A pilot study of heated and humidified low flow oxygen therapy: An assessment in infants with mild and moderate bronchiolitis (HHOT AIR study).

BACKGROUND: Heated and humidified high flow nasal cannula oxygen therapy has been used in children with severe bronchiolitis. No data exists in children with mild to moderate bronchiolitis requiring lower flows of heated and humidified oxygen therapy. METHODS: We conducted a prospective, randomized pilot study of standard dry oxygen (control) versus heated and humidified low flow nasal cannula (HHLFNC), <4 liters per minute (LPM) oxygen, (treatment) in healthy children ≤24 months old with bronchiolitis. Clinical assessments were made using Respiratory Distress Assessment Instrument (RDAI), respiratory rate (RR), and oxygen saturation. RESULTS: Thirty-two children were enrolled (16 participants in each group). There was no significant difference in mean RDAI over time between groups. There was a significant difference in mean RDAI over time within control group, at hour 12, and treatment group, at hour 1, compared to baseline. RDAI in the treatment group was overall lower over time compared to control group. There was no significant difference in mean RR over time between or within groups, between mean length of stay and duration of oxygen requirement. Subgroup analyses showed lower RDAI in subjects that had RSV infection, male gender, and non-black race. CONCLUSIONS: The use of HHLFNC oxygen therapy may provide more comfort and may result in more rapid improvements in RDAI compared to standard dry oxygen therapy over time. HHFLNC is safe and well tolerated compared to standard dry oxygen. Larger studies are needed to assess the clinical efficacy of HHLFNC oxygen therapy.

A novel nutritional intervention improves lung function in overweight/obese adolescents with poorly controlled asthma: the Supplemental Nutrition in Asthma Control (SNAC) pilot study.

Asthma in the obese is often severe, difficult to treat, and characterized by less eosinophilic inflammation than asthma in the nonobese. Obesity-associated metabolic dysregulation may be a causal factor. We previously reported that a nutrient- and fiber-dense bar [Children's Hospital Oakland Research Institute (CHORI)-bar], which was designed to fill gaps in poor diets, improved metabolism in healthy overweight/obese (OW/OB) adults. In this pilot trial, OW/OB adolescents with poorly controlled asthma were randomized to weekly nutrition/exercise classes with or without twice-daily CHORI-bar consumption. Intent-to-treat analysis did not indicate CHORI-bar-specific effects. However, restricting the analysis to participants with acceptable compliance and a relatively low fraction of exhaled nitric oxide (FENO; <50/ ppb, a surrogate for noneosinophilic asthma; study participants: CHORI-bar, n = 16; controls, n = 15) indicated that CHORI-bar-specific, significant improvements in lung function (forced vital capacity, percent-predicted forced expiratory volume in 1 s, and percent-predicted forced expiratory flow between 25 and 75% of forced vital capacity), primarily in participants with low chronic inflammation (high-sensitivity C-reactive protein <1.5 mg/L). (We previously observed that chronic inflammation blunted CHORI-bar-induced metabolic improvements in healthy OW/OB adults.) Lung function improvement occurred without weight loss and was independent of improvements in metabolic and anthropometric end points and questionnaire-based measures of asthma control and quality of life. This study suggests that a nutritional intervention can improve lung function in OW/OB adolescents with asthma and relatively low FENO without requiring major changes in dietary habits, lifestyle, or weight loss and that this effect is blunted by chronic inflammation.-Bseikri, M., McCann, J. C., Lal, A., Fong, E., Graves, K., Goldrich, A., Block, D., Gildengoren, G. L., Mietus-Snyder, M., Shigenaga, M., Suh, J., Hardy, K., Ames, B. N. A novel nutritional intervention improves lung function in overweight/obese adolescents with poorly controlled asthma: the Supplemental Nutrition in Asthma Control (SNAC) pilot study.

Cooking behaviors are related to household particulate matter exposure in children with asthma in the urban East Bay Area of Northern California.

