Eosinophilic esophagitis: Current concepts in diagnosis and management.

ABSTRACT: Eosinophilic esophagitis is an antigen-mediated chronic inflammatory disorder that has risen in incidence and prevalence over the past 2 decades. The clinical presentation is variable and consists of mainly esophageal symptoms such as dysphagia, heartburn, food impaction, and vomiting. Current management relies on dietary elimination, proton-pump inhibitors, and topical corticosteroids with different response rates and relapses after treatment discontinuation. With a better understanding of the underlying pathophysiology, many molecules emerged recently as targeted treatment including dupilumab (IL4/IL13 blocker), as the first FDA-approved biological treatment, which has changed the management paradigm.

Progressive Familial Intrahepatic Cholestasis: A Descriptive Study in a Tertiary Care Center.

Background: Progressive familial intrahepatic cholestasis (PFIC) is a rare genetic disorder that results from defective mechanisms of bile secretion. We aim to describe different types of PFIC and their clinical features, treatment modalities, and outcomes in Saudi Arabia. Patients and Methods. This is a retrospective study of all patients diagnosed with PFIC at King Faisal Specialist Hospital and Research Center in Riyadh from January 1, 2002, to December 31, 2021. All relevant information was collected from patient charts and transferred into the REDcap® database for statistical analysis. Results: A total of 79 patients were identified with PFIC, and PFIC type 3 was the most common (59.5%), followed by PFIC type 2 (34.2%), PFIC type 1 (5.1%), and PFIC type 4 (1.3%). Males and females were affected in 54.4% and 45.6%, respectively. Mutations in ATP8B1, ABCB11, and ABCB4 genes were observed in PFIC type 1, PFIC type 2, and PFIC type 3, and loss of function in a variant of TJP2 was detected in PFIC type 4, respectively. A total of 51 (64.6%) patients underwent liver transplantation: three patients (3/4) with PFIC type 1 (75%), twenty patients (20/27) with PFIC type 2 (74.1%), twenty-seven patients (27/47) with PFIC type 3 (57.4%), and one patient with PFIC type 4 (100%). The mean duration of disease before transplantation was 53.9 ± 67 months with a median of 30 months. Following liver transplantation, symptomatic control was achieved in 47 patients (92.2%). Recurrence after transplantation occurred in 4 patients (7.8%) within an average of 22.5 months and a median of 17 months. Conclusion: PFIC is considered a rare disorder in Saudi Arabia; however, early recognition of the disease is important for appropriate management and early referral for liver transplantation evaluation. The overall rate of liver transplantation in our cohort was 64.6% with an excellent five-year survival rate.

Complementary and alternative medicine use and its association with medication adherence in inflammatory bowel disease and other gastrointestinal diseases.

Background: The use of complementary and alternative medicines (CAMs) has been embedded in populations for decades. In this study, we aimed to determine the rate of their usage among inflammatory bowel disease (IBD) patients and their association with adherence to conventional therapies. Methods: In this cross sectional, survey-based study, IBD patients' (n=226) adherence and compliance were evaluated using the Morisky Medication Adherence Scale-8. A control sample of 227 patients with other gastrointestinal diseases was included to compare trends of CAM use. Results: Crohn's disease represented 66.4% of those with IBD, with a mean age of 35 ± 13.0 years (54% males). The control group had either chronic viral hepatitis B, gastroesophageal reflux disease, Celiac disease, or other non-IBD diseases, with a mean age of 43.5 ± 16.8 years (55% males). Overall, 49% of patients reported using CAMs (54% in IBD group and 43% in the non-IBD group, P =0.024). Across both groups, the most used CAMs were honey (28%) and Zamzam water (19%). There was no significant association between the severity of the illness and use of CAMs. Patients who used CAMs had a lower adherence to conventional therapies vs. those who did not use CAMs (39% vs. 23%, P =0.038). Using the Morisky Medication Adherence Scale-8, low adherence to medications was reported in 35% of the IBD group vs. 11% of non-IBD group (P = 0.01). Conclusion: In our population, patients with IBD are more likely to use CAMs and are less adherent to medications. Furthermore, the use of CAMs was associated with a lower adherence rate to conventional therapies. Consequently, further studies assessing the causes associated with the use of CAMs and nonadherence to conventional therapies should be explored and interventions designed to mitigate nonadherence.

