Pharmacokinetic variability of CFTR modulators from standard and alternative regimens.

Elexacaftor, tezacaftor, ivacaftor (ETI) is a CFTR modulator combination approved for use in ∼90 % of people with cystic fibrosis (pwCF) over 2 years old. While most pwCF tolerate this therapy well, some are intolerant to standard dosing, and others show little response. Clinical providers may adjust ETI dosing to combat these issues, but these adjustments are not well guided by pharmacokinetic evidence.

Improving knowledge, technical skills, and confidence among pediatric health care providers in the management of chronic tracheostomy using a simulation model.

Objective: The results from a recent national survey about catastrophic complications following tracheostomy revealed that the majority of events involved a loss of airway. Most of the events due to airway loss involved potentially correctable deficits in caregiver education. Training in a simulated environment allows skill acquisition without compromising patient safety.

Mobile-based asthma action plans for adolescents.

Purpose: To examine feasibility and utilization of a mobile asthma action plan (AAP) among adolescents.

Methods: Adolescents (aged 12-17 years) with persistent asthma had their personalized AAP downloaded to a smartphone application. Teens were prompted by the mobile application to record either daily symptoms or peak flow measurements and to record medications.

Limited reliability of lipid-laden macrophage index restricts its use as a test for pulmonary aspiration: comparison with a simple semiquantitative assay.

The lipid-laden macrophage index (LLMI) is a semiquantitative evaluation of alveolar macrophage lipid content used in diagnosis of pulmonary aspiration. To date, there are no published reports regarding the reliability of LLMI. We sought to evaluate the interobserver and intraobserver variability and validity of LLMI and to compare it to a simpler macrophage lipid content index (LCI).

Pediatric flexible airway endoscopy.

Flexible airway endoscopy has been used in clinical and research investigations of pediatric airway and pulmonary disorders for nearly 25 years. Not only has clinical use of the flexible bronchoscope improved our evaluation and management of a variety of airway and pulmonary diseases in children, but also research investigations using lavage and biopsy specimens obtained with the flexible bronchoscope have contributed extensively to our understanding of lung inflammation and infection. Improvements and new developments in fiberoptic endoscope technology, training of airway endoscopists, preoperative and sedative medications, patient monitoring, and airway endoscopic techniques, as well as adjunctive minimally invasive and noninvasive diagnostic modalities, continue to refine and enhance the pediatric clinical and research applications of flexible airway endoscopy.