bluuebunny/crossref_metadata_2025_split huggingface dataset:
2505 Pregnancy as a Confounding Factor in Autoimmune Hepatitis
Authors: Padial Doble Ian C. , Cruz-Cruz Jorge , Antunez Ivan , Pastrana Rafael 
Published: October 2019

INTRODUCTION:
Autoimmune hepatitis (AIH) has a variety of clinical phenotypes. AIH presenting with acute liver failure (AFL) and jaundice is generally uncommon, however, other possible liver etiologies may confuse and delay diagnosis. Prompt recognition of AIH as possible cause of ALF is crucial in order to prevent morbidity and mortality associated with ALF. We present an atypical presentation that may help future clinicians in the early recognition and diagnosis of this condition despite possible confounders.

CASE DESCRIPTION/METHODS:
This is the case of a 39-year-old female (G2P1A0) with no history of systemic illness with intrauterine pregnancy of 32 5/7 weeks gestational age that was consulted to our service due to elevated transaminase and bilirubin levels. Patient had originally arrived at OB/GYN Emergency Department with complaints of nausea, vomits, jaundice and dark urine; no acholia, fever, or change in mental status noted. Additionally, patient reported recent use of oral antibiotics for UTI. On arrival patient presented hyperbilirubinemia (11.86) with direct predominance, elevated AST/ALT (251/134) and coagulopathy (INR 1.76). Patient with sudden clinical deterioration including altered mental status. Differential diagnoses included Acute Fatty Liver of Pregnancy (AFLP), Drug Induced Liver Injury, and preeclampsia with severe features. Due to suspected AFLP she was provided with IV steroid for fetal lung maturation and emergent C-Section preformed. MRCP reported no intra or extra hepatic bile duct dilation and liver US with Doppler noted with patent vasculature. Labs remarkable for negative ANA, ASMA, AMA, Anti DS DNA among other work up. After delivery patient had slow improvement of AST/ALT and bilirubin until 5 days later when AST/ALT and bilirubin levels began to increase steadily. Patient transferred to liver transplant center and empiric steroids started. Liver biopsy preformed and findings compatible with AIH but not diagnostic. After starting steroids patient began with slow improvement and was discharged on oral steroids. At post-hospitalization clinic visit, AST/ALT and bilirubin continued to improve and patient started on Azathioprine.

DISCUSSION:
It is suspected that initial brief improvement of liver function was secondary to steroids provided for fetal lung maturation, which lead us preemptively classify the ALF secondary to AFLP instead of AIH. This case is a prime example of pregnancy as a possible confounding factor that may delay the recognition and diagnosis of AIH.
2511 Acute Cholestatic Hepatitis E Infection: Immune Dissociation With Intra-Hepatic Replication
Authors: Debes Jose D. , Doukas Michael , de Man Robert , Boonstra Andre 
Published: October 2019

INTRODUCTION:
There is a lack of understanding of immune modulation during acute hepatitis E virus (HEV) infection. We evaluate the immune response during a case of autochthonous acute HEV genotype 3 infection in the Netherlands.

CASE DESCRIPTION/METHODS:
A 73 y/o patient initially presented with typical features of acute hepatitis. Extensive work up showed detectable HEV RNA in blood with positive IgM and genotype-3 molecular detection. The liver enzymes improved, there was viral clearance from blood and the patient was discharged home. However, on follow up appointment he was found to have severe cholestasis, was readmitted and passed away shortly. Real time PCR of liver biopsy revealed presence of HEV in liver tissue, suggestive of active intrahepatic infection despite clearance in blood. Peripheral immune evaluation performed via Pro-carta multiplex array showed that during the phase of detectable HEV RNA in serum, there was a surge of T-cell related immune mediators, as well as IFN-a and IP-10 characteristic of viral infections. After clearance of the virus in blood and development of cholestatic hepatitis, several inflammatory markers subsided, followed by an increase in immune factors related to anti-inflammatory activity as well as monocyte/macrophage related markers likely due to the intrahepatic presence of the virus.

DISCUSSION:
Our case describes a dissociation of intra- and extra-hepatic immune responses during acute HEV infection. As the shedding of the virus became solely intrahepatic, we observed an immune profile reflective of the activity of hepatic resident cells reflecting “competing immune interests' during HEV infection. Moreover, it exposes a less-known aspect of HEV: intra-hepatic morbidity despite lack of detection in blood.
2514 The Biggest Loser: A Case of Dramatic Weight Loss Achieved With an Intragastric Balloon
Authors: Chauhan Kirtan , Popov Violeta 
Published: October 2019

INTRODUCTION:
The prevalence of obesity and the medical costs associated with it are steadily rising, both among the adult and adolescent populations. New procedures such as the intragastric balloon (IGB), can provide rapid improvement in overall health to help manage and maintain weight loss with few adverse events.

