, acetaminophen level was within normal limits, HIV antibody test was negative, herpes virus IgM negative, CMV IgM negative, anti-smooth muscle antibody negative for autoimmune hepatitis. Ceruloplasmin and alpha-1 antitrypsin levels were not suggestive of Wilson's disease or alpha-1 antitrypsin deficiency. A peripheral smear showed reactive lymphocytosis. There was significant elevation in EBV IgM and heterophile antibody suggesting systemic Epstein Barr viral infection as the cause of acute hepatitis. Over the next 3 days, his liver enzymes began trending down slowly with symptomatic management and he was determined stable for discharge with plans for 2 and 4 week follow ups to monitor for resolving acute EBV hepatitis.

DISCUSSION:

It is important to remember that, although rare, EBV can cause hepatitis with diffuse jaundice and significant hyperbilirubinemia along with AST and ALT levels over 10 times the upper limit of normal. Such presentation could easily be misconstrued as an alternative diagnosis and patient safety compromised with unnecessary treatment.
2467 Recognizing Macrobid as a Potential Cause for Major Liver Injury
Authors: Khan Faizan , Davis Michael , Khan Nasir
Published: October 2019

INTRODUCTION:

Nitrofurantoin is one of the most commonly prescribed antibiotics for urinary tract infections. It is also one of the most common medications known to cause drug-induced liver injury (DILI). Nitrofurantoin can cause acute or chronic liver injury. Acute liver injury occurs within a few weeks of nitrofurantoin use and resolves quickly after discontinuation. Chronic liver injury typically presents months or years after use of nitrofurantoin and can also present as an autoimmune type reaction. This case demonstrates the need to have high suspicion for DILI when patients present with elevated liver enzymes with recent use of nitrofurantoin.

CASE DESCRIPTION/METHODS:

The patient is a 56 year old female with a past medical history of recurrent urinary tract infections including ESBL who presented with abdominal pain, diarrhea, and nausea for one month. Prior to symptoms onset, she had developed burning and odor in her urine and was treated with nitrofurantoin for 14 days. Urinary symptoms resolved after antibiotic completion. Two weeks later, the patient began to develop worsening abdominal pain that was accompanied by diarrhea and subjective fevers and chills. On presentation, the patient's vitals were found to be: 98.5F, HR 107, 156/74, and saturating well on room air. On labs, she was found to have elevated alkaline phosphatase of 200 U/L, ALT 1014 U/L, AST 2011 U/L, Total Bilirubin 1.0 mg/dL, Lipase 59 U/L, negative alcohol level, negative acetaminophen level, and negative viral hepatitis panel. CT Abdomen and Pelvis showed hepatic steatosis but no acute processes. Secondary workup for liver injury was largely negative except an ANA titer of 1:320. The patient was treated supportively and had a gradual decrease in her liver enzymes over the course of three days.

DISCUSSION:

DILI can be a difficult diagnosis to make because it is a diagnosis of exclusion. In our case, the patient experienced marked elevations in her liver enzymes that resolved with only supportive care. Her workup was negative and the only identifiable etiology of her acute liver injury was her nitrofurantoin use. Additionally, our patient was seen to have features of drug-induced autoimmune hepatitis with her ANA elevation suggesting a component of chronic liver injury. She had been treated with Nitrofurantoin in past infections as well. Since Nitrofurantoin can cause sometimes severe liver damage, the indication for its use should be evidence-based, and signs/symptoms of liver injury should lead to prompt cessation of therapy.
2469 Extracapsular Hepatocellular Adenoma: A Diagnostic Dilemma
Authors: Comba Isin Y. , Henriquez Richard , Kumar Sundeep , Srinivasamurthy Ruthvik , Wallis-Crespo Maria , Karasik Olga , Bhatia Lakhinder
Published: October 2019

INTRODUCTION:

Hepatocellular adenoma (HCA) is a benign tumor of the liver and almost always located intracapsular. Bleeding (25%), and malignant transformation (5%) can complicate the disease course if left untreated. Herein, we present a case of extracapsular HCA of ectopic liver in the omentum complicated with intraperitoneal hemorrhage.