The Saudi Gastroenterology Association guidelines for quality indicators in gastrointestinal endoscopic procedures.

The quality and safety of gastrointestinal endoscopy varies considerably across regions and facilities worldwide. In this field, quality management has traditionally focused on individual performance of endoscopists, with most indicators addressing process measures and limited evidence of improvement in health outcomes. Indicators of quality can be classified according to their nature and sequence. The various professional societies and organizations have proposed many systems of indicators, but a universal system is necessary so that healthcare professionals are not overburdened and confused with a variety of quality improvement approaches. In this paper, we propose guidelines by the Saudi Gastroenterology Association pertaining to quality in endoscopic procedures aiming to improve the awareness of endoscopy unit staff toward important quality indications to enhance and standardize quality of care provided to our patients.

Mucormycosis and Cryptococcosis with Gastrointestinal Involvement in a Patient with Poorly Managed Diabetes.

Mucormycosis and cryptococcosis are invasive fungal infections that mostly infect immunocompromised patients and are associated with high mortality rates. Here, we report a case of a 54-year-old male with poorly controlled diabetes mellitus who was initially admitted with a complaint of right frontal headache and vomiting for 5 days. The patient was found to have paranasal sinuses mucormycosis, and later developed gastrointestinal cryptococcosis. A multidisciplinary approach and early management are important to avoid any delay in managing these life-threatening infections. To the best of the authors' knowledge, this is the first case reporting concurrent invasive fungal infections in a patient.

Practice guidelines for the management of Helicobacter pylori infection: The Saudi H. pylori Working Group recommendations.

The eradication rates for Helicobacter pylori globally are decreasing with a dramatic increase in the prevalence of antibiotic resistant bacteria all over the world, including Saudi Arabia. There is no current consensus on the management of H. pylori in Saudi Arabia. The Saudi Gastroenterology Association developed these practice guidelines after reviewing the local and regional studies on the management of H. pylori. The aim was to establish recommendations to guide healthcare providers in managing H. pylori in Saudi Arabia. Experts in the areas of H. pylori management and microbiology were invited to write these guidelines. A literature search was performed, and all authors participated in writing and reviewing the guidelines. In addition, international guidelines and consensus reports were reviewed to bridge the gap in knowledge when local and regional data were unavailable. There is limited local data on treatment of H. pylori. The rate of clarithromycin and metronidazole resistance is high; therefore, standard triple therapy for 10-14 days is no longer recommended in the treatment of H. pylori unless antimicrobial susceptibility testing was performed. Based on the available data, bismuth quadruple therapy for 10-14 days is considered the best first-line and second-line therapy. Culture and antimicrobial susceptibility testing should be considered following two treatment failures. These recommendations are intended to provide the most relevant evidence-based guidelines for the management of H. pylori infection in Saudi Arabia. The working group recommends further studies to explore more therapeutic options to eradicate H. pylori.

Extramedullary Involvement of the Ascending Colon in Relapsing Acute Lymphocytic Leukemia: A Case Report.

Acute lymphoblastic leukemia (ALL) accounts for <1% of adult cancers. Extramedullary relapse of ALL has been primarily reported in pediatric patients or hematopoietic stem cell transplant recipients, and the gastrointestinal (GI) tract is a less frequently reported site of extramedullary relapse. Here, we report a case of a 30-year-old male who was a known case of ALL with multiple relapses and allogenic stem cell transplantations. The patient presented with acute lower GI bleeding and was confirmed to have an extramedullary relapse of ALL in the ascending colon. As the patient already had early relapses after two hematopoietic stem cell transplants in the past, he was managed with palliative chemotherapy, consisting of vincristine, dexamethasone, and rituximab, following which the patient achieved complete remission. This case highlights the importance of recognizing uncommon presentations of ALL such as those involving the GI tract.

