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Israel Amirav,1 Michael T Newhouse2 1Pediatric Department, University of Alberta, Edmonton, AB T6G2C6, Canada; 2Firestone Institute for Respiratory Health, St. Joseph’s Hospital, McMaster University, Hamilton, ON, CanadaCorrespondence: Israel AmiravDana-Dwek Children’s Hospital, Tel Aviv, IsraelTel +1 972-55-6649359Email amirav@ualberta.caAbstract: There have recently been major objections to the use of short-acting beta-agonist (SABA) in episodic acute asthma culminating in a call for replacing SABA with combination of inhaled corticosteroids and long-acting beta-agonists despite little evidence supporting this point of view. It is regrettable to note that this attack on SABA occurs in the midst of an unprecedented demand for, and shortage of, SABA inhalers during the current COVID-19 pandemic, and the worldwide efforts to increase SABA supplies. In this commentary, we defend the well-established role of SABA and argue that the call for the phase out of SABA is inappropriate, since it is not solidly evidence based.Keywords: asthma, beta-agonists, COVID-19, inhalers

Israel Amirav,1,* Virginia Vandall-Walker,2,3,* Jananee Rasiah,2,3 Laura Saunders,4 Harsha Belur,4 Brenda Sahlin,4 Mary Roduta Roberts,5 Dorit Redlich-Amirav6 1Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada; 2Patient Engagement Platform, Alberta SPOR Support Unit, Edmonton, Alberta, Canada; 3Faculty of Health Disciplines, Athabasca University, Athabasca & Faculty of Nursing, University of Alberta, Edmonton, Alberta, Canada; 4Parent Advisory Council, Edmonton, Alberta, Canada; 5Department of Occupational Therapy, University of Alberta, Edmonton, Alberta, Canada; 6Faculty of Rehabilitation Medicine, University of Alberta, Edmonton, Alberta, Canada*These authors contributed equally to this workCorrespondence: Israel AmiravPediatric Pulmonology Unit, Dana-Dwek Children’s Hospital, Faculty of Medicine, Tel Aviv University, Tel Aviv, IsraelTel +1 972-55-664-9359Email amirav@ualberta.caObjective: Written summaries about visits with general practitioners’ have influenced increased patient knowledge, satisfaction, recollection, and compliance, and strengthened the doctor–patient relationship. All previous studies about this communication pre-dated the electronic medical record (EMR) era, and none examined views from parents of children with asthma. We explored parents’ perceptions about receiving a hard copy summary Letter immediately following the visit, with the pediatric asthma specialist about findings and the care plan for their child.Methods: A Parent Advisory Council helped inform this pilot study, an observational cross-sectional electronic survey. Each Letter included a comprehensive summary of the child’s visit with the specialist.Results: Previous findings from patients about the benefits of receiving this Letter were strongly supported by data from 51 participants. Interestingly, more than 54% of respondents preferred receiving a hard copy Letter over an electronic copy, and 98% wanted other clinicians to adopt this practice.Conclusion: Parents of children with asthma value and want timely written information of this nature from other clinicians.Practice Implications: These results can influence further asthma research to promote a change in the perceptions of clinicians, parents, and patients about timely access to health information in the EMR era.Keywords: patient perspectives, provider–patient communication, asthma, parents

Background: Small volume nebulisers (SVNs) with masks commonly provide aerosol therapy for infants with lung diseases. However, infants and toddlers are often disturbed by and thus reject masks. Aims: To […]

Purpose: Electron-beam CT (EBCT) was utilized to assess the time course of changes in airways cross-sectional area (CSA) and lung density during methacholine-induced bronchoconstriction.Materials and Methods: EBCT scans (200 ms, 3-mm thickness, 2 mm increments) were obtained before (baseline) and 30 s, 2 min, and 4 min after bolus IV injection of methacholine to pigs receiving mechanical ventilation. A total of seven experiments were analyzed using custom-made image analysis software. With each challenge, five different airways and 50 lung regions of interest were studied.Results: The time course of lung density changes paralleled the time course for CSA changes. The maximal response to methacholine, measured in terms of both CSA and lung density changes, occurred 30 s after injection. Lung density changes were unaffected by reconstruction algorithm, normal (standard) or sharp (high resolution). Overall, there was increased air content in the lung during bronchoconstriction. This effect was significantly greater at the dependent lung regions.Conclusions: EBCT is an effective tool to assess temporal and regional changes in the lung during bronchoconstriction. Measurements of lung density during bronchoconstriction allow for assessment of peripheral changes that are beyond the CT spatial resolution of airways anatomy. [ABSTRACT FROM AUTHOR]