CASE DESCRIPTION/METHODS:
A 61-year-old male patient with class III-IV obesity and multiple comorbidities had been in discussion with his gastroenterologist for over a year to undergo an IGB procedure. Peak weight was 302 lbs, with BMI of 43 kg/m2. He underwent intensive lifestyle and medical therapy, was started on Liraglutide 1.8 mg daily, with 15 lbs weight loss that plateaued. On his pre-procedure visit, hemoglobin A1C was 9.4, with a weight of 275 lbs and BMI 40.7 kg/m2. An intragastric Orbera™ IGB System (Apollo Endosurgery Inc, Austin, TX, United States) was placed and inflated with 600 ml of saline. On 2-month follow-up, the patient had lost 34 pounds. A number of his chronic medications, including those for hypertension and diabetes mellitus, were held due to improvement in lab and exam findings. The IGB was removed after 6 months. The patient continued to lose weight and has maintained daily exercise and dietary guidelines. Most recently, the patient's A1C level has trended down to 6.4%, with a weight of 202 lbs and a BMI of 29.9 kg/m2. Since starting the program, he has lost a total of 101 lbs.

DISCUSSION:
This case highlights the dramatic weight loss after a minimally invasive weight loss procedure in conjunction with proper diet and, exercise. The patient lost 27% of his body weight (275 lbs to 202 lbs) and went from class III obesity (>40 BMI), to an overweight range (25.0–29.9 BMI) with the IGB. Overall, he has lost over 100 lbs with 34.4% total weight loss, making him the “biggest loser' in our program. The effect on the patient's overall well-being has been extraordinary; over time, he has been able to start a regular exercise and dietary regimen, use less medication or experience remission of his chronic conditions. The typical weight loss expected with a gastric balloon is 10-12%. This patient's results have far exceeded the expected trajectory. Given the chronicity of obesity, and improvements in health seen with even 5-10% weight loss, a relatively safe endoscopic procedure such as the gastric balloon can significantly improve the health status of patients with obesity and should be considered before more invasive interventions.
2518 Transitory Hemophagocytic Lymphohistiocytosis (HLH)/Macrophage Activation Syndrome (MAS) in a Child With Very Early Onset (VEO) of Ulcerative Colitis (UC) Complicated With Primary Sclerosing Cholangitis (PSC)
Authors: Galeano Narmer F. , Rivera-Penera Maria T. , Bonilla Mary A. , Menell Jill S. , Kosinski Mark , Gupta Punita , Srinivasan-Mehta Jaya 
Published: October 2019

INTRODUCTION:
VEO - Inflammatory bowel disease (IBD), in children younger than 6 years of age, is frequently associated with a more severe course and distinct genetic profile compared with later onset IBD. We report the case of a child with VEO - UC complicated with PSC and HLH/MAS.

CASE DESCRIPTION/METHODS:
A 10-year-old male, presented with bloody diarrhea, hepatomegaly and elevated transaminases when he was 30 months old. He had moderate pancolitis associated with C difficile (Cdiff) infection. He improved after metronidazole and sulfasalazine (SFZ) treatment. Two months later, he had hematochezia and endoscopic worsening of his colitis, which improved after discontinuing SFZ and receiving steroids and 6-MP. MRCP was consistent with PSC (Figure 1) and he was started on ursodiol. At ∼ 7 years of age, while receiving treatment with 6MP and Infliximab, he was hospitalized due to fever, hepatomegaly, diarrhea and pancytopenia. Blood and stool cultures, and tests for Cdiff, EBV, CMV, rota and parvovirus were negative. Triglyceride and ferritin levels were normal. He improved clinically and his pancytopenia resolved after 5 days of empirical antibiotic treatment (AbTx). Bone marrow biopsy (BMBx) was deferred. Metabolites of 6MP were normal but 6MP was held temporarily. Six months later, he had fever and diarrhea associated with pancytopenia and high levels of ESR, CRP, ferritin, triglyceride and interleukin 2 receptor. Number and function of NK cells were decreased. Stools and blood cultures, and tests for EBV, HSV and CMV were negative. Cdiff stool antigen was positive but PCR was negative. BMBx showed histiocytosis and erythophagocytosis (Figure 2). Lymphocyte flow cytometry was negative for lymphoproliferative disorder. After AbTx he improved and pancytopenia and ferritin level corrected within one week. Routine immunodeficiency tests were normal. Gene mutations associated with familial HLH: PRF1, Rab27A, SH2D1A, STX11, STXBP2, UNC 13D and XIAP were negative. 6MP was held. Infliximab initially, and subsequently Adalimubab, were discontinued because of high antibodies and allergic reactions. Five months later 6MP was restarted. He has been in remission of UC for 16 months.

