Plasma Extracellular Vesicles Derived from Pediatric COVID-19 Patients Modulate Monocyte and T Cell Immune Responses Based on Disease Severity.

Background: The COVID-19 pandemic has caused significant morbidity and mortality globally. The role of plasma-derived extracellular vesicles (EVs) in pediatric COVID-19 patients remains unclear.

Methods: We isolated EVs from healthy controls (n = 13) and pediatric COVID-19 patients (n = 104) with varying severity during acute and convalescent phases using serial ultracentrifugation.

Phenotypes of persistent hen`s egg allergy in children and adolescents.

Background: Optimum management of food allergy (FA) includes consideration of co-allergies and multimorbidities and tolerance assessment. Documentation of FA practices may pave the way for better practice.

Methods: Patients aged 3-18 years, with persistent IgE-mediated hen`s egg allergy were reviewed.

A single-center study points to diverse features and outcome in patients with Hyperimmunoglobulin M Syndrome and Class- Switch Recombination defects.

Isotype class-switch recombination (CSR), somatic hypermutation (SHM), B cell signalling and DNA repair mechanisms defects are responsible for high IgM. The hyperimmunoglobulin M (HIGM) phenotype and CSR-related defects are now classified under primary antibody defects, combined immunodeficiencies or syndromic immunodeficiencies groups. The aim of the study is to evaluate the diverse phenotypic/genotypic/laboratory characteristics and outcome of patients with CSR defects and HIGM-related defects.

Exploring the Heterogeneity of IgE-Mediated Food Allergy through Latent Class Analysis.

Introduction: Food allergy (FA) is a heterogeneous disease with multiple morbidities and a huge burden for patients and healthcare systems. Variable manifestations, comorbidities (atopic dermatitis [AD], asthma, and/or allergic rhinitis [AR]), severity (anaphylaxis), and outcomes suggest the existence of different endotypes that cluster analyses may reveal. In this study, we aimed to investigate distinct subgroups among patients with FAs using data from 524 children/adolescents.

Safety and efficacy of rapid drug desensitization in children.

Background: Any drug taken at the recommended dosage may cause hypersensitivity reactions (DHR). Rapid drug desensitization (RDD) protocols have been developed in the case of a confirmed or highly suspected HSR to allow safe administration of the medicine when there is no alternative drug or in the presence of a less effective or more toxic alternative. The aim of this study was to evaluate the characteristics of children who underwent desensitization, the safety and efficacy of RDD in children, as well as, the characteristics and management of breakthrough reactions.

Oral Immunotherapy in Food Allergy: A Critical Pediatric Perspective.

There is evidence that in children with persistent IgE-mediated food allergy (FA) to cow's milk, hen's egg, and peanut, oral allergen-specific immunotherapy (OIT) may increase the reaction threshold to the culprit food allergen(s). OIT may protect patients from the occurrence of severe reactions in case of accidental ingestion of the culprit food during treatment. Notwithstanding, many gaps are still unsolved, including safety issues, identification of predictive biomarkers, and post-desensitization efficacy.

Multiplex component-based allergen macroarray test is useful to predict clinical reactivity to tree nuts in children.

Background: In tree nut (TN) allergy, singleplex tests showed the diagnostic utility of rAna o 3, rCor a 14/nCor a 9, and nJug r 1/nJug r 4 for cashew/pistachio, hazelnut, and walnut allergies, respectively. However, disadvantages of the tests include high costs and excessive blood sampling in multi-sensitized patients, and a limited number of components. We investigated the utility of a multiplex macroarray (i.

From Allergen Molecules to Molecular Immunotherapy of Nut Allergy: A Hard Nut to Crack.

Peanuts and tree nuts are two of the most common elicitors of immunoglobulin E (IgE)-mediated food allergy. Nut allergy is frequently associated with systemic reactions and can lead to potentially life-threatening respiratory and circulatory symptoms. Furthermore, nut allergy usually persists throughout life.

IgE mediated food allergy in Turkey: different spectrum, similar outcome.

Background: Food allergies (FAs) potentially differ across cultures.

Methods: All medical records of 534 children and adolescents with IgE-mediated FA over a 5-year period were reviewed to document the regional characteristics with regard to spectrum and outcome.

Results: According to their last visit, the most common FAs were tree nuts (TNs) (52.

Food protein-induced enterocolitis syndrome: Current practices in oral food challenge.

Oral food challenges (OFC) in food protein-induced enterocolitis syndrome (FPIES) are performed to confirm a diagnosis, test for development of tolerance, and find safe alternatives. We aimed to define OFC outcomes and identify safer test strategies. OFCs performed in children with FPIES over a 5-year period were reviewed.

Health-related quality of life in children with hereditary angioedema compared with patients with histaminergic angioedema.

