Israel Amirav
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View article: Respiratory effects of nusinersen treatment in pediatric patients with spinal muscular atrophy types 2 and 3
Respiratory effects of nusinersen treatment in pediatric patients with spinal muscular atrophy types 2 and 3 Open
View article: When Primary Ciliary Dyskinesia Is Diagnosed in Utero: Insights from Two Families
When Primary Ciliary Dyskinesia Is Diagnosed in Utero: Insights from Two Families Open
View article: Respiratory effects of nusinersen treatment in pediatric patients with spinal muscular atrophy types 2 and 3
Respiratory effects of nusinersen treatment in pediatric patients with spinal muscular atrophy types 2 and 3 Open
Introduction : Spinal muscular atrophy (SMA) is a progressive neuromuscular disorder characterized by respiratory decline. While nusinersen improves motor function, its long-term respiratory effects in milder SMA types (2 and 3) remain unc…
View article: Visual Measurements of Breathing Parameters in Children With a Particular Focus on Phase Angle: A Pilot Study
Visual Measurements of Breathing Parameters in Children With a Particular Focus on Phase Angle: A Pilot Study Open
View article: World Pneumonia Day 2024: Fighting Pneumonia and Antimicrobial Resistance
World Pneumonia Day 2024: Fighting Pneumonia and Antimicrobial Resistance Open
View article: Analyses of 1236 genotyped primary ciliary dyskinesia individuals identify regional clusters of distinct DNA variants and significant genotype–phenotype correlations
Analyses of 1236 genotyped primary ciliary dyskinesia individuals identify regional clusters of distinct DNA variants and significant genotype–phenotype correlations Open
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 defect…
View article: Work of Breathing: Physiology, Measurement, and Diagnostic Value in Childhood Pneumonia
Work of Breathing: Physiology, Measurement, and Diagnostic Value in Childhood Pneumonia Open
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 s…
View article: Non-ionizing measurement and quantification of bell-shaped chests in spinal muscular atrophy: a pilot study
Non-ionizing measurement and quantification of bell-shaped chests in spinal muscular atrophy: a pilot study Open
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 …
View article: Pulmonary functions, nasal symptoms, and quality of life in patients with primary ciliary dyskinesia (PCD)
Pulmonary functions, nasal symptoms, and quality of life in patients with primary ciliary dyskinesia (PCD) Open
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 prospect…
View article: SABAs as Reliever Medications in Asthma Management: Evidence-Based Science
SABAs as Reliever Medications in Asthma Management: Evidence-Based Science Open
The role of as-needed inhaled short-acting β2-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 …
View article: Pediatric inhalation therapy and the aerodynamic rationale for age-based aerosol sizes
Pediatric inhalation therapy and the aerodynamic rationale for age-based aerosol sizes Open
View article: Primary Ciliary Dyskinesia Genotypes Vary Markedly Across 19 Countries and Identify Significant Genotype-Phenotype Correlations
Primary Ciliary Dyskinesia Genotypes Vary Markedly Across 19 Countries and Identify Significant Genotype-Phenotype Correlations Open
View article: Non-ionizing measurement and quantification of bell-shaped chests in spinal muscular atrophy: A pilot study
Non-ionizing measurement and quantification of bell-shaped chests in spinal muscular atrophy: A pilot study Open
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 …
View article: Lockdown Fatigue in Pediatric Respiratory Patients: Lessons from the First COVID-19 Year
Lockdown Fatigue in Pediatric Respiratory Patients: Lessons from the First COVID-19 Year Open
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 effec…
View article: Fractional exhaled Nitric Oxide (FeNO) level as a predictor of COVID-19 disease severity
Fractional exhaled Nitric Oxide (FeNO) level as a predictor of COVID-19 disease severity Open
View article: Asthma risk after a pediatric intensive care unit admission for respiratory syncytial virus bronchiolitis
Asthma risk after a pediatric intensive care unit admission for respiratory syncytial virus bronchiolitis Open
Background Admission to a pediatric intensive care unit (PICU) has been associated with respiratory consequences in children with asthma and carries major implications for management control. Whereas respiratory syncytial virus (RSV) bronc…
View article: Unforeseen changes in seasonality of pediatric respiratory illnesses during the first COVID‐19 pandemic year
Unforeseen changes in seasonality of pediatric respiratory illnesses during the first COVID‐19 pandemic year Open
Objectives To investigate whether the three nationwide coronavirus disease 2019 (COVID‐19) lockdowns imposed in Israel during the full first pandemic year altered the traditional seasonality of pediatric respiratory healthcare utilization.…