DISCUSSION:
HLH/MAS is a very rare, potentially fatal complication in IBD patients. Our patient has a history of VEO - UC, PSC and two episodes of pancytopenia not associated with EBV or CMV - one of which fulfilled criteria for HLH/MAS. Remarkably, he recovered within one week after empirical AbTx without steroids or other treatment for HLH/MAS.
2519  A Rare Case of Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma Involving Duodenum
Authors: Sonti Sravanthi , Nagireddy Sumana , Parsa Laxmi , Birsan Christina 
Published: October 2019

INTRODUCTION:
Primary small bowel lymphomas account for only 8-9% primary gastrointestinal malignancies and mostly represent extra nodal B- or T-cell origin. Immunoproliferative small bowel disease (IPSID) is a common small bowel extra nodal primary lymphoma. Associated Lymphoid Tissue B cell lymphoma (MALT lymphoma) is a common primary gastric lymphoma. However, it is very rare to find MALT lymphoma in small bowel especially in duodenum. We report a unique case of MALT lymphoma involving duodenal bulb.

CASE DESCRIPTION/METHODS:
A 58-Yr-Old African American female, with history of type II diabetes, Hypertension, Osteoarthritis, and chronic GERD with symptom control on once a day low dose omeprazole was referred for evaluation of epigastric discomfort, bloating, and nausea for 2 months. Abdominal examination normal. Labs showed normal CBC with differential, and CMP. An upper endoscopy showed mild patchy erythema in stomach and a localized, raised, and nodular area about 4-5 cm in the duodenal bulb. Biopsies from stomach showed Helicobacter pylori chronic active gastritis. Biopsies from duodenum showed homogenous population of lymphoid cells with moderate amounts of clear cytoplasm expanding the lamina propria. Molecular studies showed positive B-cell Gene Rearrangement with positive Framework 1, 2, and 3. Immunohistochemical staining was positive for CD20, bcl2, and kappa; and negative for CD3, CD5, CD10, bcl6, CD43, Cyclin D1 and lamda. The pathology findings confirmed Margina zone lymphoma of Mucosa Associated Lymphoid Tissue (MALT lymphoma). Colonoscopy was normal except few diverticula. A CT scan of abdomen, pelvis, and chest did not show any other masses except prominent lymph nodes at the gastrohepatic ligament and duodenum. She is treated with antibiotic therapy for H pylori gastritis, and referred to oncology for further management.

DISCUSSION:
MALT lymphomas of stomach usually occur in females and can be associated with Helicobacter pylori infection. They are usually low grade lymphomas and localized to gastrointestinal epithelium. Clinical features, imaging, or endoscopy findings are not specific. Immunohistochemical staining shows positive for B cell phenotype markers like CD20, Bcl-2, and negative for CD 23, CD 10, and CD 5. Usually, they present with non-specific symptoms like epigastric pain, nausea, vomiting, early satiety, though can present with obstruction when the tumor is too large. Treatment of associated H pylori infection may help though chemoradiotherapy may be needed rarely.
2521 Jejunal Carcinoid in a Patient With Primary Biliary Cholangitis Presenting as Small Bowel Obstruction
Authors: Singh Khushboo , Subramanian Sruthi K. , Abraham Fiyinfoluwa , Zhao Bihong , Machicao Victor 
Published: October 2019

INTRODUCTION:
Gastrointestinal carcinoid tumors arise from specialized neuroendocrine cells all along the gastro-entero-pancreatic system, which secrete bioactive amines and peptides. These are often asymptomatic and rarely cause obstructive symptoms. We describe a rare case of jejunal carcinoid presenting as small bowel obstruction.