Challenges Facing Undergraduate Medical Education in Ambulatory Care Clinics at Tertiary Care Hospitals.

Background: Medical education has been rapidly growing and transforming due to the enormous evolution of medicine. There have been many proficient ways to learn in medicine, but academic lectures, attending wards, and ambulatory care clinics (ACC) remain the three main ways of gaining clinical knowledge and experience for medical students. Over the past decade, there has been a dramatic shift in care by focusing on ambulatory care rather than inpatient settings, which provides a golden opportunity to reinforce medical education. Purpose: Most of the published studies that have focused on the teaching barriers in ACC were descriptive rather than analytic studies. Herein, we aim to detect and determine the barriers to teaching in ACC settings using qualitative analysis. Methods: This is a cross-sectional, observational study, involving medical students in their clerkship years (i.e., fourth and fifth) from two different medical colleges in Riyadh, Saudi Arabia. Faculty who are involved in undergraduate medical education in both colleges were also included. Main Results: A total of 387 medical students studying at the two universities were enrolled in the study. Most of the participants preferred attending outpatient clinics with consultants (44.2%) and the majority preferred attending internal medicine (IM) and IM subspecialties clinics (40.4%). Regarding the challenges, students believe the top three barriers are related to: faculty (39%), environment (34.8%), and patients (14.8%). Faculty on the other hand see that the top three barriers are related to environment (55.6%), patients (24.4%), and faculty (20%). Conclusion: Undergraduate medical education in outpatient settings has many challenges. In our study, the most significant challenges were COVID-19-related restrictions, patient refusal, and insufficient time for teaching. Future studies are needed to investigate these barriers and explore potential solutions that can decrease their burden on undergraduate medical education.

The cost-effectiveness of sequential versus standard triple therapy for Helicobacter pylori eradication in Saudi Arabia.

Background: The utilization rate of different treatment regimens for Helicobacter pylori infection is believed to be high; however, the cost-effectiveness of these regimens has not been examined before. Therefore, the aim of this study was to examine the cost-effectiveness of the two commonly prescribed treatments for H. pylori infection. Methods: The data of.an open-label, single-center, randomized trial that compared the efficacy of sequential therapy (SQT) (i.e., esomeprazole 20 mg twice daily for 10 days, amoxicillin 1000 mg twice daily for 5 days, then clarithromycin 500 mg and tinidazole 500 mg twice daily for 5 days) to standard triple therapy (STT) (i.e., esomeprazole 20 mg, amoxicillin 1000 mg, and clarithromycin 500 twice daily for 14 days) in the eradication of H. pylori, as confirmed by the negative urea breath test (UBT), were used. Propensity score matching bin bootstrapping, with 10,000 replications and bias correction was conducted to generate the 95% confidence limits. Moreover, probabilistic sensitivity analysis was conducted by varying both the eradication rates and the costs of treatment regimens. Results: There were 82 and 88 patients who were on SQT and STT, respectively. Patients' mean age was 47 years, and approximately 55% of them were females. The mean treatment costs were SAR 2,075.51 (USD 553.47) and SAR 2,629.26 (USD 701.14) for SQT and STT, respectively. The mean eradication rates for SQT and STT were 63.41% and 67.05%, respectively. The mean difference in costs and eradication rates for SQT versus STT were SAR - 550.75 (95% CI: -563.84- -537.69) and - 3.64% (95% CI: -6.98- 5.88). The use of SQT was more likely to be cost saving and more effective with 56.25% confidence level, in comparison to STT. Conclusion: The use of SQT in the treatment of H. pylori seems to be more cost-effective than STT.

Gastroduodenal amyloidosis: a case report and review of literature.

