Wheat-Triggered Food Protein-Induced Enterocolitis Syndrome in Celiac Children on Gluten-Free Diet: A New Clinical Association.

Introduction: The association between food protein-induced enterocolitis syndrome (FPIES) and wheat ingestion in children with celiac disease is unknown at this time.

Methods: We present seven cases of children with celiac disease who presented with symptoms of wheat-triggered acute FPIES (a-FPIES). An oral food challenge (OFC) with wheat allergen followed by 4 h of observation was performed.

Assessment of Duodenal Intraepithelial Lymphocyte Composition (Lymphogram) for Accurate and Prompt Diagnosis of Celiac Disease in Pediatric Patients.

Introduction: Quantitative and phenotypic analyses of duodenal intraepithelial lymphocytes (IELs) by flow cytometry (IEL lymphogram) confer specificity and enable the diagnosis even in unconventional presentations of celiac disease (CD). To evaluate the validity of the IEL lymphograms in the pediatric population for new insights into their use as biomarkers in the natural history of CD.

Methods: We retrospectively included 1,211 children (602 with active CD, 92 on a gluten-free diet, 47 with potential CD, and 470 nonceliac controls) who required duodenal biopsies in this study.

Effectiveness of allergy testing in milk induced eosinophilic esophagitis. Description and follow-up of patients.

Introduction: Eosinophilic esophagitis (EoE) is a chronic, local immune-mediated esophageal disease that has been on the increase lately. There is currently enough evidence to conclude that EoE is an allergic disorder triggered by food allergens, with cow's milk (CM) being the most frequent. Dietary intervention is the first-line approach.

An Activating Mutation in Results in Neonatal Diabetes Through Reduced Insulin Synthesis.

Neonatal diabetes mellitus (NDM) is a rare form of diabetes diagnosed within the first 6 months of life. Genetic studies have allowed the identification of several genes linked to the development of NDM; however, genetic causes for ∼20% of the cases remain to be clarified. Most cases of NDM involve isolated diabetes, but sometimes NDM appears in association with other pathological conditions, including autoimmune diseases.

Distal duodenum versus duodenal bulb: intraepithelial lymphocytes have something to say in celiac disease diagnosis.

Background And Aim: After clinical screening and the serological test, many patients still require a duodenal biopsy for celiac disease diagnosis. Mild histological lesions, unspecific findings and patchiness are frequent outcomes of this mandatory diagnostic tool, thus complicating clinical decisions.

Methods: We analyzed the lymphoid components [number of total intraepithelial lymphocytes (IELs), TcR-γδ and CD3(-)IELs] of the duodenal epithelium by flow cytometry in samples obtained from bulb and distal duodenum during upper gastrointestinal endoscopies performed for diagnostic purposes.

A molecular risk score based on 4 functional pathways for advanced classical Hodgkin lymphoma.

Despite improvement in the treatment of advanced classical Hodgkin lymphoma, approximately 30% of patients relapse or die as result of the disease. Current predictive systems, determined by clinical and analytical parameters, fail to identify these high-risk patients accurately. We took a multistep approach to design a quantitative reverse-transcription polymerase chain reaction assay to be applied to routine formalin-fixed paraffin-embedded samples, integrating genes expressed by the tumor cells and their microenvironment.

Consumer trust to a Web site: moderating effect of attitudes toward online shopping.

In this paper, authors suggest a model that reflects the role played by the Web site characteristics and the previous level of satisfaction as determinant factors of trust in the Web site. Also, authors consider the moderating effects of consumers' motives and inhibitors to purchase online. Results show that satisfaction with previous purchases, the Web site security and privacy policies, and service quality are the main determinants of trust.

Usefulness of penicillamine-stimulated urinary copper excretion in the diagnosis of adult Wilson's disease.

Objective: Diagnosis of Wilson's disease (WD) is reliant on liver biopsy (LB) and measurement of hepatic copper. The aim of this study was to determine the usefulness of penicillamine-stimulated urinary copper excretion (PS-UCE), a non-invasive diagnostic test, for the diagnosis of WD in adults.

Material And Methods: In this prospective study of patients with suspected WD, total serum copper, ceruloplasmin, basal 24-h UCE and PS-UCE levels were measured.

Hepatitis C virus infection acquired in childhood.