CASE DESCRIPTION/METHODS:

A 43-year-old lady with no past medical history presented to emergency department with two-day history of left shoulder pain and epigastric discomfort. Her only medication was an oral contraceptive pill (OCP). On physical exam, the patient had persistent sinus tachycardia, and epigastric tenderness. Initial labs were unremarkable except for mildly elevated AST at 58 IU/L (10-37). CT of the abdomen with IV contrast showed a heterogeneously enhancing mass likely arising from the anterior wall of the stomach. T2-weighted MRI abdomen demonstrated a hypoenhancing mass measuring 4 × 7 × 8 cm with adjacent hemorrhage (Figure 1). Exploratory laparotomy revealed an infarcted, hemorrhagic mass between the fundus of the stomach, spleen, and diaphragm with a large amount of clots and free blood occupying the greater curvature. The mass was separated from surrounding viscera however, slightly attached to the diaphragm. The mass was removed with the completely resolved tachycardia and epigastric pain postoperatively. Initially, the lesion was thought to be an accessory spleen based on location and gross examination. Microscopically, an infarcted, hemorrhagic mass composed of cords of degenerated epithelioid cells with vacuolated cytoplasm was surrounded by reactive fibro- and myofibroblastic tissue. Epithelioid cells were diffusely stained for HerPar-1 (hepatocyte origin), and CK AE1/AE3 (Figure 2). These findings were consistent with HCA.

DISCUSSION:

HCA is an overlooked diagnosis and can result in serious complications if unrecognized. Among the well-known risk factors of HCA are OCPs or anabolic steroids, pregnancy, and steatohepatitis. The prevalence of these lesions has been rising parallel to increasing use of OCP and abdominal imaging. In females, lesions <5 cm with no associated symptoms or complications can be managed conservatively by terminating OCP use, and surveillance. Our patient presented with acute left shoulder pain which was likely due to phrenic nerve irritation by bleeding (Kehr's sign). To best of our knowledge, there are only three cases of extracapsular HCA reported in the English literature.
2473 The Mystery of the “Vanishing” Bile Ducts
Authors: Shah Apurva , Leung Galen , Sun Edward , Mockler Daniel
Published: October 2019

INTRODUCTION:

The etiologies of cholestatic liver disease is extensive, including immunological, ischemic, infectious, metabolic, and toxic causes. We present a rare case of a patient who was diagnosed with idiopathic vanishing bile duct syndrome (VBDS) after a thorough work-up.

CASE DESCRIPTION/METHODS:

A 61-year-old female with a history of hypertension, type II diabetes mellitus, congestive heart failure, and end stage renal disease presented with generalized pruritus and jaundice. Laboratory tests revealed a total bilirubin 10.5 mg/dL, AST 27 IU/L, ALT 27 IU/L, alkaline phosphatase 596 IU/L. Further workup was negative for viral hepatitis, autoimmune hepatitis, hemochromatosis, alpha-1 antitrypsin deficiency, and Wilson's Disease. An MRCP showed hepatosplenomegaly without ductal dilatation, and upper endoscopy with ultrasound revealed a normal biliary duct without evidence of obstruction. Pathology from fine needle biopsy was significant for chronic cholestatic changes with ductopenia (43% duct loss), obliterative portal venopathy, and congestive hepatopathy. Without an identifiable cause, these findings are most consistent with idiopathic VBDS. The patient was referred for liver transplant evaluation due to concern for progression to cirrhosis.

DISCUSSION:

VBDS is a rare hepatic condition characterized by progressive loss of the interlobular and septal bile ducts resulting in cholestasis and potentially biliary cirrhosis and liver failure. The cause of bile duct destruction is poorly understood but likely results from biliary apoptosis due to immunologic, ischemic, infectious, metabolic, and toxic assaults.
1
Approximately 1% of cases classify as idiopathic VBDS, which likely comprises of several disease processes with atypical features, such as late-onset non-syndromic paucity of intrahepatic bile ducts, small-duct primary sclerosing cholangitis without large duct involvement, non-suppurative viral cholangitis, and autoimmune cholangitis without typical autoantibodies.
2
The disease course is unpredictable, ranging from benign to fatal or necessitating transplantation.2 Treatment of VBDS includes drug withdrawal, choleretic agents, and immunosuppression. The uncertain etiology of idiopathic VBDS precludes a causative treatment. Liver transplantation has been the only successful treatment.
2
2476 Fibrinogen-Positive Ground Glass Hepatocytes in a Patient With Ulcerative Colitis
Authors: Koprowski Steven T. , Ando Yumi , Jou Janice
Published: October 2019

INTRODUCTION:

Ground-glass hepatocyte cytoplasmic inclusions (eosinophilic, glassy cytoplasmic changes) carry a unique differential diagnosis with significant diagnostic implications. This cytoplasmic change is thought to occur from accumulation of metabolic products including glycogen, fibrinogen, or viral proteins. Few cases of patients with ground-glass, fibrinogen-positive inclusions have been observed and are thought to occur from acute states of metabolic stress.

