Background Cystic fibrosis (CF) is a genetic disorder of the cystic fibrosis transmembrane conductance regulator chloride channel that leads to impaired mucus clearance in the airways, which leads to deteriorations in lung function and chronic respiratory infection. These effects of CF contribute to the hypothesis that patients with CF may be at increased risk of complications when they catch coronavirus disease 2019 (COVID-19), which swept the world in a global pandemic starting in 2019. Overall, however, the role of CF in COVID-19 has not been well studied, particularly in pediatric patients.
View Article and Find Full Text PDFObjectives: Describe clinical characteristics of adolescents hospitalized with e-cigarette or vaping product use-associated lung injury (EVALI) and to investigate association between EVALI and coagulopathy.
Methods: We conducted a retrospective cohort study of adolescents admitted to the general inpatient or ICUs at 2 major tertiary children's hospitals from January 2019 to June 2021. We included analysis of demographics, clinical findings, laboratory and imaging results, and outcomes.
Congenital central hypoventilation syndrome (CCHS) is a rare disorder that results in profound hypoventilation that is most prominent during periods of sleep. Caused by a genetic mutation in the gene, CCHS typically presents in the newborn period with symptoms of hypoventilation. However, there is a subset of patients with the same genetic mutation who present much later in life, which is termed late-onset congenital central hypoventilation syndrome (LO-CCHS).
View Article and Find Full Text PDFOmega-3 fatty acid (n3PUFA) supplementation has been proposed as a promising antiasthma strategy. The rs59439148 polymorphism affects leukotriene production and possibly inflammatory responses to n3PUFA. Assess the effects of n3PUFA supplementation and genotype on asthma control in patients with obesity and uncontrolled asthma.
View Article and Find Full Text PDFIntroduction: The prevalence of attention-deficit/hyperactivity disorder (ADHD) in pediatric cystic fibrosis (CF) patients is comparable to the general population, but the effects of ADHD on CF treatment and the outcomes have been minimally investigated.
Methods: Two cohorts were retrospectively reviewed, pediatric patients with comorbid CF/ADHD and patients with CF only. Each patient with CF/ADHD was age and sex-matched to a CF-only patient based on their most recent pulmonary office visit.