View article: Lung function from school age to adulthood in primary ciliary dyskinesia
Lung function from school age to adulthood in primary ciliary dyskinesia Open
Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes lung function trajectories in chi…
View article: Translation of the quality of life questionnaire for primary ciliary dyskinesia (QOL‐PCD) into Hebrew: The Israeli experience
Translation of the quality of life questionnaire for primary ciliary dyskinesia (QOL‐PCD) into Hebrew: The Israeli experience Open
Background Patients with primary ciliary dyskinesia (PCD) have chronic morbidities affecting their quality of life (QOL). Health‐related QOL (HRQOL) has been recognized as an important tool for assessing the burden of PCD on patients and t…
View article: Spurious Asthma Presentation during COVID-19
Spurious Asthma Presentation during COVID-19 Open
The use of face masks as a means for preventing the spread of SARS-CoV-2 is now a common practice world-wide. Three children presented to our specialty clinic with respiratory complaints during protective face mask wearing. They were diagn…
View article: Expanding clinical phage microbiology: simulating phage inhalation for respiratory tract infections
Expanding clinical phage microbiology: simulating phage inhalation for respiratory tract infections Open
Phage therapy is a promising antibacterial strategy for resistant respiratory tract infections. Phage inhalation may serve this goal; however, it requires a careful assessment of their delivery by this approach. Here we present an in vitro…
View article: Lung function from school age to adulthood in primary ciliary dyskinesia
Lung function from school age to adulthood in primary ciliary dyskinesia Open
Primary ciliary dyskinesia (PCD) presents with symptoms early in life and the disease course may be progressive, but longitudinal data on lung function are scarce. This multinational cohort study describes lung function trajectories in chi…
View article: Expanding Clinical Phage Microbiology: Simulating Phage Inhalation for Respiratory Tract Infections
Expanding Clinical Phage Microbiology: Simulating Phage Inhalation for Respiratory Tract Infections Open
Phage therapy is a promising antibacterial strategy for resistant respiratory tract infections. Phage inhalation may serve this goal; however, it requires a careful assessment of their delivery by this approach. Here we present an in-vitro…
View article: Pulse oximetry is an essential tool that saves lives: a call for standardisation
Pulse oximetry is an essential tool that saves lives: a call for standardisation Open
Pneumonia is a leading global cause of morbidity and mortality, particularly amongst adults aged >70 years and children. Annual deaths due to pneumonia in these groups was estimated at more than one million and 672 000 worldwide for both g…
View article: Hope During COVID-19 Lockdown
Hope During COVID-19 Lockdown Open
Introduction The COVID-19 pandemic has changed daily life in unexpected ways including strict lockdowns periods that may shape hope. Method This study compared hope levels among an online sample of 584 adults in late April 2020 during the …
View article: Real‐time effects of COVID‐19 pandemic lockdown on pediatric respiratory patients
Real‐time effects of COVID‐19 pandemic lockdown on pediatric respiratory patients Open
Objective A national lockdown was implemented in Israel to slow the viral spread of COVID‐19. We assessed the real‐time effects of the lockdown on disease expression and lifestyle modifications in pediatric patients with chronic respirator…
View article: CFAP45 deficiency causes situs abnormalities and asthenospermia by disrupting an axonemal adenine nucleotide homeostasis module
CFAP45 deficiency causes situs abnormalities and asthenospermia by disrupting an axonemal adenine nucleotide homeostasis module Open
Axonemal dynein ATPases direct ciliary and flagellar beating via adenosine triphosphate (ATP) hydrolysis. The modulatory effect of adenosine monophosphate (AMP) and adenosine diphosphate (ADP) on flagellar beating is not fully understood. …
View article: <p>Asthma and COVID-19: In Defense of Evidence-Based SABA</p>
Asthma and COVID-19: In Defense of Evidence-Based SABA Open
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 li…
View article: Asthma and COVID-19: In Defense of Evidence-Based SABA
Asthma and COVID-19: In Defense of Evidence-Based SABA Open
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: Isr…
View article: Whole-exome sequencing accuracy in the diagnosis of primary ciliary dyskinesia
Whole-exome sequencing accuracy in the diagnosis of primary ciliary dyskinesia Open
The diagnosis of primary ciliary dyskinesia (PCD) relies on clinical features and sophisticated studies. The detection of bi-allelic disease-causing variants confirms the diagnosis. However, a standardised genetic panel is not widely avail…