CASE DESCRIPTION/METHODS:

A 64-year-old male with history of poorly controlled ulcerative colitis presented with diffuse abdominal pain, weight loss, and two months of frequent bloody bowel movements. On arrival, he was afebrile without abnormal vital signs. His appearance was non-toxic but malnourished. Abdominal exam produced diffuse tenderness but no evidence of peritonitis. Labs were notable for AST 233 U/L, ALT 486 U/L, and Alk phos 140 U/L (normal 56-119 U/L). He failed trial of medical therapy and required total abdominal colectomy. His aminotransferase levels during this time continued to worsen without explanation (peak AST 710 U/L and ALT 1145 U/L). Serum infectious disease workup including Hepatitis C antibody, HIV Antibody/Antigen screen, Hepatitis B core antibody and surface antigen, Hepatitis E IgM antibody, HSV-1 and 2 PCR, and Varicella-zoster PCR were all negative. Serum IgG, IgA, TTG-IgA, anti-smooth muscle antibody and anti-nuclear antibody testing were unremarkable. Abdominal ultrasound with doppler showed patency of the portal, splenic, and hepatic veins without thrombus. Liver biopsy showed moderately active steatohepatitis, and hepatocytes displayed ground-glass cytoplasmic appearance. PAS staining showed weak to no staining of ground-glass regions. Due to the lack of positive PAS staining, fibrinogen staining of the liver specimen was obtained, which showed diffuse positivity within hepatocytes.

DISCUSSION:

A rarely reported finding, fibrinogen accumulation in hepatocytes can occur from inherited disease and from acquired errors in endoplasmic reticulum processing in a setting of metabolic stress. Ground glass hepatocyte inclusions staining positive with fibrinogen have only been associated with the acquired causes in humans. Only 3 cases, to our knowledge, have shown similar findings. After 1 year follow-up from total colectomy, his aminotransferases had returned to normal range.
2490 A Rare Initial Presentation of HCC
Authors: Rivera Arturo Suplee , Katkoria Gopal , Castano Juan J. , Rapoport Grigoriy , Zamir Asif
Published: October 2019

INTRODUCTION:

Metastasis accounts for approximately 1-4% of all oral malignancies. Other cancers that spread to the mandible include lung, breast, kidney and colon. Hepatocellular carcinoma (HCC) has the fifth highest prevalence worldwide but only 68 cases of mandibular metastasis have been documented between 1957 to 2011. The following is a rare case of HCC presenting as mandibular metastasis.

CASE DESCRIPTION/METHODS:

The patient is a 73-year old Hispanic male with history of diabetes mellitus type 2, hypothyroidism, mild dementia, and alcoholic liver cirrhosis who presented with a one-month history of progressively enlarging painless mass in the left mandible, anemia and a 9-month history of weight loss. Additionally, one month ago the patient suffered a traumatic fall. Physical exam was significant for the presence of a malodorous, bleeding oral mass and a left humeral fracture. Initial CT imaging revealed a well-defined, expansile lytic mass in the left mandible, with an additional mass in the right mandible and the right paraspinal area of C1. Additional imaging revealed multiple hepatic lesions, a left humeral mass associated with the fracture and multiple lytic spinal lesions. Alfa-fetoprotein was significantly elevated. Biopsy of the mandibular mass revealed HCC with positive staining of hep par 1 glypican-3 and focal villin. After discussion, the patient and family decided to pursue conservative management with hospice care.

DISCUSSION:

Mandibular metastasis has been reported during the late stages of HCC, however mandibular involvement as an initial presenting sign is extremely uncommon. Therefore primary liver malignancy should always be considered in patients who present with mandibular tumors with HCC risk factors.
2491 The Alternative Outcome of Drug Induced Liver Injury
Authors: Bader Nimrah , Tardibono George
Published: October 2019

INTRODUCTION:

Drug Induced Liver Injury (DILI) is a common cause for hepatoxicity seen in the emergency room. In this setting inquiring about herbal and over the counter medications becomes important. Kratom is a plant derived psychoactive extract from the Mitragyna speciosa plant indigenous to Southeast Asia. It is used by chronic pain patients to avoid the withdrawal effects from narcotics. Although banned by the FDA, it is widely available for purchase on the internet. Several case reports have described its association with DILI. In cases where liver biopsies were obtained, kratom-toxicity was histologically characterized by acute cholestatic liver injury and canalicular cholestasis. Here we present a patient with features typical for DILI secondary to kratom-toxicity but was found to have an additional diagnosis.

CASE DESCRIPTION/METHODS:

A previously healthy 45-year-old woman consulted her primary care physician for jaundice. She was then referred to our hospital with elevated liver enzymes, nausea, pruritis and jaundice in the past 5 days. She reported she had been taking Kratom -an herbal medication- for back pain for the past 1 month. She had no prior history of liver disease. She was jaundiced, no stigmata of liver disease, and she had a benign abdominal exam. Based on this, a clinical diagnosis of acute liver injury secondary to drug injury was made. Pertinent labs results at the time of admission are summarized in Figure 1. Acetaminophen level and serologies for autoimmune hepatitis, viral hepatitis were non contributive. She was started on N-Acetyl Cysteine with subsequent improvement in her bilirubin, ALT and AST levels; ALP levels showed mild improvement. The patient's symptoms improved but minimal improvement in ALP was noted. A liver ultrasound was done which revealed mild dilation of the intrahepatic duct; the common bile duct was dilated at 1.1 cm, with hepatic steatosis. Further workup with MRCP revealed an ill-defined hypo enhancing soft tissue mass in the region of the pancreatic head. The patient's final diagnosis was pancreatic adenocarcinoma confirmed on pancreatic biopsy.

