Clinical Findings in Adolescents Hospitalized With EVALI; Novel Report on Coagulopathy.

OBJECTIVES: 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. RESULTS: Forty-four hospitalizations met diagnostic criteria for inclusion per Centers for Disease Control and Prevention guidelines, with 55% of patients admitted after April 2020. Compared with adults, pediatric patients were less likely to present with pulmonary symptoms. Significant laboratory work included elevated white blood cell count of 14.3 k/uL (confidence interval [CI], 13.7-15.0) with neutrophilic predominance, C-reactive protein of 25.2 mg/dL (CI, 22.1-28.2), and erythrocyte sedimentation rate of 66.7 mm/hour (CI, 26.9-76.4). Chest radiographs were poor predictors of disease in 53% of our patients but computed tomography was 100% sensitive. Significant coagulation abnormalities included prothrombin time of 17.7 seconds (CI, 16.4-19.1) and international normalized ratio of 1.54 (CI, 1.43-1.66). Coagulation studies improved with vitamin K and steroid administration. Nine of 16 patients (56%) had abnormal diffusing capacity of the lung for carbon monoxide divided by alveolar volume <80% predicted, suggesting evidence of pulmonary vascular disease, or >100%, suggesting pulmonary hemorrhage. CONCLUSIONS: EVALI continues to be an important differential diagnosis in the adolescent population. EVALI is likely a result of systemic inflammation with consequences beyond the pulmonary system. The novel report of coagulopathy among adolescents with EVALI in this cohort reveals an opportunity to detect coagulopathy and initiate early therapy.

Altered Mental Status and Respiratory Failure in an 11-Year-Old Female.

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 PHOX2B 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). The reason for its late presentation is unclear but is often dramatic. Given its rarity, the diagnosis can be difficult to establish but can be accomplished by using a systematic approach. Here, we present a case of LO-CCHS in an 11-year-old female who presented with respiratory failure and altered mental status.

Fish Oil Supplementation in Overweight/Obese Patients with Uncontrolled Asthma. A Randomized Trial.

Rationale: Omega-3 fatty acid (n3PUFA) supplementation has been proposed as a promising antiasthma strategy. The rs59439148 ALOX5 polymorphism affects leukotriene production and possibly inflammatory responses to n3PUFA. Objectives: Assess the effects of n3PUFA supplementation and ALOX5 genotype on asthma control in patients with obesity and uncontrolled asthma. Methods: This multicenter trial among 12- to 25-year-olds with overweight/obesity and uncontrolled asthma randomized subjects in a 3:1 allotment to n3PUFA (4 g/d) or soy oil control for 24 weeks. Asthma Control Questionnaire was the primary outcome; secondary outcomes included blood leukocyte n3PUFA levels, urinary leukotriene-E4, spirometry, and asthma-related events. The number of SP1 tandem repeats in rs59439148 determined ALOX5 genotype status. Simple and multivariable generalized linear models assessed effects on outcomes. Results: Ninety-eight participants were randomized (77 to PUFA, 21 to control), and more than 86% completed all visits. Asthma and demographic characteristics were similar among treatment groups. n3PUFA treatment increased the n3-to-n6 PUFA ratio in circulating granulocytes (P = 0.029) and monocytes (P = 0.004) but did not affect mean Asthma Control Questionnaire change at 6 months (n3PUFA: mean, -0.09; 95% confidence interval [CI], 0.09 to 0.10; vs. control: mean, -0.18; 95% CI, -0.42 to 0.06; P = 0.58). Changes in urinary leukotriene-E4 (P = 0.24), forced expiratory volume in 1 second % predicted (P = 0.88), and exacerbations (relative risk [RR], 0.92; 95% CI, 0.30-2.89) at 6 months were similar in both groups. n3PUFA treatment was associated with reduced asthma-related phone contacts (RR, 0.34; 95% CI, 0.13-0.86; P = 0.02). ALOX5 genotype did not affect n3PUFA treatment responses. Conclusions: We did not find evidence that n3PUFA use improves most asthma-related outcomes and cannot recommend it as a prevention strategy for overweight/obese patients with asthma. Clinical trial registered with www.clinicaltrials.gov (NCT01027143).

The Prevalence and Effect of Comorbid Cystic Fibrosis and Attention Deficit Hyperactivity Disorders on Hospitalizations: A Retrospective Analysis.

