Mucosa-Associated Lymphoid Tissue Lymphoma of the Lacrimal Gland: Sustained Remission after Eradication of Helicobacter Pylori Infection

Mucosa-associated lymphoid tissue (MALT) lymphoma is the third most common non-Hodgkin lymphoma, and it is strongly associated with helicobacter pylori infection of the stomach. MALT lymphoma of the lacrimal gland usually presents as a localized disease process in extranodal tissues. The treatment options of MALT lymphoma of the lacrimal gland chiefly include radiation of the tumor, chemotherapy, surgical removal, or a combination of these strategies. We report a case of localized MALT lymphoma of the lacrimal gland, with prolonged sustained remission after eradication of gastric Helicobacter pylori (H. Pylori) infection. He sustains in remission of lacrimal MALT lymphoma for four years without chemotherapy or radiotherapy

Congenital short bowel syndrome: A case report and review of the literature

Congenital short bowel syndrome (SBS) is a rare condition of the newborn, with several reports demonstrating high mortality. A six-week-old boy presented with chronic diarrhea and failure to thrive. An upper gastrointestinal endoscopy showed a straight duodenum, and multiple small bowel biopsies were histologically normal. An upper gastrointestinal series showed malrotation. At laparotomy, the small bowel was 50 cm in length, confirming the diagnosis of congenital SBS. Parenteral nutrition was initiated and enteral feeding with an amino acid-based formula containing long-chain fatty acids was introduced early and gradually advanced. At the last follow-up examination at 24 months, he was thriving on a regular diet, with normal growth and development. Long-term survival of children with congenital SBS is now possible if enteral feeds are introduced early to promote intestinal adaptation, with subsequent weaning off parenteral nutrition

Knowledge and practice of pediatricians regarding childhood constipation in the Arab world: results from a multicenter survey

Objectives We aimed to evaluate knowledge and practice styles among medical providers with different professions and working in different Arab countries regarding their approach to childhood constipation. Methods We conducted a cross-sectional multinational survey in eight Arab countries. Pediatric care providers (PCPs), including pediatric specialists (PSs), pediatric residents (PRs), pediatric consultants (PCs), pediatric gastroenterologists (PGs), general practitioners (GPs), and pediatric surgeons (PSu), were included in our study. The survey was anonymous, and participants provided input on the definition and management of constipation. Results Of 4000 PCPs, 2579 completed the survey (response rate of 64.5%). Although the majority of respondents were aware of the Rome IV criteria to diagnose constipation, significant differences in the percentage of participants in different geographic countries regarding the definition of constipation were noted. Polyethylene glycol (PEG) was prescribed as a first-line treatment of fecal disimpaction significantly more frequently by pediatricians (PSs, PRs, PCs, PGs) than GPs and PSu (12.8% versus 7.2%, respectively; p < 0.001). Additionally, we found that pediatricians used special milk (high magnesium) as a first-choice formula significantly more often than other physicians (53.7% versus 37%, P < 0.001). For maintenance therapy, both pediatricians and nonpediatricians used dietary management with a special milk formula more than other treatment options (84.2% and 84%, respectively). Conclusions Despite increased awareness of the Rome IV criteria, significant differences in knowledge and practice patterns regarding fecal disimpaction exist among PCPs from different Arab countries. The identification of these gaps may be helpful for policy-makers to produce targeted instructional resources on constipation for PCPs.Medicine, Faculty ofNon UBCGastroenterology, Division ofMedicine, Department ofReviewedFacultyResearche

Neuroblastoma amplified sequence gene mutation: A rare cause of recurrent liver failure in children

Neuroblastoma amplified sequence (NBAS) gene mutation or infantile liver failure syndrome type 2 (ILFS type 2) is an extremely rare disease characterized by episodic liver failure precipitated by intercurrent febrile illness, and liver function recovering completely. Here, we report a 4-year-old girl with recurrent hepatitis. A diagnosis of ILFS type 2 was made based on NBAS mutation gene found by whole-exome sequencing. Our case provides a new insight toward considering NBAS mutation as a part of the differential diagnoses of any infant presenting with recurrent liver failure or hepatitis. We recommend sequencing NBAS in cases of recurrent hepatitis in infancy of unknown cause, especially in individuals with fever-associated hepatic dysfunction

Characteristics of Pediatric Crohn’s Disease in Saudi Children: A Multicenter National Study

Background and Aims. Crohn’s disease (CD) is an evolving disease in KSA. Little is known about its characteristics in the Saudi population. The aims of this study were to describe the characteristics of Saudi children with CD and to determine whether the characteristics of CD in KSA are different from those seen in Western countries. Methods. In this study, children younger than eighteen years of age diagnosed with CD between January 2003 and December 2012 were included. Results. Of 330 patients identified, 186 (56.4%) were males. The median age at diagnosis was 15.8 years. A positive family history for IBD in first-degree relatives occurred in 13.6% of patients. The most common symptoms were abdominal pain (84.2%), weight loss (75.2%), and diarrhea (71.8%). The main disease location was ileocolonic (42.1%) and the main disease behavior was nonstricturing and nonpenetrating (63.6%). Perianal involvement was seen in 60 (18.2%) patients. Laboratory findings revealed anemia in 57.9% of patients, low albumin in 34.5%, and high CRP in 39.4%. Conclusions. Saudi children with CD have lower frequency of first-degree relatives with IBD, lower prevalence of early onset disease, longer diagnostic delay, higher prevalence of growth failure, and greater frequency of stricturing and penetrating disease behavior compared to Western patients