Amyloidosis is a disorder characterized by deposition of abnormally folded proteins in the extracellular space of various tissues and organs, possibly leading to their dysfunction. In the majority of cases, amyloidosis presents with systemic involvement including the gastrointestinal tract; however, localized gastroduodenal amyloidosis is rare. We report a case of gastroduodenal amyloidosis in a 36-year-old male with multiple comorbidities who presented with right upper quadrant abdominal pain. Reports of gastroduodenal amyloidosis and other relevant literature were also reviewed and discussed alongside this case.

Comprehensive multi-omics analysis of G6PC3 deficiency-related congenital neutropenia with inflammatory bowel disease.

Autosomal recessive mutations in G6PC3 cause isolated and syndromic congenital neutropenia which includes congenital heart disease and atypical inflammatory bowel disease (IBD). In a highly consanguineous pedigree with novel mutations in G6PC3 and MPL, we performed comprehensive multi-omics analyses. Structural analysis of variant G6PC3 and MPL proteins suggests a damaging effect. A distinct molecular cytokine profile (cytokinome) in the affected proband with IBD was detected. Liquid chromatography-mass spectrometry-based proteomics analysis of the G6PC3-deficient plasma samples identified 460 distinct proteins including 75 upregulated and 73 downregulated proteins. Specifically, the transcription factor GATA4 and LST1 were downregulated while platelet factor 4 (PF4) was upregulated. GATA4 and PF4 have been linked to congenital heart disease and IBD respectively, while LST1 may have perturbed a variety of essential cell functions as it is required for normal cell-cell communication. Together, these studies provide potentially novel insights into the pathogenesis of syndromic congenital G6PC3 deficiency.

Epidemiology of Pancreatic Cancer in Saudi Arabia: A Retrospective Analysis of Pancreatic Cancer Diagnosed in Saudi Arabia Between 2004 and 2015.

PURPOSE: Over the last decades, the incidence of pancreatic cancer has increased, particularly in countries with a higher socioeconomic status. The present work aimed to provide detailed epidemiological data on the incidence of pancreatic cancer in Saudi Arabia. PATIENTS AND METHODS: In this retrospective descriptive study, the epidemiological data on pancreatic cancer cases diagnosed in 13 administrative regions of Saudi Arabia between January 2004 and December 2015 were extracted from the Saudi Cancer Registry. The frequency, the crude incidence rate (CIR), and the age-standardized incidence rate (ASIR), stratified by geographical region, gender, and the year of diagnosis, were analyzed. RESULTS: From January 2004 to December 2015, a total of 2338 cases of pancreatic cancer were registered, including 1443 males and 895 females. The overall CIR was 1.28/100,000 among males and 0.80/100,000 in females, with an overall ASIR of 2.26 and 1.41/100,000 for males and females, respectively. Higher ASIR and CIR were observed among males than females (ratio 1.6). In both genders, the ASIR of pancreatic cancer increased with increasing age, with the highest incidence in patients aged 70 years or more. The ASIR in the Eastern Region (3.2/100,000) and the regions of Riyadh (3.0/100,000) and Tabuk (2.6/100,000) proved to be significantly higher than in the other regions of the country. Among women, the ASIR was significantly higher in Riyadh (2.3/100,000), the northern region (2.2/100,000), and Tabuk (2.0/100,000). CONCLUSION: This study revealed a slight increase of the CIR and ASIR of pancreatic cancer among males and females of the Saudi population. Eastern region, Riyadh, and Tabuk had the highest overall ASIRs of pancreatic cancer among males, Riyadh, Northern region, and Tabuk among Saudi females. The area least affected by pancreatic cancer was observed in Jazan among male and female Saudis. The rates of pancreatic cancer in Saudi Arabia were significantly higher among males compared with female Saudis. Further analytical studies are needed to identify the potential risk factors for pancreatic cancer among the Saudi population.

Large lipoma of the ascending colon: a case report and review of literature.