BACKGROUND: Asthma is a common childhood disease that leads to many missed days of school and parents' work. There are multiple environmental contributors to asthma symptoms and understanding the potential factors inside children's homes is crucial. METHODS: This is a dual cohort study measuring household particulate matter (PM2.5), behaviors, and factors that influence air quality and asthma symptoms in the urban homes of children (ages 6-10) with asthma; one cohort had cigarette smoke exposure in the home (n = 13) and the other did not (n = 22). Exposure data included measurements every 5 minutes for a month. RESULTS: In the entire study population, a large contributor to elevations in indoor PM2.5 above 35 µg/m3 was not using the stove hood when cooking (8.5% higher, CI 3.1-13.9%, p<0.005). Median PM values during cooking times were 0.88 µg/m3 higher than those during non-cooking times (95% CI 0.33-1.42). Mean monthly household PM2.5 level was significantly related to the presence of a cigarette smoker in the home (10.1 µg/m3 higher, 95% CI 5.2-15.1, p<0.001) when controlling for use of the stove hood and proximity to major roadway. There was a trend toward increased odds of persistent asthma with increases in average monthly PM2.5 (OR 1.1, 95% CI 0.97-1.3, p = 0.16). CONCLUSIONS: Consideration of only outdoor PM2.5 may obscure potentially modifiable risks for asthma symptoms. Specifically, this preliminary study suggests that cooking behaviors may contribute to the burden of PM2.5 in the homes of children with asthma and thus to asthma symptoms.

Maximum inspiratory pressure as a clinically meaningful trial endpoint for neuromuscular diseases: a comprehensive review of the literature.

Respiratory muscle strength is a proven predictor of long-term outcome of neuromuscular disease (NMD), including amyotrophic lateral sclerosis, Duchenne muscular dystrophy, and spinal muscular atrophy. Maximal inspiratory pressure (MIP), a sensitive measure of respiratory muscle strength, one of several useful tests of respiratory muscle strength, is gaining interest as a therapeutic clinical trial endpoint for NMD. In this comprehensive review we investigate the use of MIP as a measure of respiratory muscle strength in clinical trials of therapeutics targeting respiratory muscle, examine the correlation of MIP with survival, quality of life, and other measures of pulmonary function, and outline the role of MIP as a clinically significantly meaningful outcome measure. Our analysis supports the utility of MIP for the early evaluation of respiratory muscle strength, especially of the diaphragm, in patients with NMD and as a surrogate endpoint in clinical trials of therapies for NMD.

Implementing evidence-driven individualized treatment plans within Morquio A Syndrome.

Morquio A Syndrome (mucopolysaccharidosis IVA [MPS IVA]) is an inherited, autosomal recessive lysosomal storage disorder that occurs in ~1 in 200,000 to 300,000 live births.(1) (Online access http://www.elseviercme.com/559) Individuals with Morquio A Syndrome have mutations in the gene that encodes N-acetylgalactosamine-6-sulfate sulfatase (GALNS), an enzyme responsible for the metabolism of the glycosaminoglycans (GAGs) keratin sulfate and chondroitin-6-sulfate.(2-4) Reduced activity or lack of GALNS leads to cellular and tissue accumulation of these GAGs to result in progressive, multisystem dysfunction and impaired functional capacity.(5) Individuals with Morquio A Syndrome suffer from a broad spectrum of impairment, including a variety of widespread skeletal abnormalities, respiratory compromise, valvular heart disease, visual and auditory impairments, and dental abnormalities.(6-8) Cognition is not typically affected.(9) Morquio A Syndrome exhibits extensive allelic heterogeneity, which results in extensive clinical heterogeneity.(2-4) This educational intervention on the management of patients with Morquio A Syndrome provides updated information and guidelines concerning the early and accurate diagnosis as well as an earlier intervention to improve patient outcomes. The activity is based on a live satellite symposium conducted during the 2015 official ACMG Annual Clinical Genetics Meeting program. Recent advances in the science of enzyme replacement therapies have presented opportunities for pharmacological interventions that improve quality of life. Clinicians will be updated on the clinical trial data and practical solutions for applying newer therapeutics to daily practice. Strategies to manage cardiopulmonary comorbidities and recommendations for the ideal clinical care model will wrap up this informative and up-to-date review of Morquio A Syndrome. This CME activity is also available through the Website of Molecular Genetics and Metabolism. Click on the CME button in the navigation bar for full access. Or access: http://www.elseviercme.com/559.