Pediatric Allergy Immunology and Pulmonology最新文献

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a severe adverse drug-induced hypersensitivity reaction. We report a case of DRESS syndrome in a 17-year-old female caused by itraconazole, confirmed by patch testing, that required treatment with both corticotherapy and cyclosporine. Our case highlights the importance of clinical suspicion of this syndrome in pediatric age and the novelty of an antifungal drug being identified as the culprit.

Background: In Chile, patients with hereditary angioedema (HAE) type I and type II are protected under Ley Ricarte Soto (LRS), which guarantees access to on demand plasma-derived C1-INH (pdC1-INH) since 2018. We aimed to analyze the first 3 years of LRS. Methods: Review of the LRS database between 2018 and 2021. Results: During the study period, 154 patients were covered by LRS, with an estimated prevalence of HAE in Chile at 0.8:100,000 inhabitants. A delay in diagnosis of 22 years was noted, 50 patients received epinephrine during an attack before the diagnosis of HAE. Mean number of attacks per year was 8, with 50% of adults and 42% of children experiencing more than 1 attack per month. Conclusion: Disease awareness must improve to reduce the diagnostic delay of HAE. Long-term prophylactic medications should be included in LRS to treat patients with high attack rates and control the costs of frequent on-demand treatment with pdC1-INH.

Background: Lower respiratory tract infections frequently complicate the care of children with chronic tracheostomies. Pediatric patients have significantly more risk to have tracheostomy infections than adults. Better understanding of modifiable risk factors for pulmonary exacerbations may improve the care of technology-dependent children. Methods: A retrospective single-center cohort study conducted on children with tracheostomy and chronic home ventilator to determine the incidence of pulmonary exacerbations leading to hospitalizations, emergency room (ER) visits, and antibiotic prescriptions. Oral and nebulized antibiotic prescriptions were collected and correlated to the type of exacerbation. Results: Gram-negative enteric organisms were the most common microbes seen in the lower airways, with Pseudomonas aeruginosa cultured in 86% of the subjects. P. aeruginosa presence predicted a 4-fold increased rate of pulmonary-related hospitalization. In pediatric patients with chronic respiratory failure, 64% of readmissions were pulmonary or tracheostomy related. When compared to standard care subjects on dual agent, alternating monthly nebulized antibiotic therapy (for chronic pseudomonas colonization) experienced 41% fewer hospitalizations [incidence rate ratios (IRR) 0.59 (0.18), P = 0.08], 46% fewer ER visits [IRR 0.56 (0.16), P = 0.04], and 41% fewer pulmonary-related ER visits [IRR 0.59 (0.19), P = 0.94]. Discussion: Children who require artificial airways are at an increased risk for bacterial bronchopulmonary infections. Most notable risk factors for hospitalization in tracheostomized children included neurologic impairment, dysphagia, aspiration, gastrotomy tube dependence, and gastroesophageal reflux disease. Pathogenic microbes such as P. aeruginosa species, certain gram-negative bacteria, candida, and yeast also predicted increased hospitalizations. Use of nebulized antibiotics prophylaxis in a subset of patients predicted lower rates of hospitalization or ER visits. More studies are needed to assess whether there is increased antimicrobial resistance with this strategy, and whether the benefits persist in the long-term nebulized antibiotics utilization.

Background: Immunoskeletal dysplasia with neurodevelopmental abnormalities (ISDNA) caused by Exostosin-Like Glycosyltransferase 3 (EXTL3) biallelic mutations is a very rare syndrome with only 16 cases reported in the literature. Skeletal dysplasia, neurodevelopmental delay, immunodeficiency, liver, and kidney cysts are the most common findings of this syndrome. Case Presentation: Here, we report on a patient who exhibited a lethal phenotype with clinical characteristics of this syndrome and had a homozygous pathogenic mutation in EXTL3 gene. Conclusions: ISDNA should be kept in mind in the differential diagnosis of patients presenting with neuro-immuno-skeletal dysplasia phenotype.

Introduction: Data on the use of remote spirometry are limited in the pediatric population. We sought to assess the feasibility and accuracy of a digital turbine spirometer, Medical International Research (MIR) Spirobank Smart (MIR, New Berlin, WI, USA), compared with a pneumotachography spirometer, Pneumotrac (Vitalograph Inc., Lenexa, KS, USA), in field-based clinical research. Methods: This is a cross-sectional study of a subgroup of school-aged participants enrolled in the Air quality, Environment, and Respiratory Outcomes in Bronchopulmonary Dysplasia (BPD) study, who performed same-day paired coached baseline spirometry measurements from the Pneumotrac and MIR devices. Proportion of successful tests was estimated for each device and compared using McNemar's test. Correlation between devices forced expiratory volume in 1 second (FEV₁) and forced vital capacity (FVC) was analyzed by Lin's concordance correlation, and Bland-Altman plots were generated. Results: Twenty-one participants with history of BPD completed home spirometry maneuvers on both devices. The mean age of participants was 8.7 years. The mean FEV₁ and FVC measurement was 81% predicted and 90.4% predicted, respectively. The proportion of acceptable tests appeared higher using Pneumotrac (81%) than when using MIR (67%), although without evidence of discordance (P = 0.317). Among subjects with successful tests on both devices, Lin's concordance correlation demonstrated moderate agreement (FEV₁ r = 0.955, 95% confidence interval [CI]: 0.87-0.98; FVC r = 0.971, CI: 0.91-0.99). The mean difference in FEV₁ between Pneumotrac and MIR was 0.079 L (95% limits of agreement were -0.141 to 0.298 L) and FVC was 0.075 L (95% limits of agreement were -0.171 to 0.322 L). These were relatively small and without evidence of systematic or volume-dependent bias. Conclusions: Utilizing turbine spirometers may be a promising and feasible way to perform pulmonary function testing for field research in children.

