bluuebunny/crossref_metadata_2025_split huggingface dataset:
A141 SPONTANEOUS ESOPHAGEAL PERFORATION: AN UNREPORTED COMPLICATION OF LYMPHOCYTIC ESOPHAGITIS
Authors: LAGROTTERIA A , Collins A W , Someili A , Narula N 
Published: March 2021

Abstract

Background
Lymphocytic esophagitis is a new and rare clinicopathological entity. It is a histological pattern characterized by lymphocytic infiltrate without granulocytes. Its etiology and clinical significance remains unclear. The clinical manifestations are typically mild, with reflux and dysphagia the most commonly reported symptoms.

Aims
We describe a case report of spontaneous esophageal perforation associated with lymphocytic esophagitis.

Methods
Case report

Results
A previously well 31-year-old male presented to the emergency department with acute food impaction. His antecedent symptoms were acute chest discomfort and continuous odynophagia following his most recent meal, with persistent globus sensation. The patient had no reported history of allergies, atopy, rhinitis, or asthma. A previous history of non-progressive dysphagia was noted after resuscitation. Emergent endoscopy revealed no food bolus, but a deep 6 cm mucosal tear in the upper-mid esophagus extending 24 to 30 cm from the incisors. Chest computed tomography observed small volume pneumoperitoneum consistent with esophageal perforation. The patient’s recovery was uneventful; he was managed conservatively with broad-spectrum antibiotics, proton pump inhibitor therapy, and a soft-textured diet. Endoscopy was repeated 48 hours later and revealed considerable healing with only a residual 3-4cm linear laceration. Histology of biopsies taken from the mid and distal esophagus demonstrated marked infiltration of intraepithelial lymphocytes. There were no eosinophils or neutrophils identified, consistent with a diagnosis of lymphocytic esophagitis.
Autoimmune indices including anti-nuclear antibodies and immunoglobulins were normal, ruling out a contributory autoimmune or connective tissue process. The patient was maintained on a proton pump inhibitor (pantoprazole 40 mg once daily) following discharge.
Nearly six months following his presentation, the patient had a recurrence of symptoms prompting representation to the emergency department. He described acute onset chest discomfort while eating turkey. Computed tomography of the chest redemonstrated circumferential intramural gas in the distal esophagus and proximal stomach.

Conclusions
Esophageal perforation is a potentially life-threatening manifestation of what had been considered and described as a relatively benign condition. From isolated dysphagia to transmural perforation, this case significantly expands our current understanding of the clinical spectrum of lymphocytic esophagitis.

Funding Agencies
None
A143 ACUTE ESOPHAGEAL NECROSIS: A COMPLICATION OF DIABETIC KETOACIDOSIS
Authors: Mahmood T , Moss K , Spaziani R 
Published: March 2021

Abstract

Background
Acute esophageal necrosis (AEN) is a rare entity associated with diabetic ketoacidosis (DKA). The pathogenesis is thought to be linked to low volume state, microvascular disease, impaired gastric and esophageal motility increasing acid reflex, all rendering the esophagus prone to injury.

Aims
We report a case of AEN as a complication of DKA in a patient without any overt gastrointestinal bleeding (GIB), along with a literature review.

Methods
Keywords “esophageal necrosis” and “diabetic ketoacidosis” were used in MEDLINE and BASE to retrieve English articles reporting cases of AEN in DKA.

Results
A 63 year old male with history of hypertension, dyslipidemia and non-insulin dependent diabetes mellitus presented to Emergency with 5 day history of severe epigastric pain, dysphagia to solids and liquids, nausea and vomiting (without any overt GIB). Most recent HbA1c was 8.4%. His diabetes was managed with metformin and semaglutide. Bloodwork revealed a hemoglobin of 165g/L and leukocytes of 17.9x109/L. Chemistries showed an anion gap of 25 with bicarbonate of 5mmol/L. Venous blood gas showed acidemia (pH=7.02). B-hydroxybutyrate level was 10.2mmol/L. Urinalysis was negative for leukocytes or nitrites. An abdominal CT ruled out bowel obstruction or intra-abdominal infection/abscess as the source of his discomfort but demonstrated circumferential wall thickening of the distal esophagus. No other triggers were found for this patient’s DKA except perhaps a recently started ketogenic diet. After resolution of DKA, he continued to experience severe epigastric pain, reflux symptoms, and dysphagia. An esophagogastroduodenoscopy (EGD) was performed, which showed AEN with circumferential black, necrotic inflammatory changes in the mid to distal esophagus. Erosions were seen in the body and antrum of the stomach, and multiple clean based ulcers were seen in the duodenum. Patient was started on an insulin regimen prior to discharge. Review of literature shows a total of 13 cases of AEN in DKA, with only one case where the patient did not present with any clinical bleeding. Risk factors for AEN include, hypertension, diabetes mellitus, malignancy, male gender, older age, chronic kidney disease, alcohol abuse and cardiovascular disease. While no medications have been linked to AEN, our patient was recently started on semiglutide, which has been implicated in impaired gastric emptying and increased GERD symptoms. This may further explain why the patient developed AEN.