Gastrointestinal lipomas are a rare benign non-epithelial neoplasms derived from mature adipocytes. The colon is the commonest organ involved in the entire digestive tract and has an incidence rate ~4.4% in autopsy series. Most of the colonic lipomas are asymptomatic and incidentally detected. Lipomas need to be distinguished from true neoplasia, because in most cases they do not need to be resected unless when they cause a clear symptom or they are large in size. Surgical rather than endoscopic resection is preferred for lipomas > 2 cm to avoid complications such as bleeding and perforation. We report a case of a 56-year-old female, a known case of locally advanced breast cancer and positive adenomatous polyposis coli mutation, who was found to have 4 cm ascending colon lipoma by imaging and confirmed by colonoscopy and histopathology.

Brunner's glands hamartoma with pylorus obstruction: a case report and review of literature.

Proliferative lesions of the Brunner's glands are uncommonly encountered lesions of the small intestine, originating from the deeply seated mucosal and submucosal Brunner's glands, mainly in the duodenum. The vast majorities of these lesions are benign and include Brunner's glands hyperplasia (adenomas/nodules) and hamartomas. The etiology and pathogenesis of these lesions are not fully understood, and the diagnosis can sometimes be challenging. We report a case of Brunner's gland hamartoma in a 57-year-old man who presented with chronic dyspepsia, hematemesis and weight loss. Endoscopic and radiological investigations show a submucosal polypoid lesion at the first part of the duodenum. Routine endoscopic biopsies demonstrated normal duodenal mucosa. The lesion considered endoscopically unresectable and was surgically resected. Frozen section examination and intraoperative consultation showed unremarkable duodenal mucosa and histologically bland Brunner's glands.

The Value of Screening for Celiac Disease in Systemic Lupus Erythematosus: A Single Experience of a Tertiary Medical Center.

INTRODUCTION: Systemic lupus erythematosus (SLE) is a multi-organ inflammatory disease associated with autoimmune diseases. The aim of the study is to assessed the frequency of celiac disease (CD) in adults and children with SLE (aSLE and cSLE, respectively) and compare them with rheumatoid arthritis (RA) and juvenile idiopathic arthritis (JIA) patients; the study also explored the clinical impact of CD serologic markers on SLE disease activity and severity. METHODS: This was a cross-sectional study. Patients with SLE who had regular follow-up in rheumatology clinics were evaluated for laboratory and clinical variables using serology and the SLE Disease Activity Index (SLEDAI). To assess the occurrence of CD serology in cSLE and aSLE and the clinical impact of CD serologic markers on SLE, patients were tested for antigliadin (AGA), anti-endomysium (EmA) and anti-tissue transglutaminase (tTG) antibodies. RA and JIA patients were included for comparison. Duodenal biopsy was conducted in patients who exhibited CD markers. RESULTS: The CD marker was found in 29 (11.6%) of the 250 patients. AGA was present in seven aSLE patients and tTG in two (11.1%). Among cSLE patients, the autoantibody was present in 17.6% (AGA in four, tTG in two, and EmA in three). For RA patients, five had AGA and tTG and one had EmA, with an overall positivity of 9.7%. Five JIA patients had AGA (four with EmA and five with tTG) with overall positivity of 10.9%; the serum IgA level was normal in all patients except one. Duodenal endoscopic biopsy was performed in patients with positive CD markers (two declined). Histologic confirmation of CD was reported in one RA and one JIA patient but in none of the SLE patients. There was no correlation between the presence of CD markers and autoantibodies in SLE. CONCLUSION: CD antibodies did not influence SLE activity. Thus, SLE patients may not need to be screened for CD antibodies.

Development and validation of metric-based-training to proficiency simulation curriculum for upper gastrointestinal endoscopy using a novel assessment checklist.