DISCUSSION:

This case demonstrates the value of imaging in cases of DILI. In this patient her timeline fit well with DILI; she was completely healthy before this acute illness and did not have symptoms suggestive of an underlying malignancy. It is likely that this patient may have delayed seeking care if not for the acute manifestations of her symptoms. She is currently undergoing chemotherapy.
2492 Everolimus-Induced Transaminitis in the Setting of Stage IV Carcinoid Tumor With Liver Metastasis
Authors: Nehme Christian , Ghazaleh Sami , Ghanim Marcel , Nesheiwat Zeid , Awad Mohammed , Khader Yasmin , Nawras Ali
Published: October 2019

INTRODUCTION:

Everolimus may lead to mild and asymptomatic liver enzymes elevation in up to a quarter of the patients taking it, but it rarely leads to symptomatic and significant transaminitis. We describe a case of everolimus induced transaminitis in the setting of stage IV carcinoid tumor with liver metastasis.

CASE DESCRIPTION/METHODS:

A 58-year-old female with a history of stage IV carcinoid tumor with liver metastasis presented with fatigue and worsening epigastric pain. She was first diagnosed with atypical bronchial carcinoids S/P left lower lobe lobectomy and lymph node dissection. Liver metastasis was diagnosed by biopsy two years later. She was started on everolimus one month earlier, and she reports sharp epigastric pain every time she takes the medication. On presentation, patient's vital signs were within normal limits. Physical exam revealed right upper quadrant and epigastric tenderness. Laboratory findings revealed elevated liver enzymes. AST was 723 U/L, ALT was 580 U/L, and alkaline phosphatase was 513 U/L. Total bilirubin was 1.2 mg/dl. To note, liver function tests were normal two weeks earlier. CT scan of the abdomen showed prominent intrahepatic biliary tracts and gallbladder wall thickening. Hepatitis panel and acetaminophen level were negative. Everolimus was discontinued on presentation. On the second day, bilirubin trended up to 1.6 mg/dl. Afterwards, patient's liver enzymes started to trend down and her bilirubin has normalized. Her abdominal pain has resolved. There was no indication at the time for endoscopic intervention.

DISCUSSION:

The trend of the liver enzymes is most consistent with a drug- induced liver injury. Patient was recently started on everolimus which was held on presentation. Liver enzymes started to trend down afterwards. AST and ALT were rapidly improving. Bilirubin became normal. It is important to consider everolimus as a cause of hepatocellular injury when other causes are ruled out.
2500 Pyogenic Liver Abscess Due to Klebsiella ozaenae: An Unlikely Culprit
Authors: Desai Parth M. , Gonzalez Elena , Vohra Ishaan , Mathew Madhu , Lingamaneni Prasanth , Mbachi Chimezie
Published: October 2019

INTRODUCTION:

Pyogenic liver abscess usually occurs in the setting of peritonitis or cholangitis where seeding may occur through the portal circulation or directly through the biliary tract. Gram negative enteric organisms such as E. coli and K. pneumoniae are most commonly implicated implicated organisms, with gram positives such a Strep species occurring usually in the setting of metastatic infection. Here we describe a case of a pyogenic liver abscess, where Klebsiella ozaenae, a rare pathogen, was isolated from the patient's blood cultures.

CASE DESCRIPTION/METHODS:

A 42 year old gentleman presented to the emergency department with 4 days of severe diffuse abdominal pain and fevers. He was seen 1 day prior in an outside hospital and was discharged from the emergency department. However, he was called the next morning and told that he was growing bacteria in his blood and was told to present to the nearest hospital. On presentation, vital signs revealed a fever of 103 degrees F, heart rate 109, blood pressure 120/70 mmHg. The patient was toxic appearing and had diffuse abdominal tenderness. Laboratory studies were notable for a white cell count of 14.2 k/uL. Serum chemistry and liver function tests were normal. Abdominal CT revealed a mildly lobulated low density lesion in the right hepatic lobe measuring 2.0 by 2.3 cm, with a smaller lesion measuring 0.7 cm and mild surrounding edema. The patient was started on ceftriaxone and metronidazole for coverage of pyogenic liver abscess. The larger lesion was successfully drained by the interventional radiology service, with a favorable clinical response. Two blood culture bottles from the outside hospital grew Klebsiella ozaenae. After initial improvement, patient was discharged home with 4 week course of oral ciprofloxacin and metronidazole and was stable upon follow-up.