Hepatitis C virus (HCV) infection occurs less frequently in children than in adult patients, and the natural history, prognosis, and clinical significance of HCV infection in children are poorly defined. We report here a descriptive follow-up of the clinical course, biochemical data, and viral markers observed in 37 children with anti-HCV. Ten patients included in the study tested persistently negative for serum HCV-RNA (group 1) and 27 patients tested persistently positive (group 2).

Age-related variation of intraepithelial lymphocytes subsets in normal human duodenal mucosa.

The enumeration of intestinal intraepithelial lymphocytes (IELs), and the phenotyping of CD3+CD 103+ (TcRalphabeta, TcRgammadelta) and CD3-CD103+ IEL subsets constitute useful diagnostic tools for the correct interpretation of the mucosal histology of duodenal/jejunal biopsies in many pathological conditions of the small intestine, particularly celiac disease (CD). This work evaluates the ranges of duodenal IEL counts by flow cytometry in healthy mucosa from pediatric and adult controls, establishing normal reference values for CD3+ TcRgammadelta and CD3- subsets and their variation with age. Seventy-four pediatric controls and 36 adult controls were identified on the basis of their normal histology from more than 1,000 duodenal diagnostic biopsies performed in Caucasian subjects.

Cytokine production by intestinal intraepithelial lymphocyte subsets in celiac disease.

One of the earliest signs of mucosal immune activation in celiac disease (CD) is an increase in the intraepithelial lymphocyte (IEL) count in the small intestinal epithelium. Though most of those IELs express T cell receptor (TcR)-alphabeta chains, CD is characterized by an increase in TcR-gammadelta+ IELs and by the loss of CD3- IELs. There is currently little evidence that these changes in IEL subset distribution are of relevance in the pathogenesis of CD.

Human small-intestinal epithelium contains functional natural killer lymphocytes.

Background & Aims: CD3(-) non-T lymphocytes constitute the second most abundant lymphoid subset in the human small-bowel epithelium, and these CD3(-) intraepithelial lymphocytes are virtually absent in active celiac disease. Phenotypically, they resemble natural killer cells and have been termed natural killer-like intraepithelial lymphocytes. Because of the limited availability of appropriate human samples, functional studies have not yet been reported, and it is not yet clear whether these are true natural killer cells.

Anti-transglutaminase IgA ELISA: clinical potential and drawbacks in celiac disease diagnosis.

Background: Since the identification of tissue transglutaminase (tTG) as the antigen for the anti-endomysial antibodies (EMA), several antigen-specific immunoassays have been reported for celiac disease (CD) screening. A first objective was to evaluate the suitability for CD screening of three different IgA tTG ELISAs, two of them based on guinea pig liver tTG (gp-tTG) (an in-house ELISA with a partially purified extract and a commercial ELISA with purified gp-tTG antigen) and a third recombinant human tTG (rh-tTG) ELISA. The results are compared with EMA and with the final clinical diagnosis.

Intestinal intraepithelial lymphocytes contain a CD3- CD7+ subset expressing natural killer markers and a singular pattern of adhesion molecules.

Intestinal intraepithelial lymphocytes (i-IEL) represent one of the largest, non-organized lymphoid population in the body. They are located outside the epithelial basement membrane among the mucosal epithelial cells. We, and previously other groups, have reported the presence of a CD7+CD3-IEL subset in the epithelium of human small intestine.

Intraepithelial lymphocytes and coeliac disease: permanent changes in CD3-/CD7+ and T cell receptor gammadelta subsets studied by flow cytometry.

Unlabelled: Permanent changes in intestinal intraepithelial lymphocytes have been observed in coeliac patients. The aim of this investigation was to study small intestinal intraepithelial lymphocytes by using flow cytometry and to evaluate its diagnostic value in coeliac disease. Three-colour flow cytometry analyses were performed on isolated epithelial cells of 117 intestinal biopsies obtained from 113 children (54 coeliac disease, 4 other enteropathies, 18 Helicobacter pylori associated gastritis and 37 normal controls).

Hepatitis E virus: relevance in blood donors and risk groups.

Background And Objectives: Hepatitis E virus (HEV) infection usually causes acute self-limited disease. HEV is associated with faecal-contaminated drinking water, but other vectors, such as blood, are possible. The aim of this study was to investigate the prevalence of HEV in blood donors and in two groups at high risk for parenteral infections, namely, haemodialysis patients, and children infected with HCV via blood transfusion.