BACKGROUND/AIMS: : This study aimed to design a structured simulation training curriculum for upper endoscopy and validate a new assessment checklist. MATERIALS AND METHODS: A proficiency-based progression stepwise curriculum was developed consisting of didactic, technical and non-technical components using a virtual reality simulator (VRS). It focused on: scope navigation, anatomical landmarks identification, mucosal inspection, retro-flexion, pathology identification, and targeting biopsy. A total of 5 experienced and 10 novice endoscopists were recruited. All participants performed each of the selected modules twice, and mean and median performance were compared between the two groups. Novices pre-set level of proficiency was set as 2 standard deviations below the mean of experts. Performance was assessed using multiple-choice questions for knowledge, while validated simulator parameters incorporated into a novel checklist; Simulation Endoscopic Skill Assessment Score (SESAS) were used for technical skills. RESULTS: : The following VRS outcome measures have shown expert vs novice baseline discriminative ability: total procedure time, number of attempts for esophageal intubation and time in red-out. All novice trainees achieved the preset level of proficiency by the end of training. There were no statistically significant differences between experts' and trainees' rate of complications, landmarks identification and patient discomfort. SESAS checklist showed high degree of agreement with the VRS metrices (kappa = 0.83) and the previously validated direct observation of procedural skills tool (kappa = 0.90). CONCLUSION: : The Fundamentals of Gastrointestinal Endoscopy simulation training curriculum and its SESAS global assessment tool have been primarily validated and can serve as a valuable addition to the gastroenterology fellowship programs. Follow up study of trainee performance in workplaces is recommended for consequences validation.

COVID-19 and endoscopy services in intermediately affected countries: a position statement from the saudi gastroenterology association.

With the global pandemic due to coronavirus disease 2019 (COVID-19), there has been a significant strain on healthcare facilities. The infectivity rate, as well as the rate of healthcare workers who have fallen ill to the disease, has raised concerns globally on the proper management of patients as well as the role of safe healthcare provision utilizing personal protective equipment (PPE). Furthermore, the limited supply of PPEs has mandated rationing their use to achieve maximum utility and preservation. Multiple gastroenterology associations have issued guidance and statements that would help healthcare providers in navigating these unprecedented and difficult times, and the Saudi Gastroenterology Association has provided this statement in an effort to bring the most up to date information for the management of endoscopy units in terms of resources, manpower planning, scheduling, as well as infection control policies and leadership.

Efficacy of a bismuth-based quadruple therapy regimen for Helicobacter pylori eradication in Saudi Arabia.

BACKGROUND/AIM: The treatment efficacy of Helicobacter pylori (H. pylori) has been decreasing over time due to resistance to multiple antimicrobial therapies. The most effective treatment regimen for Saudi Arabian patients infected with H. pylori is still unknown. We aimed to study the eradication rate of 10 days of quadruple therapy; bismuth subcitrate potassium 140 mg, metronidazole 125 mg, and tetracycline 125 mg for H. pylori infection in a Saudi population. PATIENTS AND METHODS: This was a prospective, open-label, non-randomized controlled trial. Patients with H. pylori infection were diagnosed by upper gastrointestinal (GI) endoscopy and rapid urease test (RUT) or histology. Patients who tested positive were recruited. Eligible patients were prescribed a 10-day course of quadruple therapy and received three capsules 4 times daily for 10 days along with omeprazole 20 mg twice daily. H. pylori was considered eradicated if the urea breath test (UBT) was negative after 6 weeks of completing the treatment. RESULTS: Ninety-two patients with H. pylori infection were recruited. Three patients withdrew from the trial and another seven patients lost follow-up. We analyzed 82 patient's data as per-protocol analysis, of whom 66 (80%) were naive to H. pylori treatment. Four patients had failed previous treatment with the sequential regimen and 12 patients had treatment with clarithromycin-based triple therapy. The post-treatment UBT for H. pylori infection was negative by per-protocol analysis in 72/82 patients (87.8%), and 72/92 (78.3%) by intention-to-treat analysis. There was no correlation between previous treatment failure and treatment response to the bismuth-based quadruple therapy (P value = 0.28). CONCLUSIONS: Treatment with a bismuth-based quadruple therapy was effective in eradicating H. pylori infection in 78.3% of Saudi patients with an ITT analysis and in 87.8% as per-protocol analysis.