DISCUSSION:

Klebsiella ozaenae is a rare cause of infection and bacteremia. It is thought to be a colonizer of the nasopharynx and and is typically associated with chronic atrophic rhinitis. To our knowledge, there has only been one previous case reported of K. ozaenae associated pyogenic liver abscess. Risk factors for bacteremia with K. ozaenae include elderly age, alcohol use, antibiotic exposure, immunosuppression, malignancy and rhinitis. Aminoglycosides, third generation cephalosporins and fluoroquinolones are considered to be drugs of choice for this organism. Clinicians should be aware of K. ozaenae and its potential to cause bacteremia and pyogenic liver abscess.
2506 A Fatal Case of Gemcitabine-Induced Acute Liver Failure
Authors: Hutton Anneka , Takkouche Sahar , Patino Sanja , Cespedes Carlos Miniet
Published: October 2019

INTRODUCTION:

Gemcitabine is a common chemotherapeutic agent with broad anti-tumor activity. Gemcitabine induced acute liver failure (ALF) is exceedingly rare, resulting in severe steatohepatosis and high mortality. We report the ninth case of Gemcitabine induced ALF.

CASE DESCRIPTION/METHODS:

A 65 year-old female on her second cycle of palliative Gemcitabine for metastatic breast cancer presented with five days of weakness, nausea and vomiting after chemotherapy. She was afebrile but hypotensive. She was not visibly jaundiced, sclerae anicteric, and without palpable hepatomegaly. Her initial labs were significant for elevated direct bilirubin, ALP and transaminitis suggestive of a cholestatic pattern (Figure 1). Notably, she had no hepatic dysfunction one month prior to presentation. Ultrasound revealed fatty infiltration of the liver without evidence of obstruction or acute cholecystitis. She developed worsening steatohepatitis with direct bilirubin predominance (Figure 2). CT abdomen revealed fatty replacement of the liver without obstruction or metastasis. Her course was complicated by severe thrombocytopenia, anemia, and renal failure. She deteriorated despite supportive care, becoming increasingly jaundiced and encephalopathic, expiring on hospital day 5. Table 1.

DISCUSSION:

Chemotherapy agent Gemcitabine is a deoxycitadine analog that incorporates into DNA resulting in apoptosis. Generally well-tolerated, common side effects include myelosuppression and transient elevations in LFTs. However, gemcitabine is implicated as the causative agent of a rare, severe and highly fatal cholestatic ALF. Gemcitabine induced ALF appears idiosyncratic, without clear correlation between dose or duration of treatment. In Gemcitabine induced ALF, imaging shows fatty infiltration of hepatic parenchyma and biopsy reveals hepatocyte ballooning and marked cholestasis. These cases are often rapidly fatal, complicated by thrombocytopenia, coagulopathy and renal failure. Our literature review revealed two cases of patient survival; in one case, Gemcitabine was promptly recognized as the offending agent and high dose steroid therapy was initiated. This may represent a treatment strategy where thus far no others exist. As drug induced hepatotoxicity is the most common cause of ALF in the USA, clinicians should be aware of this hepatotoxic potential, especially given the high mortality rate of Gemcitabine induced ALF.
2515  Rise of the Aminotransferases: A Liver's Call for Help!
Authors: Banerjee Namita , Shrimanker Isha , Singh Pratiksha , Biswal P. , Malini G.
Published: October 2019

INTRODUCTION:

Acute liver failure is characterized by a biochemical diagnosis of liver damage along with coagulopathy defined by international normalized ratio (INR) >2.0 despite intravenous vitamin K replacement or INR >1.5 with encephalopathy, without any chronic liver disease. It is a rare occurrence in a previously healthy adolescent. We report the case of acute severe hepatitis culminating into acute liver failure with suspected Wilson disease (WD).

CASE DESCRIPTION/METHODS:

A 14-year-old Asian female presented with hematemesis, watery stools and abdominal pain in the right upper quadrant for two days. The patient had 2-3 episodes of vomiting consisting of semi-digested food particles, watery in consistency that gradually progressed to one episode of emesis containing bright-red blood, about 15-20 ml in quantity associated with six episodes of watery stools, without any blood or mucus. She had consumed food cooked outside a few days prior. On admission, she had a pulse of 70 beats/minute, and blood pressure of 98/50 mm Hg. Physical examination revealed icterus, tenderness in the right hypochondriac region and palpable liver, about 3 cm below the costal margin. Laboratory investigations revealed hemoglobin of 10.7 gm/dl, aspartate aminotransferase of 9018 U/L, alanine aminotransferase of 6637 U/L, alkaline phosphatase of 261 U/L, total bilirubin of 2.8 mg/dl with a direct component of 1.6 mg/dl, prothrombin time of 32 seconds, partial thromboplastin time of 39 seconds, international normalized ratio of 2.69. Workup for viral markers was negative. Ultrasound of the abdomen revealed altered echotexture of the liver. The ophthalmologic examination did not reveal any abnormality. The serum ceruloplasmin level was 13 mg/dL. The patient was diagnosed with acute severe hepatitis with acute liver failure with suspected underlying WD. She was treated with intravenous doses of antibiotics including ciprofloxacin and amikacin, vitamin K, and octreotide, oral lactulose and ursodeoxycholic acid and was transfused two units of fresh frozen plasma. A gradual decrease of liver enzymes (Figure 1) and coagulation profile (Figure 2) was seen and she was followed up on an outpatient basis.

DISCUSSION:

The management of acute liver failure can be arduous requiring early diagnosis and aggressive treatment to improve better prognosis. Physicians should maintain a high amount of suspicion of concomitant disease in the setting of a young patient such as ours presenting with fulminant liver disease.
2517 Isn't Herpes Esophagitis Enough?
Authors: Nichols Sarah , Costello Patrick N. , Malkani Anjali
Published: October 2019

INTRODUCTION:

Herpes simplex (HSV) esophagitis, typically found in immunocompromised hosts, is increasingly being seen in immunocompetent hosts. A PubMed search covering the last 10 years yielded 8 publications of HSV esophagitis in the immunocompetent pediatric population, 3 of which were associated with eosinophilic esophagitis. No literature was found regarding HSV esophagitis associated with H. pylori infection. This is not surprising as the incidence of H. pylori is <10% in developed countries. To our knowledge, this is the first case report of co-infection of HSV esophagitis and H. pylori gastritis.

CASE DESCRIPTION/METHODS:

A healthy 16 year old male presented with 7 days of fever, severe odynophagia, and epigastric pain with no oral ulcers. He had a one year history of heartburn and dysphagia. Family history positive for H. pylori in paternal grandmother. He was seen at multiple facilities prior to transfer with prior diagnosis of costochondritis, pericarditis, pneumonia, and cholecystitis. Work up showed a normal KUB, abdominal ultrasound and upper GI series. WBC was 12.7 × 103/µL with left shift and elevated CRP at 86 mg/L. He was unresponsive to 24 hours of IV acid suppression therapy. Urgent endoscopy revealed extensive shallow ulcerations with exudate of the mid and distal esophagus and mild nodularity of the antrum. Empiric IV acyclovir was started with rapid symptom improvement within 24 hours. Biopsies showed ulcerated squamous esophageal mucosa with acute inflammation and exudate without eosinophilia. Adjacent reactive epithelium included binucleated to multinucleated cells with eosinophil nuclear structures suggestive of Cowdry type A inclusion bodies. A single cell showed a Cowdry type B inclusion but without the presence of classic multinucleated cells with molded nuclei. Immunohistochemistry confirmed the presence of HSV and was negative for CMV. Gastric biopsy showed mild, diffuse chronic gastritis with scant acute inflammation. CLO test was negative, but Diff-Quik and immunohistochemistry stains confirmed H. pylori. Triple therapy for H. pylori and a 10 day acyclovir course were completed. Currently his immune work-up is still pending.

DISCUSSION:

Since herpes esophagitis is a rare diagnosis in pediatrics, it is easy to ignore the possibility of co-infection. The importance of considering a co-infection in acute herpes esophagitis important to consider as the acute symptoms did not explain the patient's chronic peptic symptoms.
2520 Burkitt's Lymphoma of the Duodenum
Authors: Elkhouly Mohamed A. , Attar Bashar M. , Elkafrawy Ahmed A. , Salazar Miguel , Vohra Ishaan , Mutneja Hemant , Chicas Oscar Rivas
Published: October 2019

INTRODUCTION:

Burkitt's lymphoma is an aggressively growing tumor commonly, involving the jaw and facial bones. A rare variant of this type of lymphoma is found in immunocompromised patients specifically in the gastrointestinal tract, usually affecting the stomach, distal ileum, and cecum and rarely the duodenum. We are reporting a case of primary lymphoma of the duodenum in a patient with newly diagnosed AIDS.