Environmental Risk Factors for Childhood Inflammatory Bowel Diseases: A Multicenter Case-Control Study

Objective: Multiple environmental factors can be linked to the development of inflammatory bowel disease (IBD).With an increase in the cases of IBD, the objective of this research is to investigate environmental risk factors for IBD in the Saudi population. Methods: A retrospective multicenter case&ndash;control study was performed among IBD children from 2009 to 2021.The variables analyzed to be the possible risk factors included their socioeconomic status, living and demographic characteristics, and lifestyle related to IBD. The questionnaire included a list of IBD risk factors that was given to the control and the patient group. For every variable, the 95% confidence interval (CI) and odds rations were also estimated. Results: There were 335 individuals considered in this study: 168 controls (50.1%) and 167 IBD patients (49.9%). Of these, 93 IBD patients (56%) had CD and 74 patients (44%) had UC. Most of participants were female (72.1%) and were aged above 10 years (51.5%). Vaginal delivery (OR 0.551, 95% CI: 1.59&ndash;4.14), age above 10 years (OR 1.040, 95% CI: 1.012&ndash;1.069), deficient fruit intake (OR 2.572, 95% CI: 1.59&ndash;4.14), no exposure to antibiotics (OR 2.396, 95% CI: 1.51&ndash;3.81), appendectomy (OR 2.098, 95% CI: 1.87&ndash;2.35), less physical activity (OR 2.033, 95% CI: 1.05&ndash;3.93) and gastroenteritis admissions &gt; 2 times/year (OR 0.107, 95% CI: 0.037&ndash;0.311) were the risk factors for IBD. These factors depicted a more significant link with CD than UC (p &lt; 0.05). Interestingly, sleep disturbance was estimated to be a CD risk factor (adjusted OR: 3.291, 95% CI = 0.97&ndash;11.22). Pets in house was risk factor for UC (p &lt; 0.001). Conclusions: This study highlights association between vaginal delivery, age above 10 years, deficient fruit intake, low physical activity, exposure to antibiotics, appendectomy, and frequent gastroenteritis admissions as risk factors for IBD. Knowledge of these risk factors can help pediatricians to prospectively identify patients at risk of environmental exposure

IgG4-related sclerosing mesenteritis in a 7-year-old Saudi Girl

Sclerosing mesenteritis (SM) is a rare, benign inflammatory disorder of unknown etiology, affecting the membranes of the digestive tract that involves lymphoplasmacytic inflammation, fat necrosis, and fibrosis of the mesentery. We report a child patient with a history of recurrent abdominal pain and fever who was found to have an intra-abdominal mass suspicious for malignancy. A tissue biopsy revealed the diagnosis of SM associated with IgG4-related systemic disease. The patient is currently maintained on 5 mg prednisone daily and no recurrence of symptoms was noted during the 24-month follow-up period. We emphasize, therefore, that SM can present clinical challenges and the presence of SM should cue clinicians to search for other coexisting autoimmune disorders that can have various outcomes

Reported Prevalence and Nutritional Management of Functional Constipation among Young Children from Healthcare Professionals in Eight Countries across Asia, Europe and Latin America

Background: The prevalence of functional constipation (FC) among children varies widely. A survey among healthcare professionals (HCPs) was conducted to better understand the HCP-reported prevalence and (nutritional) management of FC in children 12–36 months old. Methods: An anonymous e-survey using SurveyMonkey was disseminated via emails or WhatsApp among HCPs in eight countries/regions. Results: Data from 2199 respondents were analyzed. The majority of the respondents (65.9%) were from Russia, followed by other countries (Indonesia (11.0%), Malaysia (6.0%)), Mexico, KSA (5.1% (5.7%), Turkey (3.0%), Hong Kong (2.2%), Singapore (1.1%)). In total, 80% of the respondents (n = 1759) were pediatricians. The prevalence of FC in toddlers was reported at less than 5% by 43% of the respondents. Overall, 40% of the respondents reported using ROME IV criteria in > 70% of the cases to diagnose FC, while 11% never uses Rome IV. History of painful defecation and defecations < 2 x/week are the two most important criteria for diagnosing FC. In total, 33% of the respondents reported changing the standard formula to a specific nutritional solution, accompanied by parental reassurance. Conclusion: The most reported prevalence of FC in toddlers in this survey was less than five percent. ROME IV criteria are frequently used for establishing the diagnosis. Nutritional management is preferred over pharmacological treatment in managing FC