Hereditary angioedema (HAE) is a potentially life-threatening disease that remarkably impacts patients' health-related quality of life (HRQoL). This study evaluated the HRQoL in children with HAE and compared it with healthy controls and patients with histaminergic angioedema (HA). Fifty-nine children with HAE (median [interquartile range {IQR}], ages, 8.

Oral food challenge in IgE mediated food allergy in eastern Mediterranean children.

Background: The oral food challenge (OFC) in IgE mediated food allergy causes anxiety both in parents and in patients due to its inherent risks.

Objective: Documentation of the rate, spectrum, and predictors of positive reactions is instructive.

Methods: Children, who underwent OFC between January 1, 2017 and December 31, 2019 were analyzed.

Phenotypes and natural history of food protein-induced enterocolitis syndrome in the east Mediterranean region.

Food protein-induced enterocolitis syndrome (FPIES) is a rare non-IgE mediated food allergy. To delineate the differences in the spectrum of culprit foods, remission patterns, and predictors among varying cultures. We reviewed demographics, culprit foods, outcomes, and predictors in 81 children with a diagnosis of FPIES who were followed up between 2015 and 2020.

IgE-mediated food allergy throughout life.

Food allergy (FA) has become an increasing problem throughout the world. Over the last 2 decades, the frequency of FA has increased in both children and adults. The prevalence differs according to the research methodology, age, and geographic regions, ranging between 2.

Food-induced anaphylaxis in infants, as compared to toddlers and preschool children in Turkey.

Background: The literature includes scarce data on infants with food-induced anaphylaxis (FIA).

Materials And Methods: Medical records of the patients diagnosed with FIA aged 0-6 years between 2015 and 2020 were retrospectively analyzed.

Results: During the study period, there were 451 instances of FIA in 314 patients, of which 175 (38.

Hypersensitivity Reactions to Biologicals: from Bench to Bedside.

Purpose Of Review: Biologic agents are new treatment options for chronic inflammatory diseases and cancers. As a result of their unique mechanism of action, they are more effective and less toxic treatment option and their clinical usage is increasing. While they are more commonly used, various adverse effects have been observed including life-threatening ones including anaphylaxis.

Visceral fat index: a novel predictor for coronary collateral circulation.

Objective This study was designed to investigate the role of visceral adiposity along with other clinical parameters in predicting poor coronary collateral circulation (CCC) among patients with severe obstructive coronary artery disease (CAD). Subjects and methods A total of 135 patients with severe obstructive CAD and good (n = 70) or poor (n = 65) CCC were included. Data on angiographically detected CCC, the quality criteria for CCC (Rentrop scores) and visceral fat index (VFI) obtained via bioelectrical impedance were compared between good and poor CCC groups.

Identification of two novel PNPLA1 mutations in Turkish families with autosomal recessive congenital ichthyosis.

Dökmeci-Emre S, Taşkıran ZE, Yüzbaşıoğlu A, Önal G, Akarsu AN, Karaduman A, Özgüç M. Identification of two novel PNPLA1 mutations in Turkish families with autosomal recessive congenital ichthyosis. Turk J Pediatr 2017; 59: 475-482.

Identification of KIF21A mutations as a rare cause of congenital fibrosis of the extraocular muscles type 3 (CFEOM3).

Purpose: Three congenital fibrosis of the extraocular muscles phenotypes (CFEOM1-3) have been identified. Each represents a specific form of paralytic strabismus characterized by congenital restrictive ophthalmoplegia, often with accompanying ptosis. It has been demonstrated that CFEOM1 results from mutations in KIF21A and CFEOM2 from mutations in PHOX2A.

Is vitamin D hypothesis for schizophrenia valid? Independent segregation of psychosis in a family with vitamin-D-dependent rickets type IIA.

The vitamin D hypothesis of schizophrenia is a recent concept bringing together old observations on environmental risk factors and new findings on the neurodevelopmental effects of vitamin D. Candidate genes related to the vitamin D endocrine system have not yet been fully explored for this purpose. The coexistence of vitamin-D-dependent-rickets type II with alopecia (VDDR IIA) and different forms of psychosis in the same inbred family has provided us with an opportunity to investigate the presumed relationship between vitamin D deficiency and psychosis.

Heterozygous mutations of the kinesin KIF21A in congenital fibrosis of the extraocular muscles type 1 (CFEOM1).

Congenital fibrosis of the extraocular muscles type 1 (CFEOM1; OMIM #135700) is an autosomal dominant strabismus disorder associated with defects of the oculomotor nerve. We show that individuals with CFEOM1 harbor heterozygous missense mutations in a kinesin motor protein encoded by KIF21A. We identified six different mutations in 44 of 45 probands.