Immunoglobulin G4 in eosinophilic esophagitis: Immune complex formation and correlation with disease activity.

Background: Recent studies have shown deposition of immunoglobulin G4 (IgG4) and food proteins in the esophageal mucosa of eosinophilic esophagitis (EoE) patients. Our aims were to assess whether co-localization of IgG4 and major cow's milk proteins (CMPs) was associated with EoE disease activity and to investigate the proteins enriched in proximity to IgG4 deposits.

Methods: This study included adult subjects with EoE (n = 13) and non-EoE controls (n = 5).

Persistent Diarrhea and Eosinophilic Esophagitis Resulting From Chronic Cannabidiol Usage for Refractory Epilepsy.

Cannabidiol is used in the care of treatment-resistant epilepsy. It has been associated with varying side effects, ranging from somnolence to diarrhea and weight loss. We present a patient on chronic cannabidiol therapy who had persistent diarrhea, abdominal pain, weight loss, and esophageal eosinophilia that improved with cannabidiol dose adjustment.

Food antigen consumption and disease activity affect food-specific IgG4 levels in patients with eosinophilic esophagitis (EoE).

Introduction: High levels of serum food-specific IgG4 (sIgG4) have been reported in patients with EoE. The objective of this study was to examine whether serum sIgG4 levels to foods and aeroallergens are higher in EoE patients than allergic controls and to investigate the association between sIgG4 and EoE clinical characteristics.

Methods: This was a case-control study nested in a prospective EoE Cohort.

Advancing Eosinophilic Esophagitis Diagnosis and Phenotype Assessment with Deep Learning Computer Vision.

Eosinophilic Esophagitis (EoE) is an inflammatory esophageal disease which is increasing in prevalence. The diagnostic gold-standard involves manual review of a patient's biopsy tissue sample by a clinical pathologist for the presence of 15 or greater eosinophils within a single high-power field (400× magnification). Diagnosing EoE can be a cumbersome process with added difficulty for assessing the severity and progression of disease.

Specific IgG antibodies to cow's milk proteins in pediatric patients with eosinophilic esophagitis.

Background: Allergen-specific IgG (sIgG) antibodies are often associated with tolerance, but sIgG antibodies to causally relevant foods have been reported recently in adults with eosinophilic esophagitis (EoE). Prevalence and levels of food sIgG are not well established in the general pediatric population.

Objective: We sought to investigate serum food sIgG with component diagnostics in children with EoE and children from an unselected birth cohort and to explore the effects of sex, age, and milk consumption on sIgG levels.

Gain-of-function mutations in SMAD4 cause a distinctive repertoire of cardiovascular phenotypes in patients with Myhre syndrome.

Myhre syndrome is a rare, distinctive syndrome due to specific gain-of-function mutations in SMAD4. The characteristic phenotype includes short stature, dysmorphic facial features, hearing loss, laryngotracheal anomalies, arthropathy, radiographic defects, intellectual disability, and a more recently appreciated spectrum of cardiovascular defects with a striking fibroproliferative response to surgical intervention. We report four newly described patients with typical features of Myhre syndrome who had (i) a mildly narrow descending aorta and restrictive cardiomyopathy; (ii) recurrent pericardial and pleural effusions; (iii) a large persistent ductus arteriosus with juxtaductal aortic coarctation; and (iv) restrictive pericardial disease requiring pericardiectomy.

Ethnic differences in the link between insulin resistance and elevated ALT.

Background: Nonalcoholic fatty liver disease (NAFLD) exhibits tight links with insulin resistance (IR) and the metabolic syndrome (MetS), a cluster of cardiovascular risk factors. Compared with non-Hispanic whites, non-Hispanic black adolescents have more IR but a lower prevalence of NAFLD and MetS. Our hypothesis was that IR would be a better predictor of alanine aminotransferase (ALT) elevations than is MetS among non-Hispanic blacks.

Changes in inflammation and QoL after a single dose of infliximab during ongoing IBD treatment.

Background And Objectives: Infliximab is used increasingly as maintenance therapy for inflammatory bowel disease (IBD); however, the effects of a single maintenance dose of infliximab are unclear with respect to the quality of life and hormones related to growth and puberty. The aim of the present study was to determine the time course of inflammatory, hormonal, and quality-of-life changes following a single dose of infliximab in the context of ongoing therapy, as related to presence of IBD symptoms at time of administration.

Methods: Children and adolescents with IBD receiving ongoing therapy with infliximab for clinical indications were recruited.

Cholangiocytes as immune modulators in rotavirus-induced murine biliary atresia.

Background/aims: Biliary atresia (BA) is a progressive disease characterized by bile duct inflammation and fibrosis. The aetiology is unknown and may be due to a virus-induced, autoimmune-mediated injury of cholangiocytes. Cholangiocytes are not only targets of injury but may also modulate hepatic inflammation.

Discordant erythrocyte sedimentation rate and C-reactive protein in children with inflammatory bowel disease taking azathioprine or 6-mercaptopurine.

Background: Inflammatory bowel disease (IBD) is characterized by periods of relapse and remission. Treatment is aimed at reducing symptoms during relapse and prolonging the duration of remissions. 6-Mercaptopurine (6-MP) and its prodrug azathioprine (AZA) are commonly used to prolong clinical remissions.