Lung ultrasound in children with primary ciliary dyskinesia or cystic fibrosis.

Introduction: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are respiratory conditions requiring regular chest radiography (CXR) surveillance to monitor pulmonary disease. However, CXR is insensitive for lung disease in CF and PCD. Lung ultrasound (LU) is a radiation-free alternative showing good correlation with severity of lung disease in CF but has not been studied in PCD.

Impact of Transplant Body Mass Index and Post-Transplant Weight Changes on the Development of Chronic Lung Allograft Dysfunction Phenotypes.

Introduction: Chronic lung allograft dysfunction (CLAD) is a lung transplant complication for which four phenotypes are recognized: Bronchiolitis obliterans syndrome (BOS), Restrictive allograft syndrome (RAS), mixed and undefined phenotypes. Weight gain is common after transplant and may negatively impact lung function. Study objectives were to describe post-transplant weight trajectories of patients who developed (or did not) CLAD phenotypes and examine the associations between BMI at transplant, post-transplant changes in weight and BMI, and the risk of developing these phenotypes.

Efficacy and safety of LAU-7b in a Phase 2 trial in adults with cystic fibrosis.

Background: Lung inflammation is associated with tissue damage in cystic fibrosis (CF). LAU-7b, a novel oral drug candidate, was shown to control inflammation and stabilize CFTR protein in the epithelial membrane during inflammatory stress in preclinical models of CF.

Methods: A double-blind, randomized, placebo-controlled Phase 2 study was conducted to evaluate efficacy and safety of LAU-7b in adults with CF.

Card9 Broadly Regulates Host Immunity against Experimental Pulmonary 52D Infection.

The ubiquitous soil-associated fungus causes pneumonia that may progress to fatal meningitis. Recognition of fungal cell walls by C-type lectin receptors (CLRs) has been shown to trigger the host immune response. Caspase recruitment domain-containing protein 9 (Card9) is an intracellular adaptor that is downstream of several CLRs.

Reduced exercise capacity for muscle mass in adolescents living with obesity.

Background: Adolescents living with obesity (AlwO) can have limited exercise capacity. Exercise capacity can be predicted by a 2-factor model comprising lung function and leg muscle function, but no study has looked at cycling leg muscle function and its contribution to cycling exercise capacity in AlwO.

Methods: Twenty-two nonobese adolescents and 22 AlwO (BMI > 95 percentile) were studied.

Preventative therapies for respiratory Syncytial virus (RSV) in children: Where are we now?

Respiratory Syncytial Virus (RSV) is a leading cause of hospitalization in young children and represents a substantial health-care burden around the world. Advances in research have helped identify the prefusion F protein as the key target component in RSV immunization. In this article, we review the previous, current, and ongoing research efforts for immunization against RSV in children.

Canadian Cystic Fibrosis-related Diabetes Clinical Practice Survey: Analysis of Current Practices and Gaps in Clinical Care.

Objectives: Our aim in this study was to identify challenges and gaps in Canadian practices in screening, diagnosis, and treatment of cystic fibrosis-related diabetes (CFRD), with the goal of informing a Canadian-specific guideline for CFRD.

Methods: We conducted an online survey of health-care professionals (97 physicians and 44 allied health professionals) who care for people living with CF (pwCF) and/or CFRD (pwCFRD).

Results: Most pediatric centres followed <10 pwCFRD and adult centres followed >10 pwCFRD.

Cystic fibrosis related diabetes is not associated with maximal aerobic exercise capacity in cystic fibrosis: a cross-sectional analysis of an international multicenter trial.

Background: Previous studies have reported differences in aerobic exercise capacity, expressed as peak oxygen uptake (VO), between people with and without cystic fibrosis (CF) related diabetes (CFRD). However, none of the studies controlled for the potential influence of physical activity on VO. We investigated associations between CFRD and VO following rigorous control for confounders including objectively measured physical activity.

Lung ultrasound findings in asymptomatic healthy children with asthma.

Background: Lung ultrasound (LUS) has been shown to be an effective tool to rapidly diagnose certain causes of pediatric respiratory distress. However, very little is known about LUS findings in pediatric asthma.

Objectives: The primary objective of this study was to characterize LUS findings in a cohort of pediatric patients with a definitive diagnosis of asthma, outside of an asthma exacerbation.

New-onset Obesity After Lung Transplantation: Incidence, Risk Factors, and Clinical Outcomes.

Background: Lung transplant (LTx) recipients who gain weight after transplantation may experience an upward shift in body mass index (BMI) that places them in the obese category. The incidence, risk factors, and impact on metabolic health and mortality of new-onset obesity have not been documented in the LTx setting.