Conclusions
AEN is a rare entity, especially in the context of DKA. Usually patients present with overt GIB; however, on occasion dysphagia, nausea, and vomiting can be the predominant symptoms. Hence, the threshold to perform EGD in patients with DKA should be low, given their low volume state and potentially impaired gastrointestinal motility due to microvascular disease or medications, putting them at higher risk for AEN.

Funding Agencies
None
A144 ISOLATED UPPER ESOPHAGEAL VARICES DUE TO SUPERIOR VENA CAVA SYNDROME: A CASE OF UPPER GI BLEEDING
Authors: Squirell E , Ropeleski M , Fardy J 
Published: March 2021

Abstract

Background
Esophageal varices represent a cause of upper gastrointestinal bleeding (UGI) with significant associated morbidity and mortality. The majority of variceal bleeds are due to portal hypertension and cirrhosis. In some very rare cases isolated upper esophageal varices may occur. These present management challenges, as typical management options for varices in cirrhosis are not applicable due to differences in pathophysiology. Infusions to decrease portal pressure and TIPS procedures may be used in cirrhosis, but are not effective in patients with SVC syndrome.

Aims
We present a literature review on isolated upper esophageal varices due to SVC syndrome, and present a case that provides further confirmation that surgical vascular reconstruction is a viable option in these cases.

Methods
The case of a 49 year old female with variceal hemorrhage secondary to SVC syndrome was reviewed. A search of the literature was undertaken using the terms “varices + superior vena cava syndrome” and “upper esophageal varices + management.”

Results
Overall, the majority of cases of isolated upper esophageal varices are secondary to SVC syndrome. They typically occur in cases of benign, rather than malignant SVC syndrome. Only one previous case was reported to be managed with surgical vascular reconstruction. We present the case of a 49-year old woman with SVC syndrome secondary to a thrombosed dialysis line who went on to develop isolated upper esophageal varices and large volume UGI bleeding. The pathophysiology of these “downhill” varices differs enough from portal hypertension that typical variceal management options may not be appropriate. Given a previous history of unsuccessful balloon angiography and the risk associated with variceal bleeding, she was taken back to the operating room for surgical vascular reconstruction. A surgical bypass was performed. She did well post-surgery and was discharged. She had no recurrence of bleeding and had a resolution of her edema at 6 month follow up.

Conclusions
Isolated upper esophageal varices are a potentially serious complication of SVC syndrome, and should be considered in cases of upper GI bleeding in patients with dialysis lines as they can have limited management options. This case was successfully managed by surgical vascular reconstruction, which represents an important treatment option in these difficult cases.

Funding Agencies
None
A158 THE EFFECTIVENESS AND SAFETY OF HYPERBARIC OXYGEN THERAPY IN INFLAMMATORY BOWEL DISEASE AND ITS ASSOCIATED CONDITIONS: A SYSTEMATIC REVIEW AND META-ANALYSIS
Authors: Chin Koon Siw K , Kandel R , Rosenfeld G , Boet S , Larrigan S , McCurdy J D 
Published: March 2021

Abstract

Background
Hyperbaric oxygen therapy (HBOT) corrects tissue hypoxia, mobilizes stem cells and has immunomodulatory effects, all of which are key mechanisms for healing wounds. A number of studies have suggested that HBOT may be effective for healing inflammatory bowel disease (IBD).

Aims
Our systematic review aimed to quantify the effectiveness and safety of HBOT in IBD and its associated conditions.