CASE DESCRIPTION/METHODS:

A 50-years-old male with no significant past medical history presented with gradually progressive generalized weakness with easy fatigability of one-month duration. He had no history of shortness of breath, dysphagia, nausea, vomiting, hematemesis, melena, hematochezia or bleeding from any sites. Vitals were normal and examination was negative for abdominal tenderness, organomegaly or lymphadenopathy. Labs showed severe iron deficiency anemia with hemoglobin of 5.9 g/dl with ferritin of 21 ng/ml and Transferrin saturation of 8.8%. Other labs including WBC count, platelets count, creatinine, liver enzymes and LDH were normal. In suspicious of occult gastrointestinal tract bleeding, upper endoscopy was performed revealing a large semi circumferential malignant appearing mass partially obstructing the lumen of the third portion of the duodenum. Multiple biopsied where taken showing small bowel mucosa infiltrated by uniform, intermediate sized cells strongly staining positive for CD10, CD20, Ki-67 (100%) and BCL-6; diagnostic for Burkitt's lymphoma. CT Abdomen/Pelvis/Chest for evaluation of other masses or lymphadenopathy showed a 41 × 40 mm mass arising from the third and fourth portions of the duodenum with short segment wall thickening of the distal small bowel and soft tissue nodule in the right hemipelvis with no significant lymphadenoptahy. His labs reveleaed came positive for HIV-1 with 144,000 and CD4 count of 161. Bone marrow was negative for any involvement He received 2 cycles of alternating chemotherapy with R-CODOX-M and R-IVAC for 4 months duration. PET scan after 2 month of chemotherapy completion was negative for any uptake. Patient is currently doing well, on HAART therapy with no recurrence of his symptoms.

DISCUSSION:

Primary duodenal Burkitt lymphoma is a subtype of non-Hodgkin's lymphoma and represents an aggressive and rare malignancy with only a small number of cases reported worldwide. Clinical and radiological presentation is non-specific and usually mimics other gastric lymphomas, leading usually to late presentation and diagnosis.
2523 A Rare Location for a Rare Gastrointestinal Tumor With a Classical Presentation of Abdominal Pain, and Gastrointestinal Bleeding
Authors: Kesler Alex M. , Tan Winston , Gabriel Emmanuel , Bowman Andrew , Kröner Paul T.
Published: October 2019

INTRODUCTION:

Yolk sac tumors belong to the class of nonseminomatous germ cell tumor (NSGCT), which rarely present as extragonadal germ cell tumors (GCT) of the retroperitoneum. Patients present with small bowel obstruction or gastrointestinal bleeding. Most commonly, these retroperitoneal tumors affect the ileum or jejunum, but here we present a case of a 31-year-old man who presented with a retroperitoneal yolk sac tumor with associated fistulization to the duodenum, jejunum, and ileum.

CASE DESCRIPTION/METHODS:

A 31 year-old man with no medical or surgical history was admitted with abdominal pain and melena. He underwent contrast-enhanced abdominal computed tomography (CT) showing an enlarging mass with central necrosis, and small bowel fistulization. Gastroenterology was consulted and upper endoscopy was performed, showing evidence of a duodenal mass and fistula, which was biopsied. Initial biopsies were inconclusive. Surgical oncology was consulted, and the patient underwent attempted surgical resection but the majority of the small intestine, colon, and associated mesentery were tethered and resection was not possible. A repeat EGD-guided biopsy was performed and pathology had the histological and immunohistochemical features of a yolk sac tumor. Laboratory studies revealed elevated serum alpha-fetoprotein (AFP) of 992 ng/mL [<6.0 ng/mL], normal serum beta-human chorionic gonadotropin (beta-hCG), and elevated serum lactic acid dehydrogenase (LDH) of 421 U/L [122-222 U/L]. Testicular ultrasound was negative for any abnormalities. Oncology initiated the patient on neoadjuvant cisplatin, etoposide, and bleomycin (BEP) every three weeks for three cycles. Two weeks after starting first cycle, AFP had dropped to 65 ng/mL, and thereafter to 11 ng/mL. Repeat imaging showed that the tumor had decreased in size. Approximately three months after completion of the last cycle of chemotherapy the patient underwent retroperitoneal tumor resection, along with partial small bowel resection. There was no evidence of malignancy in the final surgical pathology. One year later he is cancer free.

DISCUSSION:

This case highlights a successful collaboration between gastroenterology, radiology, surgery and oncology. There are no randomized trials in the treatment of retroperitoneal GCTs, thus case reports such as this provide much needed guidance in the treatment of this rare condition. It also illustrates the importance of tissue diagnosis via EGD in order to provide prompt diagnosis without invasive surgical biopsy.
2534 Clinical Pearls in Current Diagnosis and Management of Salmonella paratyphi Infection
Authors: Hershman Melissa , Kambo Varinder
Published: October 2019

INTRODUCTION:

Typhoid and paratyphoid infections are rarely encountered in the United States, though are a major contributor to disease in developing countries. Growing virulence of organisms and lack of effective paratyphoid vaccination yield major public health concerns. We present two related individuals with typical symptoms of infection at varied presentation intervals.