INTRODUCTION: 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. Each patient was reviewed for forced expiratory volume in one-second percent predicted (FEV1%pred), body mass index (BMI) percentile, and hospitalizations for one year prior to the last pulmonary visit. RESULTS: A total of 624 patients with CF were identified, with 52 having co-morbid CF/ADHD (8.3%). Of those identified, 46 met inclusion criteria and were analyzed in the CF/ADHD cohort. The mean total hospital admissions between the CF/ADHD cohort and the CF-only cohort were not statistically significant (2.22 vs 1.834, p=.467). The difference between the BMI percentiles was not statistically significant (48.634 vs 38.634, p=.135). The difference between FEV1%pred was statistically significant at 84% for the CF/ADHD group and 74% for the CF-only group (p=.042). CONCLUSION: The difference in total hospital admissions between the CF/ADHD cohort and the CF-only cohort did not reach statistical significance, but the study was underpowered. There was a significant difference between FEV1%pred between the two groups, in favor of the comorbid CF/ADHD population. More research is needed to further evaluate the effects of a comorbid ADHD diagnosis on outcomes in the CF pediatric population.

Effects of an Antioxidant-enriched Multivitamin in Cystic Fibrosis. A Randomized, Controlled, Multicenter Clinical Trial.

RATIONALE: Cystic fibrosis (CF) is characterized by dietary antioxidant deficiencies, which may contribute to an oxidant-antioxidant imbalance and oxidative stress. OBJECTIVES: Evaluate the effects of an oral antioxidant-enriched multivitamin supplement on antioxidant concentrations, markers of inflammation and oxidative stress, and clinical outcomes. METHODS: In this investigator-initiated, multicenter, randomized, double-blind, controlled trial, 73 pancreatic-insufficient subjects with CF 10 years of age and older with an FEV1 between 40% and 100% predicted were randomized to 16 weeks of an antioxidant-enriched multivitamin or control multivitamin without antioxidant enrichment. Endpoints included systemic antioxidant concentrations, markers of inflammation and oxidative stress, clinical outcomes (pulmonary exacerbations, anthropometric measures, pulmonary function), safety, and tolerability. MEASUREMENTS AND MAIN RESULTS: Change in sputum myeloperoxidase concentration over 16 weeks, the primary efficacy endpoint, was not significantly different between the treated and control groups. Systemic antioxidant (ß-carotene, coenzyme Q10, γ-tocopherol, and lutein) concentrations significantly increased in the antioxidant-treated group (P < 0.001 for each), whereas circulating calprotectin and myeloperoxidase decreased in the treated group compared with the control group at Week 4. The treated group had a lower risk of first pulmonary exacerbation requiring antibiotics than the control group (adjusted hazard ratio, 0.50; P = 0.04). Lung function and growth endpoints did not differ between groups. Adverse events and tolerability were similar between groups. CONCLUSIONS: Antioxidant supplementation was safe and well tolerated, resulting in increased systemic antioxidant concentrations and modest reductions in systemic inflammation after 4 weeks. Antioxidant treatment was also associated with a lower risk of first pulmonary exacerbation. Clinical trial registered with www.clinicaltrials.gov (NCT01859390).

Household proximity to water and nontuberculous mycobacteria in children with cystic fibrosis.

BACKGROUND: Nontuberculous mycobacteria (NTM) have a particular affinity for patients with cystic fibrosis (CF). Recent studies suggest a possible relationship between acquiring NTM and the level of environmental water in a given area. We sought to determine if there is an association between household proximity to water and NTM in children with CF. MATERIALS AND METHODS: An IRB-approved retrospective chart review was completed on 150 children with CF in Florida. Inclusion criteria required regular follow-up, at least two acid-fast bacilli cultures, and a consistent home address over a 3-year period. The distance from each patient's home to the nearest body of water was measured using ArcMap®, a Geographic Information System, and the mean distance to water for NTM-positive and NTM-negative groups were compared. A stepwise backwards logistic regression was used to evaluate for predictors of NTM-positivity. RESULTS: Of the 150 CF patients, 65 met inclusion criteria and 21 (32.3%) tested positive for NTM. Comparison of the mean distance to water for NTM-positive versus NTM-negative groups revealed a cutoff of 500 meters. On the logistic regression, CF patients who lived within 500 meters of water were 9.4 times more likely to acquire NTM (P = 0.013). Other significant predictors included a history of Aspergillus fumigatus (OR 7.9, P = 0.011) and recent history of Pseudomonas aeruginosa (OR 2.5, P = 0.007). CONCLUSIONS: In the regions studied, children with CF who live closer to water are more likely to acquire nontuberculous mycobacteria. Future studies in other geographic areas are needed to determine if these results are generalizable. Pediatr Pulmonol. 2017;52:324-330. © 2016 Wiley Periodicals, Inc.