Methods
We performed a proportional meta-analysis. MEDLINE, EMBASE, Web of Knowledge and The Cochrane Central Register of Controlled Trials were systematically searched from inception through November 2020 with no language restriction. We included randomized controlled studies, cohort studies and case series that contained a minimum of three patients and reported effectiveness and/or safety outcomes for HBOT in patients with IBD. Studies were stratified by IBD phenotype and weighted summary estimates with 95% confidence intervals (CI) were calculated for clinical response and remission using random-effects models. Study quality was assessed using the Cochrane risk-of-bias tool for randomized trials and a modified version of the National Institutes of Health (NIH) checklist for observational studies.

Results
Nineteen studies met our study criteria: 3 randomized controlled trials and 16 case series. The studies reported outcomes for luminal ulcerative colitis (UC) (n=373), luminal Crohn’s disease (CD) (n=250), enterocutaneous fistulae (ECF) (n=21), perianal CD (n=115), pouch disorders (n=60), pyoderma gangrenosum (PG) (n=5) and perianal sinus/metastatic CD (n=7). Rates of clinical response were 86% (95% CI, 66–95%) for luminal UC, 86% (95% CI, 81–90%) for luminal CD, 85% (95% CI, 61–95%) for ECF, 80% (95% CI, 70–87%) for perianal CD, 65% (95% CI, 52–76%) for pouch disorders, 92% (95% CI, 38–99%) for PG and 79% (95% CI, 36–96%) for perianal sinus/metastatic CD. Rates of clinical remission were 87% (95% CI, 10–100%) for luminal UC, 88% for luminal CD (95% CI, 46–98%), 50% for ECF (95% CI, 12–88%), 64% (95% CI, 52–75%) for perianal CD, 31% (95% CI, 16–50%) for pouch disorders, 92% (95% CI, 38–100%) for PG and 65% (95% CI, 10–97%) for perianal sinus/metastatic CD. Of the ten studies that reported on safety of HBOT, 19 patients (10.5%) had minor adverse events and no major event was reported. Study quality was low in the majority of studies due to an absence of comparator arms, inadequate description of interventions, and poorly defined outcomes.

Conclusions
Limited high-quality evidence suggest that HBOT is safe and associated with high rates of clinical response and remission for luminal IBD, perianal CD and pouch disorders. A well-designed large multicenter randomized controlled trial is warranted to confirm the benefit of HBOT in IBD.
doi:10.17605/osf.io/gpz6d

Funding Agencies
None
A166 INCIDENCE OF INFLAMMATORY BOWEL DISEASE IN SOUTH ASIAN AND CHINESE PEOPLE: A POPULATION-BASED COHORT STUDY FROM ONTARIO, CANADA
Authors: Dhaliwal J , Tuna M , Murthy S , Shah B , Griffiths A , Benchimol E I 
Published: March 2021

Abstract

Background
Studies using population-based data from Ontario previously found South Asian (SA) and East Asian immigrants develop IBD at lower rates than non-immigrants. However, incidence among the Canadian-born children of SA immigrants was comparable to those of non-immigrants, and younger age at arrival to Canada was associated with an increased risk.

Aims
The primary aim of the study was to determine the incidence of IBD in South Asian and Chinese people compared with the general Ontario population.

Methods
All incident cases of IBD in children (1994–2015) and adults (1999–2015) were identified from population-based health administrative data in Ontario, Canada using validated algorithms. Ethnicity was assigned by linkage of a validated surname-based database to health administrative data, immigration records, and hospital birth data. We determined standardized incidence of IBD and the adjusted incidence rate ratio (aIRR) in SAs and Chinese comparable to the general population. Based on existing evidence, we adjusted for confounders selected a priori: immigration status, sex, income and rurality.