To evaluate the effect of negative ionisation of inspired air on bronchial reactivity, 11 asthmatic children were challenged twice by exercise and 10 were challenged twice by histamine inhalation. The children breathed negatively ionised air (4 X 10(5) - 10 X 10(5) ions/cm3) or control room air in random order in a double-blind fashion. All challenges were matched in terms of basal lung function and the exercise tests were matched in terms of ventilation and respiratory heat loss. Exercise-induced asthma was significantly attenuated by exposure to negatively ionised air, the mean postexercise fall in one-second forced expiratory volume (FEV1) being 29% (SE 5%) of the initial value after the control and 21% (3%) after the ionised air test (p less than 0.02). Ten of the 11 subjects developed less exercise-induced asthma while breathing ionised air. Although the median dose of histamine (cumulative breath units) which caused a constant fall in FEV1 for each individual was higher with the ionised air challenge than with the control challenge the difference was not significant. Five of the 10 subjects were less sensitive to histamine and the other five more sensitive when breathing ionised air. It is concluded that negative ionisation of inspired air can modulate the bronchial response to exercise but the effect on the response to histamine is much more variable. [ABSTRACT FROM AUTHOR]

To evaluate the effect of positive ionisation of inspired air on bronchial reactivity, 12 asthmatic children were twice challenged by exercise in random order. During one test positively ionised air (5-10 X 10(5) ions/cm) was breathed. All challenges were matched in terms of basal lung function and exercise tests were matched in terms of ventilation and respiratory heat loss. Exercise induced asthma was significantly aggravated by exposure to positively ionised air, the postexercise fall in FEV1 (delta FEV1) being 24.7% (SEM and 5.3%) and 35.3% (5%) after the control and ionised air tests respectively (p less than 0.04). It is concluded that positive ionisation aggravates the bronchial response to exercise. [ABSTRACT FROM AUTHOR]

Background: Primary ciliary dyskinesia (PCD) represents a group of rare hereditary disorders characterised by deficient ciliary airway clearance that can be associated with laterality defects. We aimed to describe the underlying gene defects, geographical differences in genotypes and their relationship to diagnostic findings and clinical phenotypes., Methods: Genetic variants and clinical findings (age, sex, body mass index, laterality defects, forced expiratory volume in 1 s (FEV 1 )) were collected from 19 countries using the European Reference Network's ERN-LUNG international PCD Registry. Genetic data were evaluated according to American College of Medical Genetics and Genomics guidelines. We assessed regional distribution of implicated genes and genetic variants as well as genotype correlations with laterality defects and FEV 1 ., Results: The study included 1236 individuals carrying 908 distinct pathogenic DNA variants in 46 PCD genes. We found considerable variation in the distribution of PCD genotypes across countries due to the presence of distinct founder variants. The prevalence of PCD genotypes associated with pathognomonic ultrastructural defects (mean 72%, range 47-100%) and laterality defects (mean 42%, range 28-69%) varied widely among countries. The prevalence of laterality defects was significantly lower in PCD individuals without pathognomonic ciliary ultrastructure defects (18%). The PCD cohort had a reduced median FEV 1 z-score (-1.66). Median FEV 1 z-scores were significantly lower in CCNO (-3.26), CCDC39 (-2.49) and CCDC40 (-2.96) variant groups, while the FEV 1 z-score reductions were significantly milder in DNAH11 (-0.83) and ODAD1 (-0.85) variant groups compared to the whole PCD cohort., Conclusion: This unprecedented multinational dataset of DNA variants and information on their distribution across countries facilitates interpretation of the genetic epidemiology of PCD and indicates that the genetic variant can predict diagnostic and phenotypic features such as the course of lung function., Competing Interests: Conflict of interest: The authors have no potential conflicts of interest to disclose., (Copyright ©The authors 2024.)

