Response to SARS-CoV-2 initial series and additional dose vaccine in pediatric patients with predominantly antibody deficiency.

Data regarding response to SARS-CoV-2 immunization in pediatric patients with predominantly antibody deficiency (PAD) is limited. We evaluated SARS-CoV-2 immunization response by anti-SARS-CoV-2-spike antibody level in 15 pediatric PAD patients. These data were compared to a published cohort of adult PAD patients (n=62) previously analyzed following SARS-CoV-2 immunization at our single center institution.

Benralizumab for eosinophilic gastritis: a single-site, randomised, double-blind, placebo-controlled, phase 2 trial.

Background: In eosinophilic gastrointestinal diseases, the role of eosinophils in disease pathogenesis and the effect of eosinophil depletion on patient outcomes are unclear. Benralizumab, an eosinophil-depleting monoclonal antibody that targets the interleukin-5 receptor α, might eliminate gastric tissue eosinophils and improve outcomes in eosinophilic gastritis. We aimed to assess the efficacy and safety of benralizumab in patients with eosinophilic gastritis.

Diagnosis and Management of Pediatric Hypereosinophilic Syndrome.

Hypereosinophilic syndrome (HES) is a diverse group of disorders characterized by peripheral blood eosinophilia of 1.5 × 10/L (1,500/μL) or greater with evidence of end-organ damage attributable to eosinophilia and no other cause of the end-organ damage. The HES is rare, especially in children.

Approach to Eosinophilia Presenting With Pulmonary Symptoms.

Eosinophilia with pulmonary involvement is characterized by the presence of peripheral blood eosinophilia, typically ≥500 cells/mm, by pulmonary symptoms and physical examination findings that are nonspecific, and by radiographic evidence of pulmonary disease and is further supported by histopathologic evidence of tissue eosinophilia in a lung or pleura biopsy specimen and/or increased eosinophils in BAL fluid, usually >10%. Considering that there are a variety of underlying causes of eosinophilia with pulmonary manifestations and overlapping clinical, laboratory, and radiologic features, it is essential to approach the evaluation of eosinophilia with pulmonary findings systematically. In this review, we will describe a case presentation and discuss the differential diagnosis, a directed approach to the diagnostic evaluation and supporting literature, the current treatment strategies for pulmonary eosinophilia syndromes, and the levels of evidence underlying the recommendations, where available.

Prenatal and perinatal history in Kabuki Syndrome.

Kabuki syndrome (KS) is a disorder of epigenetic dysregulation due to heterozygous mutations in KMT2D or KDM6A, genes encoding a lysine-specific methyltransferase or demethylase, respectively. The phenotype is highly variable, including congenital cardiac and renal anomalies, developmental delay, hypotonia, failure to thrive, short stature, and immune dysfunction. All affected individuals have characteristic facial features.

Pediatric Hypereosinophilia: Characteristics, Clinical Manifestations, and Diagnoses.

Background: Eosinophilia is associated with various conditions, including allergic, infectious, and neoplastic disorders. The diagnostic differential is broad, and data on hypereosinophilia in pediatric patients are limited.

Objective: The objectives of this study were to identify cases of hypereosinophilia in a tertiary pediatric medical center, determine clinical characteristics and disease associations, and estimate the incidence of hypereosinophilia in the hospital and geographic populations.

Prevalence of eosinophilic colitis and the diagnoses associated with colonic eosinophilia.

Pediatric eosinophilic colitis (EC) is a rare disease with a prevalence of ~1/63,000 individuals, and at our tertiary care pediatric medical center, only 2% of patients with the ICD-9/10 code for EC likely have the disease.