Results
Amongst 14,483,769 people in the general population, standardised incidence of IBD per 100,000 person-years (PY) was 24.7 (95%CI 24.4–25.0), compared with 14.6 (95%CI 13.7–15.5) in 982,472 SAs and with 5.4 (95%CI 4.8–5.9) in 764,397 Chinese. The risk of IBD in SAs was lower to the general population prior to adjusting for immigration, IRR 0.82 (95%CI 0.78–0.86), but comparable after adjusting for confounders (aIRR 1.10, 95%CI 1.05–1.16). Only SA adults (18-64y) had a higher risk of IBD (aRR 1.10, 95% CI 1.02–1.18), not children or seniors. Compared to the general population, SAs had a lower risk of Crohn disease (CD), irrespective of age (aIRR 0.66, 95%CI 0.60–0.77), but a higher risk of ulcerative colitis (UC) (aIRR 1.47 (95% CI 1.34–1.61).). SAs were more likely to develop UC than CD (aIRR 1.51, 95%CI 1.38–1.66). The risk of IBD was much lower in Chinese people, irrespective of age (aIRR 0.24, 95%CI 0.20–0.28). This lower risk was similar in CD (aIRR 0.21, 95%CI 0.17–0.26) and UC (aIRR 0.28, 95%CI 0.23–0.25). The SA population had a considerably higher risk of developing IBD compared to the Chinese population (aIRR 4.29, 95%CI 3.62–5.08), and were more likely to develop UC (aIRR 1.36, 95% CI 1.08–1.73).

Conclusions
Canadian SAs had a similarly high risk of developing IBD compared with the general population after adjusting for immigration status, a finding distinct from the Chinese population. There was an age-response relationship in SAs only. Furthermore, SAs were more likely to develop UC than people of Chinese origin, potentially owing to differences in genetic susceptibility and/or environmental factors.

Funding Agencies
None
A167 UNDERSTANDING THE IMPACT OF DOWNSTREAM OF KINASE 4 (DOK4) DAMAGING GENETIC VARIANTS IN THE PATHOGENESIS OF PEDIATRIC INFLAMMATORY BOWEL DISEASE (IBD).
Authors: Batura V , Ricciuto A , Warner N , Guo C , Kotlarz D , Klein C , Muise A 
Published: March 2021

Abstract

Background
IBD is a chronic inflammatory disorder of the gastrointestinal (GI) tract whose precise pathological mechanisms remain elusive. It is thought that in pediatric IBD, pathogenic exposure does not appear sufficient to cause disease, thus genetic variations are critical to disease pathogenesis. The Muise Lab uses genetic sequencing of patients with IBD from all over the world to identify crucial genetic variations that are critical to IBD development.
We report two patients with IBD from unrelated families with mutations in DOK4. Both patients had profound extra-intestinal disease complicating their IBD.
Downstream of kinase (DOK) proteins are a family of adaptor molecules that are important in regulating cell signaling, especially in immune cells. They are known to suppress MAPK and PI3K/AKT pathways, whose dysregulation result in cancer. DOK4 has not been extensively studied, but research suggests that this gene produces two isoforms. It is known to have negative regulatory effects on immune cell activation but is also expressed across various other tissues, where its function is yet to be determined.

Aims
We hypothesize that these variations in DOK4 lead to immune cell dysregulation, which manifests in both gastrointestinal and systemic chronic inflammatory disease. Through this study, we aim to elucidate the mechanism of novel genetic defects in DOK4.

Methods
It will be critical to understand how variants within both patients are contributing to the onset of IBD through in vitro studies. Therefore, we will characterize and quantify how changes in expression of DOK4 alters essential cell signaling pathways. We have established immortalized cell lines from patients bearing these mutations to specifically characterize potential immune defects. We will also be using knock out cell models to understand the effect of loss of function of DOK4 in different cell types.

Results
Preliminary data shows variation in expression of the protein within patient peripheral blood mononuclear cells (PBMCs) compared to a healthy donor. Overexpression in HEK293T cells shows changes in MAPK and NFkB signaling.

Conclusions
With this study, we hope to identify new therapeutic targets for patients with DOK4 mutations.

Funding Agencies
CIHRThe Leona M. and Harry B. Helmsley Charitable Trust
A172 THE ASSOCIATION OF EFFICACY, OPTIMISM, UNCERTAINTY AND HEALTH ANXIETY IN INFLAMMATORY BOWEL DISEASE ACTIVITY OVER TIME
Authors: Stone J , Shafer L , Graff L A , Witges K , Lix L , Haviva C , Targownik L E , Bernstein C N 
Published: March 2021

Abstract

Aims
We aimed to assess if the presence of positive or negative psychological attributes are associated with disease activity in a prospective inflammatory bowel disease (IBD) cohort using validated psychological assessment tools and various measures of disease activity.

Methods
The study included 146 adults with confirmed and recently active IBD enrolled in a prospective longitudinal cohort study. Demographics, disease information, validated measures of psychological functioning related to self-efficacy, optimism, health anxiety and intolerance of uncertainty were collected at baseline, week 26 and week 52. Disease activity indicators included fecal calprotection (FCAL), the Inflammatory Bowel Disease Symptom Inventory (IBDSI), and self-reported flares and were collected at study baseline, week 26 and week 52. Logistic regression was used to identify the relationship between psychological functioning and disease activity.