CASE DESCRIPTION/METHODS:
A 60-year-old female with primary biliary cholangitis and scleroderma presented with three-months of abdominal pain, vomiting, and constipation with worsening three days prior to presentation. Patient was hemodynamically stable with unremarkable physical examination findings. Computed Tomography (CT) of abdomen and pelvis and follow up magnetic resonance enterography (MRE) showed a focal small bowel ileus with enlarged mesenteric lymph nodes (Figure 1). Chromogranin-A level was elevated to 11 nmol/L. The patient underwent exploratory laparotomy for small bowel obstruction; mesenteric mass and jejunal resection with jejunojejunal anastomosis was performed. The final histopathology report of both the lymph node and mesenteric mass revealed well-differentiated neuroendocrine tumor (NET), also termed carcinoid tumor (Figure 2). A follow-up Gallium PET/CT three weeks later showed residual disease in the small bowel mesentery near the site of resection for which the patient was started on Lanreotide.

DISCUSSION:
Carcinoid tumors are most common in the small bowel (30.8%) typically within 60 cm of ileocecal valve and are mostly asymptomatic. While midgut carcinoid may present with abdominal pain due to intermittent obstruction, mesenteric ischemia, or intraluminal tumor, it is rare. Although the initial modality of choice for small bowel obstruction is CT abdomen, it has low sensitivity in detecting small primary intestinal masses. MRE is more sensitive in detecting the dominant finding of a mesenteric mass. Octreotide scan and gallium-68 PET/CT can detect extent of disease or preoperatively diagnose patients with suspected NET that could not be detected using other modalities. Surgical resection with lymphadenectomy remains the standard treatment with a favorable prognosis of 83% five-year survival rate. Somatostatin analogs are the first line of treatment for any residual disease. With a favorable prognosis in mind, our case highlights the need for high clinical suspicion for carcinoid as part of the differential, especially in a patient presenting with small bowel obstruction and mesenteric mass in the setting of autoimmune disease.
2524 Rare Case of Numerous Duodenal Neuroendocrine Tumors Encompassing Entire Proximal Duodenum
Authors: Kendrick Katherine , Adsay Volkan , Sarmiento Juan , Sedghi Shahriar 
Published: October 2019

INTRODUCTION:
Gastrointestinal neuroendocrine tumors (GI-NETs) are rare neoplasms, but incidence is on the rise, in part due to increased detection. GI NETs can be found from the esophagus to the rectum. The appendix is the most common location.1,2 Primary duodenal NETs (D-Nets) are rare and account for less than 2% of tumors1. We report a case of a rare multiple duodenal NET.

CASE DESCRIPTION/METHODS:
44-year-old female who presented for anemia complaining of belching, flatus, and loose stools. EGD found the post-bulbar area studded with numerous 1 cm nodules, covering the entire proximal colon. Endoscopic biopsy revealed a spectrum of proliferations, which followed the distribution of Brunner glands. Many of the proliferations turned in the full-blown D-NETs positive for synaptophysin and chromogranin, the Ki-67 index was less than 3%, consent with neuroendocrine neoplasm grade 1 (NET tumor), in the 1st through the 3rd portion of the duodenum. Octrotide scan was negative. The patient was sent for a Whipple procedure. MEN type 1, NF, TSH, and 24 hr urine 5-HIAA were normal.

DISCUSSION:
GI NETs are often diagnosed in the 5th–6th decade of life and are common in African Americans and females.3 Primary tumors in the duodenum are rare and their frequency decreases toward the distal duodenum.4 Symptoms are non-specific, presenting with dyspepsia and epigastric pain. EGD is the best method for preoperative diagnosis, neuroendocrine markers such as serum chromogranin A and urine 5-HIAA, are supportive.4 In duodenal carcinoid tumors, ∼4% are associated with carcinoid syndrome.5 Duodenal carcinoids can be associated with clinical syndromes, such as Zollinger-Ellison syndrome or Multiple endocrine neoplasia-1 3. Carcinoid tumors, with metastasis express, have a high affinity for octreotide, and can be visualized with an OctreoScan.2,3 Duodenal tumors can be removed endoscopically or with resection. Our patient underwent Whipple's pancreaticoduodenectomy due to the peri-ampullary location of the tumors (Figure 1). The current study describes a rare case of multiple D-NETs carpeting the proximal duodenum, in a patient without elevated neuroendocrine markers, ZES, or MEN. Proliferation in the distribution of the Brunner glands indicates an underlying genetic alteration in the neuroendocrine cells associated with THE Brunner glands. From our literature search, we were only able to find two other case reporting multiple D-NETs and no cases reporting D-NETs proliferations from Brunner glands, thus a rare presentation.
2526 A Rare Cause of Intestinal Pseudo-Obstruction: Gastrointestinal Amyloid
Authors: Kochar Tanureet , Dhingra Parminder , Shah Hamza 
Published: October 2019

INTRODUCTION:
Amyloidosis is characterized by extracellular deposition of amyloid protein. It affects most commonly heart, kidney and GI tract. It can occur sporadically or in association with other conditions like multiple myeloma, chronic inflammatory diseases, infections etc. It has variable manifestations in GI tract from involving the stomach to the large bowel including liver.