CASE DESCRIPTION/METHODS:

A 31-year-old previously healthy female presented with four days of weakness, fevers, nausea, vomiting, and non-bloody diarrhea. She traveled to Pakistan two weeks prior. Admission examination was significant for fever of 100.5°F, hypotension, dry mucous membranes, ill appearance, and diffuse abdominal pain. Laboratory tests revealed white blood cell count 6.5 K/uL, hypoalbuminemia of 3.3 g/dL, alkaline phosphatase 178 U/L, AST 151 U/L, alanine aminotransferase ALT 130 U/L. Abdominal CT demonstrated terminal ileitis with right lower quadrant mesenteric lymphadenopathy up to 1.3 cm (Figure 1). Admission blood cultures grew SalmonellaParatyphi in both aerobic and anaerobic bottles, yet stool cultures remained negative on a second repeated sample. The laboratory reported sensitivity to ampicillin, ceftriaxone, ciprofloxacin and trimethoprim/sulfamethoxazole. However, additional requested sensitivity testing found nalidixic acid resistance, and minimum inhibitory concentration (MIC) of 1 to ciprofloxacin. The husband of the patient above, a 27-year-old male, presented two weeks after the discharge of his wife. He reported fever and chills for five days, with later abdominal cramping and diarrhea. On admission, white blood cell count was 4.9 K/uL with 19% bands, and liver function tests mildly elevated (AST 68 U/L, ALT 72 U/L). One of four blood culture bottles grew Salmonella Paratyphi with equivalent sensitivities. Two stool sample cultures were negative for Salmonella or enteric pathogen. Both patients completed two weeks of oral antibiotics with trimethoprim-sulfamethoxazole and did well subsequently.

DISCUSSION:

Our cases highlight diagnostic pearls of this condition including relative bradycardia after restoring hydration, leukopenia, transaminitis and ileitis. We demonstrate the utility of blood cultures, and how negative stool cultures may lead to false reassurance. Antibiotic resistance with Salmonella is challenging, particularly if providers are unaware of minimum inhibitory concentration cut-offs before selecting conventional fluoroquinolone therapy.
2535 Localized AL-Type Intestinal Amyloidosis Presenting as Refractory Iron Deficiency Anemia and Right Lower Quadrant Abdominal Pain
Authors: Kassim Gassan , Bsrat Makda , Rastogi Neelesh , Atluri Srilakshmi , Smith Michael S.
Published: October 2019

INTRODUCTION:

Amyloidosis is characterized by extracellular deposition of abnormally folded proteins creating insoluble fibrils, which disrupt tissue structure and function. Amyloidosis can be either systemic or localized. Localized amyloidosis is defined by the production of precursor proteins in the same location as amyloid deposition. We present a case of biopsy-proven localized AL intestinal amyloidosis.

CASE DESCRIPTION/METHODS:

A 91-year-old female with a past medical history significant for hypertension and iron deficiency anemia not improving with oral iron presented with progressively worsening dyspnea on minimal exertion, with diffuse weakness and fatigue. She denied any NSAID use. Hemoglobin on admission was 3.8 g/dL, improving to 6.4 g/dL after transfusion of 3 units of packed red blood cells. Upper endoscopy showed very friable mucosa with contact bleeding in the duodenum, along with patchy areas of abnormal mucosa in the 2nd portion (Figure 1). Duodenal biopsies were consistent with amyloidosis based on Congo red and crystal violet stains. Liquid chromatography tandem mass spectrometry was performed on peptides extracted from the specimen and indicated AL (lambda)-type amyloid deposition. Cardiac MRI showed no evidence of infiltrative heart disease or amyloid deposition. Bone survey showed no lytic lesion, and a marrow biopsy showed no morphologic evidence of plasma cell neoplasm with normal karyotype. Urine immunofixation electrophoresis showed no abnormalities. Urine and serum protein electrophoresis showed no monoclonal protein. With no evidence of systemic amyloidosis, diagnosis of localized intestinal amyloidosis was made. No systemic therapy was indicated. Patient was treated with IV iron transfusions. At a subsequent admission, she presented with subacute right lower quadrant pain. CT abdomen/pelvis with oral and intravenous contrast showed thickening of the distal small bowel with shouldering (Figure 2). Lesion was presumed to be secondary to intestinal amyloidosis. Patient refused further invasive interventions.

DISCUSSION:

Localized AL-type intestinal amyloidosis is rarely diagnosed. Common symptoms are non-specific and can include erosion, ulceration, bleeding, malabsorption, intractable diarrhea, obstruction or ischemia. This patient presented with recurrent symptomatic iron deficiency anemia refractory to oral iron repletion. Intestinal amyloidosis should be considered in similar presentations.