Background: Severe bronchiolitis is a strong childhood asthma risk factor. Early and accurate asthma prediction is key. We applied the Asthma Predictive Index (API), the modified Asthma Predictive Index (mAPI), and the Pediatric Asthma Risk Score (PARS) in a cohort of high-risk infants to predict asthma at age 6 years. Methods: We conducted a 17-center cohort of infants (age <1 year) hospitalized with severe bronchiolitis during 2011-2014. We used only infancy data to predict asthma at age 6 years. Results: The prevalence of parent-reported asthma at age 6 years was 328/880 (37%). The prevalences of a positive index/score for stringent and loose API, mAPI, and PARS were 21%, 51%, 11%, and 34%, respectively. Area under the receiver operating characteristic curves [95% confidence interval (CI)] ranged from 0.57 (95% CI 0.55-0.60) for mAPI to 0.66 (95% CI 0.63-0.70) for PARS. Conclusions: An asthma prediction tool for high-risk infants is needed to identify those who would benefit most from asthma prevention interventions.

Background: Seeds are widely consumed as a traditional snack and have rich contents beneficial to health. With an increase in consumption rates, allergic reactions occur more frequently. We focus on multiple seed consumption related to recurrent anaphylaxis in this case. Case Presentation: We evaluated an 11-year-old boy with recurrent anaphylaxis. According to his medical records, he had been hospitalized several times, diagnosed with anaphylaxis, and treated. The family noticed direct (eating) or indirect contact with pumpkin seeds. In addition, the family mentioned another anaphylactic episode after watermelon seed and poppy seed bread consumption. We conducted skin prick-to-prick tests, examined total immunoglobulin E levels, and prescribed the treatment with an adrenalin autoinjector and preventive dietary recommendations. Conclusion: Anaphylaxis, particularly recurrent ones, should be evaluated with detailed anamnesis and supported with laboratory tests. Although seeds are beneficial and highly nutritious, it is necessary to consider them a source of allergens.

Study Objective: Food allergy is an essential growing public health concern that affects the quality of life of children and their parents. This study aimed to identify the parents' awareness and daily practice about food labels and allergy warnings on packaged foods. Materials and Methods: The study investigated the parents of children with food allergies who applied to the pediatric allergy outpatient clinic between October 01, 2020 and March 30, 2021. A total of 106 questionnaires were collected and statistically analyzed. Results: One hundred six parents with an average age of 31.6 ± 5.17 years were questioned. Most of the participants were mothers (88.7%). The most common food allergens observed in children were hen's egg (75%), cow's milk (56%), nuts (24.5%), peanuts (19.8%), walnuts (11.3%), and wheat (10.4%). Of the children, 39.6% rarely consumed packaged products, and the proportion of children who did not consume packaged products at all was 32.1%. All parents reported that they read the labels. Of them, 65.1% stated that allergy food labels were inadequate and it could be more noticeable if symbols (53.6%) or bold text (39.1%) were used for labeling. Conclusion: This study shows that parents with a diagnosis of food allergy in their child were highly aware of labels that indicate the content of the product. However, they thought that food labels are insufficient in scope and shape and needed to be improved. Parents preferred allergen labels with both symbols and bold text.

The etiology of Kawasaki disease (KD) and its precise genetic basics remain unknown. Genetic variants affecting immunity have been found in some patients. The occurrence of KD in siblings is rare, but KD pedigrees with multiple affected members have been described in Japan and North America. Cases in twins have been documented. We report 2 pairs of trizygotic triplets who developed KD associated with SARS-CoV2 infection from 2 different families. Our cases show that KD is multifactorial in origin, and both infectious etiology (particularly SARS-CoV2 as in our cases) and genetic factors are relevant in the disease.

Objective: Asthma is a common chronic disease and a substantial public health problem for children, adolescents, and adults. Adolescence, a period of increased independence and striving for autonomy, is an opportune time for youth transitioning to adulthood to assume more responsibility for their own asthma self-management. However, accurate measures of adolescent asthma outcomes are limited. The purpose of this systematic review is to identify self-reported asthma measures currently available in the empirical literature focused on adolescent populations. Methods: Search terms were based on the National Library of Medical Subject Headings and followed the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. Databases searched included CINAHL, Nursing Allied Health Prevention, Medline, ProQuest, and PubMed. Included studies were peer reviewed and published in English between 2010 and 2022. All studies reported on asthma measures for adolescents between 10 and 19 years old. Results: Nineteen studies were included, comprising 15 experimental and 4 quasi-experimental. This review revealed the following asthma measure domains: asthma knowledge, self-efficacy, attitudes, self-care, self-regulation, symptom prevention and management, medication adherence, asthma disease control, symptoms, and quality of life (QOL) for evaluating psychosocial, behavioral, clinical, and QOL outcomes. Conclusion: This review revealed the necessity of developing a comprehensive measure to assess the asthma self-management behaviors of adolescents. A comprehensive tool related to adolescent asthma self-management behavior would enhance the assessment and evaluation of adolescent asthma self-management behaviors and extend the science and clinical practice around adolescent self-management. Present measures for asthma self-management behavior for adolescents are limited; therefore, developing a valid and reliable measure is necessary not only to assess adolescents' asthma self-management behavior outcomes but also to identify and evaluate the essential components to include in educational interventions for adolescent self-management.