Though PCD usually presents after birth in term neonates, diagnosing PCD during the neonatal and infancy stages is uncommon, particularly in children who do not exhibit laterality defects. We report our recent experience with the diagnosis of PCD in the neonatal and early infantile period in a highly consanguine population. This was achieved by implementing a novel genetic-based diagnostic approach based on direct testing for recognized regional genetic variants. We conducted a retrospective analysis of children diagnosed with PCD at Soroka University Medical Center during the neonatal or early infantile period between 2020 and 2023. We included children under 3 months of age who had a genetic confirmation of PCD, as evidenced by the presence of two pathogenic variants in recognized genes. Genetic testing targeted regional genetic variants in previously identified PCD genes. Eight patients were included. The median age at diagnosis was 12.5 days. Three (38%) were born prematurely < 34 weeks gestational age. All patients were presented with respiratory distress and hypoxemia after birth. The median duration of oxygen support was 23 days, and upper lobe atelectasis was present in five patients (63%). Congenital cardiac malformation was present in four patients. Organ laterality defects were present in four patients. Genetic mutations identified were in the DNAAF5, DNAL1, DNAAF3, and DNAH1 genes.     Conclusion: Neonatal diagnosis of PCD is uncommon, especially in atypical presentations such as children without laterality defects or preterms. Focusing on a genetic diagnosis of the local tribal pathogenic variants promotes a potential cost-efficient test leading to earlier diagnosis. There is a need for a standardized protocol for earlier diagnosis of PCD in high-consanguinity areas. What is Known: • Primary ciliary dyskinesia (PCD) typically presents after birth in term neonates. • Diagnosing PCD during neonatal and infancy stages is challenging, particularly in children without laterality defects. What is New: • A novel genetic-based diagnostic approach was implemented on the neonatal population in a highly consanguine community, focusing on direct testing for regional genetic variants, leading to early and rapid diagnosis of PCD., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

In clinical practice, increased "work of breathing" (WOB) is used to rapidly identify the acutely ill child in need of immediate clinical care, and is commonly used to support a clinical diagnosis of pneumonia. However, this key clinical sign is poorly understood and inconsistently defined. This review discusses the physiology, measurement, and clinical assessment of WOB, highlighting its utility in the recognition of pneumonia in under-resourced settings, where access to diagnostic imaging may be limited.

Background: Several factors may influence quality of life (QOL) for patients with primary ciliary dyskinesia (PCD). We aimed to evaluate the association between pulmonary functions, nasal symptoms and QOL in PCD patients., Methods: A prospective single center study. Patients performed spirometry, whole body plethysmography, forced oscillation technique (FOT), lung clearance index (LCI), 6-min walk test (6MWT), and filled two questionnaires: a specific PCD QOL questionnaire (PCD-QOL) and Sino-nasal outcome test (SNOT-22) questionnaire, assessing symptoms of chronic rhinosinusitis and health related QOL., Results: Twenty-seven patients (56% females), age 19.4 ± 10.5 years were included; their, FEV1 was 74.6 ± 22.7%, and RV/TLC was (157.3 ± 39.3% predicted). Health perception and lower respiratory symptoms domains of PCD-QOL had the lowest score (median [IQR]: 50 [33.3-64.6] and 57.1 [38.9-72.2], respectively). FOT parameters correlated with several PCD-QOL domains. R5 z-score (indicating total airway resistance) and AX z-score (indicating airway reactance) correlated negatively with physical domain (r = -0.598, p = .001, and r = -0.42, p = .03, respectively); R5 z-score also correlated negatively with hearing domain (r = -0.57, p = .002). R5-20 z-score (indicating small airway resistance) correlated negatively with role domain (r = -0.49, p = .03). SNOT-22 score correlated negatively with several PCD-QOL domains (lower respiratory symptoms r = -0.77, p < .001; physical r = -0.72, p < .001; upper respiratory symptoms r = -0.66, p < .001). No correlations were found between spirometry values, LCI, 6MWT, and PCD-QOL., Conclusions: FOT suggested small airway dysfunction, and correlated negatively with several PCD-QOL domains. Nasal symptoms had strong negative correlations with PCD-QOL. Larger longitudinal studies will further elucidate factors affecting QOL in PCD., (© 2023 The Authors. Pediatric Pulmonology published by Wiley Periodicals LLC.)