Methods: This single-center retrospective study included 564 LTx recipients.

Re-opening the pediatric pulmonary function laboratory during the ongoing COVID-19 pandemic.

The COVID-19 pandemic continues with new waves of intensification. This review provides an update based on international recommendations concerning the conduct of pulmonary function testing in a manner to limit risk to both patient and tester.

Characterizing pediatric lung ultrasound findings during a chemically induced bronchospasm.

Background: Lung ultrasound (LUS) has been shown to be a useful clinical tool in pediatrics, but very little is known about the LUS findings of asthma in children.

Objectives: The primary objective was to characterize LUS findings of pediatric patients before and after a chemically induced bronchospasm. The secondary objective was to evaluate the effect of bronchodilators on LUS findings.

A scoping review of mHealth monitoring of pediatric bronchial asthma before and during COVID-19 pandemic.

Mobile (m) Health technology is well-suited for Remote Patient Monitoring (RPM) in a patient's habitual environment. In recent years there have been fast-paced developments in mHealth-enabled pediatric RPM, especially during the COVID-19 pandemic, necessitating evidence synthesis. To this end, we conducted a scoping review of clinical trials that had utilized mHealth-enabled RPM of pediatric asthma.

Effects of a Partially Supervised Conditioning Program in Cystic Fibrosis: An International Multicenter, Randomized Controlled Trial (ACTIVATE-CF).

The long-term effects of vigorous physical activity (PA) on lung function in cystic fibrosis are unclear. To evaluate effects of a 12-month partially supervised PA intervention using motivational feedback. In a parallel-arm multicenter randomized controlled trial (ACTIVATE-CF), relatively inactive patients aged at least 12 years were randomly assigned (1:1 ratio) to an intervention group or control group.

Long-term bone mineral density changes and fractures in lung transplant recipients with cystic fibrosis.

Background: Little is known about long-term bone mineral density (BMD) changes and fractures in lung transplant recipients with cystic fibrosis (CF). We examined femur and lumbar spine (LS) BMD changes in men and women with CF up to 10 years post-transplant and documented post-transplant fracture prevalence.

Methods: Retrospective study of individuals who had undergone a lung transplant (2000-2015) and had a pre-transplant and at least one BMD measurement after transplant.

Brief report: International perspectives on the pediatric COVID-19 experience.

The 2019 novel coronavirus (SARS-CoV-2) is endangering human health worldwide; scarcity of published pediatric cases and current literature and the absence of evidence-based guidelines necessitate international sharing of experience and personal communication. On 31 March 2020 the International Committee of the American Thoracic Society Pediatrics Assembly recorded an online podcast, during which pediatric pulmonologists worldwide shared their experience on the novel coronavirus disease (COVID-19) in children. The aim was to share personal experience in organizing pediatric care in different health care settings globally, protecting health care workers, and isolation practices.

Comparison of oscillometry devices using active mechanical test loads.

Noninvasiveness, low cooperation demand and the potential for detailed physiological characterisation have promoted the use of oscillometry in the assessment of lung function. However, concerns have been raised about the comparability of measurement outcomes delivered by the different oscillometry devices. The present study compares the performances of oscillometers in the measurement of mechanical test loads with and without simulated breathing.

Trends, Determinants, and Impact on Survival of Post-Lung Transplant Weight Changes: A Single-center Longitudinal Retrospective Study.

Background: Weight gain is commonly seen in lung transplant (LTx) recipients. Although previous studies have focused on weight changes at fixed time periods and relatively early after transplant, trends over time and long-term weight evolution have not been described in this population. The study objectives were to document weight changes up to 15 years post-LTx and assess the predictors of post-LTx weight changes and their associations with mortality.

Elexacaftor-Tezacaftor-Ivacaftor for Cystic Fibrosis with a Single Phe508del Allele.

Background: Cystic fibrosis is caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein, and nearly 90% of patients have at least one copy of the Phe508del mutation. In a phase 2 trial involving patients who were heterozygous for the Phe508del mutation and a minimal-function mutation (Phe508del-minimal function genotype), the next-generation CFTR corrector elexacaftor, in combination with tezacaftor and ivacaftor, improved Phe508del CFTR function and clinical outcomes.

Methods: We conducted a phase 3, randomized, double-blind, placebo-controlled trial to confirm the efficacy and safety of elexacaftor-tezacaftor-ivacaftor in patients 12 years of age or older with cystic fibrosis with Phe508del-minimal function genotypes.