CASE DESCRIPTION/METHODS:
This is 55 year old Caucasian male with past medical history of recent diagnosis of multiple myeloma, chronic kidney disease, hypertension, atrial flutter who presented to the hospital with chief complaints of multiple episodes of nausea and vomiting, diffuse abdominal pain, subjective fevers and chills from past 2 days. On presentation, vitals were stable. CBC- wnl, CMP – wnl, CT abdomen/pelvis without contrast revealed partial small bowel obstruction. Patient had similar symptoms in prior admissions. During the initial hospitalization 1 month ago, he underwent exploratory laparotomy with no findings to suggest mechanical small bowel obstruction. Subsequently, he had an EGD with push enteroscopy which identified enteritis and a Mallory-Weiss tear. Small bowel biopsies were performed with pathology noting: focal acute cryptitis and a background of moderate chronic nonspecific inflammation. Repeat EGD with push enteroscopy revealed non erosive esophagitis, mild gastritis and severe duodenitis, biopsies were obtained. Congo red staining of the biopsy revealed amyloidosis of the small bowel but the gastric biopsy was negative. The patient was then referred to higher center for consideration of bone marrow transplant. The fat pad biopsy was never obtained for systemic amyloidosis.

DISCUSSION:
The AL amyloidosis which is most commonly associated with monoclonal free light chains deposition, which was also previously called primary amyloidosis is most common. The AA amyloidosis (also called as secondary amyloidosis) is secondary to chronic inflammation in the body due to infection, rheumatoid arthritis etc. Diagnosis of amyloid, irrespective of the type, depends upon clinical, laboratory and histological findings. Serum and urine immunoelectrophoresis are the initial laboratory tests, but confirmation requires histological evaluation. Overall median survival in untreated patients or nonresponders with systemic amyloidosis is <2 years. Treatment should be directed at the underlying cause. GI complications are managed with symptomatic control.
2528 Extensive Small Bowel Ischemia With Heavy Alcohol Consumption
Authors: Leung David , Gaddipati Kishore 
Published: October 2019

INTRODUCTION:
Acute mesenteric ischemia (AMI) is a disorder associated with atherosclerosis and cardiovascular disorders. It includes a range of disease etiologies including nonocclusive mesenteric ischemia (NOMI). NOMI is the most lethal form of AMI with mortality rates up to 70%. Alcohol is well recognized as a systemic toxin that causes serious health problems. Intestinal complications caused by alcohol consumption, however, are not well documented. We present a rare case of a patient who developed NOMI following excessive alcohol consumption.

CASE DESCRIPTION/METHODS:
A 52-year old male presented with a chief complaint of abdominal pain. The abdominal pain began after uncharacteristically drinking 11 shots of hard liquor. The patient developed abdominal pain along with several bouts of emesis. Initial imaging with a CT abdomen demonstrated non-specific enteritis. Later that evening, the patient developed peritoneal signs. A repeat CT abdomen showed progressive proximal small bowel distension, free fluid in the abdomen and pelvis along with pneumatosis in the proximal ileum (Figure 1). Surgery revealed frank necrosis, gangrene and extreme distension of small bowel, 20 cm proximal to the ileocecal valve. The ischemic small bowel was resected as a result. The proximal and distal ends of the small bowel were stapled and left in discontinuity. Pathology collected from the small bowel resection revealed acute inflammation and both mucosal necrosis along with transmural gangrenous necrosis of the small bowel (Figure 2). It additionally showed patent mesenteric vessels without evidence of fibrin thrombi (Figure 3). The patient subsequently underwent a small bowel anastomosis and was discharged.

DISCUSSION:
NOMI secondary to excessive alcohol binging has only been reported in the literature only once before. Similar to the case reported in the Journal of the Korean Society of Coloproctology, there was no history of CVD or atrial fibrillation to suggest thrombotic or embolic mesenteric ischemia. Additionally, there were no findings suggestive of correlating mesenteric involvement on pathology. Of note, our case differed in that our patient did not have a significant alcohol abuse history which the previously reported case had noted. Studies in the literature suggest that ethanol can produce prolonged vasoconstriction in cerebral blood vessels which become involved in hypoxic, ischemic and hemorrhagic actions on the brain. However, mechanisms of alcohol-induced vasoconstriction of splanchnic vessels are still unclear.