Results
Participants’ mean age was 42.9 years (SD 12.6; range 18–70), with 70.5% women. Almost two thirds (65.1%) had a diagnosis of Crohn’s disease (CD), 34.2% had ulcerative colitis (UC), and 0.7% (n=1) was IBD unclassified. 22% had income &lt;$50,000 and 63% were in a current relationship. Patient-reported active disease (i.e., IBDSI; flare self-report) was significantly less likely with higher self-efficacy [OR= 0.87, 95% CI 0.82–0.93 (IBDSI); OR= 0.86, 95% CI 0.81–0.91 (self-report)] and significantly more likely with higher health anxiety (OR=1.09, 95% CI 1.04–1.15) with the association remaining for self-reported flares after adjusting for demographic variables. The psychological attributes were not associated with active disease based on FCAL levels.

Conclusions
Higher health anxiety increases the likelihood of experiencing an IBD flare, while higher general self-efficacy may be protective of a disease flare.

Funding Agencies
None
A176 THE IMPACT OF COVID-19 ON ANXIETY, DEPRESSION, AND STRESS IN WOMEN WITH INFLAMMATORY BOWEL DISEASE: A CROSS-SECTIONAL SURVEY
Authors: Tandon P , O’Connor K , Maxwell C , Nguyen G C , Huang V W 
Published: March 2021

Abstract

Background
Women with inflammatory bowel disease (IBD) are at an increased risk of mental-health illness and reduced fertility.

Aims
To determine the impact of the coronavirus disease 2019 (COVID-19) pandemic on the mental-health and pregnancy plans of women with IBD.

Methods
Women with IBD (age 18–45) were asked to anonymously complete surveys on baseline demographics, IBD characteristics, and mental health comorbidities. They were also asked to comment on prior symptoms consistent with COVID-19 and whether they were tested for the virus. Finally, patients were asked to complete three mental health surveys to reflect on anxiety (Generalized Anxiety Disorder-7 (GAD7)), depression (Patient Health Questionnaire-9 (PHQ9)), and stress (Perceived Stress Scale (PSS)) symptoms prior to and during the pandemic. Total scores were reported as continuous variables and means with standard deviations (SD) were compared using paired T-tests.

Results
Twenty-nine patients (12 UC, 17 CD) were included. 14 patients were preconception, 12 were pregnant, and 3 were post-partum. The mean age was 31.4 (SD 3.7). Fifteen of 29 (51.7%) of patients were on anti-tumor necrosis factor therapy. Twelve (41.4%) and 6 (20.7%) patients had pre-morbid anxiety and depression prior to the pandemic. COVID-19 symptoms were reported in 8 patients (27.5%). Six patients had undergone COVID-19 testing, all of whom had a negative test. Four patients indicated that COVID-19 had negatively affected their plans for pregnancy, with reasons reported including fear of the hospital (n=1), fear of COVID-19 impact on the fetus (n=2), and uncertainty on the duration of COVID-19 (n=1). During the pandemic, fourteen of 28 (50%) patients experienced symptoms of anxiety (GAD score &gt; 5), with a majority (70%) experiencing mild symptoms (score 5–9). During the pandemic, 60.7% (17/28) and 71.4% (20/28) reported symptoms of depression (PHQ9 &gt; 4) and at least moderate stress (PSS &gt; 14) respectively. Furthermore, compared to pre-pandemic, 57.1% (16/28) and 67.9% (19/28) had an increase in depression and stress symptoms during the pandemic respectively. This appeared to only apply to those with CD, but not UC (Table 1). Compared to pre-pandemic, those with stricturing CD appeared to have higher stress scores whereas those with fistulizing and perianal disease appeared to have higher depression scores during the COVID-19 pandemic (Table 1). A lower house-hold income and a reduction in exercise during the COVID-19 pandemic appeared to increase the risk of stress, depression, and anxiety symptoms.

Conclusions
Over half of women with IBD indicate worsening of anxiety, depression, and stress symptoms during the COVID-19 pandemic. It remains critical that health-care professionals address these mental health concerns during these otherwise difficult times.

Funding Agencies
None