Background: Spinal Muscular Atrophy (SMA) is manifested by deformation of the chest wall, including a bell-shaped chest. We determined the ability of a novel non-ionizing, non-volitional method to measure and quantify bell-shaped chests in SMA., Methods: A 3D depth camera and a chest x-ray (CXR) were used to capture chest images in 14 SMA patients and 28 controls. Both methods measure the distance between two points, but measurements performed by 3D analysis allow for the consideration of the curve of a surface (geodesic measurements), whereas the CXR allows solely for the determination of the shortest path between two points, with no regard for the surface (Euclidean measurements). The ratio of the upper to lower chest distances was quantified to distinguish chest shape in imaging by both the 3D depth camera and the CXR, and the ratios were compared between healthy and SMA patients., Results: The mean 3D Euclidean ratio of distances measured by 3D imaging was 1.00 in the control group and 0.92 in the SMA group ( p  = 0.01), the latter indicative of a bell-shaped chest. This result repeated itself in the ratio of geodesic measurements (0.99 vs. 0.89, respectively, p  = 0.03)., Conclusion: The herein-described novel, noninvasive 3D method for measuring the upper and lower chest distances was shown to distinguish the bell-shaped chest configuration in patients with SMA from the chests of controls. This method bears several advantages over CXR and may be readily applicable in clinical settings that manage children with SMA., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (© 2024 Amirav, Rabin, Levi, Har-Even Cohn, Lior, Shiran, Sagi, Fatal, Zvirin, Honen, Lavie and Kimmel.)

The role of as-needed inhaled short-acting β 2 -agonists (SABAs) in the management of asthma has become a subject of debate due to differing opinions in the professional community relating to the use of SABAs. In this article, we summarize the current position of SABAs when used as reliever medications and examine the challenges to appropriate use including a critique of the data that have led to the condemnation of SABA used as a reliever. We consider the evidence for the appropriate use of SABA as a reliever together with practical solutions to ensure such use, including identifying patients at risk of misusing their SABA relievers and managing issues of inhaler technique and treatment adherence. We conclude that inhaled corticosteroid (ICS)-based maintenance treatment with SABA used as-needed as a reliever is an effective and safe treatment for patients with asthma, with no scientific evidence of a causal link between SABA use as a reliever and mortality or serious adverse events (including exacerbations). Increased SABA use warns of a deterioration in asthma control, and patients at risk of misusing their ICS and SABA medication should be rapidly identified to ensure they are receiving adequate ICS-based controller therapy. Appropriate use of ICS-based controller therapy and as-needed SABA should be encouraged and promoted with educational activities., (© 2023. The Author(s).)

Lockdown policies have been implemented worldwide to limit the spread of COVID-19. “Lockdown fatigue” is a state of exhaustion related to the accumulating effects of repeated lockdowns. The aim of the current study was to examine the effects of repeated lockdowns on children with respiratory disorders. Data on children aged 0−18 years old with respiratory disorders were collected by an electronic survey during the third lockdown in Israel. The retrieved information included demographics and baseline medical status, respiratory clinical status during the third lockdown compared to pre-lockdown periods, lockdown adherence, lifestyle modifications and caregiver emotional status. The results were compared to those of a similar questionnaire distributed during the first lockdown. A total of 234 patients (62% males, 37% females, mean age 6.8 years (confidence interval 6.1−7.5)) were enrolled. Respiratory symptoms and exacerbation frequency were reduced in 76 (35.5%) and 58 (27.4%) patients, respectively, compared to the pre-lockdown period and similar to the first lockdown (p = 0.840 and p = 0.063, respectively). However, compared to the first lockdown, the third lockdown was associated with greater use of reliever medications (p = 0.006), less use of inhaled corticosteroids as routine treatment (p = 0.027), and more pediatric emergency room visits and hospitalizations (p = 0.001 and p < 0.001, respectively). The results also showed an increase in sedentary lifestyle (e.g., reduced physical activity (p = 0.025), less healthy eating habits (p = 0.001)) and reduced adherence to lockdown policies (p < 0.001). These data show that the continuing stability of clinical improvement during lockdown was accompanied by deleterious sequelae that potentially indicate “lockdown fatigue” among children with respiratory disorders.