A5008668894- Cystic Fibrosis Research Advances
- Tracheal and airway disorders
- Muscle Physiology and Disorders
- Neonatal Respiratory Health Research
- Congenital Diaphragmatic Hernia Studies
- Pneumonia and Respiratory Infections
- Neurogenetic and Muscular Disorders Research
- Respiratory viral infections research
- Cytomegalovirus and herpesvirus research
- Inhalation and Respiratory Drug Delivery
- Foreign Body Medical Cases
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Airway Management and Intubation Techniques
- Pleural and Pulmonary Diseases
- Nuclear Structure and Function
- Biotin and Related Studies
- Genomics and Rare Diseases
- RNA Research and Splicing
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Hepatitis Viruses Studies and Epidemiology
- Epigenetics and DNA Methylation
- Cardiomyopathy and Myosin Studies
- Single-cell and spatial transcriptomics
- Muscle and Compartmental Disorders
- Growth Hormone and Insulin-like Growth Factors
Hadassah Medical Center
2017-2023
Hebrew University of Jerusalem
2021-2023
University Medical Center
2022
The University of Sydney
2018
Alfred Health
2018
Rationale: Ivacaftor's clinical effects in the residual function mutations 3849 + 10kb C→T and D1152H warrant further characterization.Objectives: To evaluate ivacaftor's effect people with cystic fibrosis aged ≥6 years or to explore correlation between ivacaftor-induced organoid-based transmembrane conductance regulator measurements response ivacaftor.Methods: Participants were randomized (1:1) this placebo-controlled crossover study; each treatment sequence included two 8-week treatments...
The diagnosis of primary ciliary dyskinesia (PCD) relies on clinical features and sophisticated studies. detection bi-allelic disease-causing variants confirms the diagnosis. However, a standardised genetic panel is not widely available new genes are continuously identified. To assess accuracy untargeted whole-exome sequencing (WES) as diagnostic tool for PCD, patients with symptoms highly suggestive PCD were consecutively included. Patients underwent measurement nasal nitric oxide (nNO)...
Abstract Rationale Necrotizing pneumonia is characterized by destruction and liquefaction of the lung tissue loss normal pulmonary parenchymal architecture. During course resolution areas hyperlucency are formed, sometimes with development giant cysts that can be a field fluid resembling abscess. There no consensus on management these abnormalities. Objective To assess prevalence as complication necrotizing to report our experience conservative treatment achieved complete resolution. Methods...
Abstract Background Normal values (>80%) of Forced Expiratory Volume in one second (FEV 1 ) patients with cystic fibrosis (CF) may lead to the interpretation that there is no lung disease. This study a comprehensive analysis involvement CF having normal FEV . Methods Patients were recruited from two centers: Hadassah Medical Center, Jerusalem and Vall d' Hebron Hospital, Barcelona. Lung disease was assessed by clearance index (LCI), chest CT‐Brody score, respiratory cultures, number...
Abstract Background Since the outbreak of coronavirus disease 2019 (COVID‐19) pandemic, there has been a decline in pediatric emergency department visits. Our aim was to assess pattern foreign body aspiration (FBA) during first year COVID‐19 comparison prior years. Methods In this retrospective multicenter study, we compared number children who presented with FBA (March 1, 2020 February 28, 2021) annual average years 2016–2019. We also lockdown periods postlockdown periods, and percentage...
Abstract Herpes simplex virus (HSV) is rarely the cause of pneumonia in immunocompetent patients. We describe a previously healthy child, with no evidence an immunodeficiency, who presented to emergency department severe pneumonia, wheezing, and pleural effusions history orolabial HSV infection. On admission, he was started on antibiotics systemic corticosteroids but continued deteriorate. Oral lesions, blood, fluid tested positive for HSV, improvement achieved only after addition acyclovir...
Consistently abnormal glucose levels on oral tolerance test (OGTT) are the most effective screening tool for cystic fibrosis-related diabetes (CFRD). However, some fibrosis (CF) patients demonstrate profiles not reaching required CFRD diagnosis and are, therefore, left untreated. Since is associated with disease deterioration, early treatment desirable.To explore association between area under curve of (G-AUC) obtained during a five-point 2-h standard OGTT CF severity parameters.All referred...
Abstract Background Pulmonary disease is the leading cause of morbidity and mortality in people with cystic fibrosis (pwCF). Several studies have shown no benefit for bronchoscopy bronchoalveolar lavage (BAL) over sputum to obtain microbiological cultures, hence role pwCF unclear. Aim To analyze how results affected clinical decision‐making assess safety. Methods A retrospective analysis all charts from three CF centers Israel, between years 2008 2019. We collected BAL culture as well...
Background: Since the outbreak of Coronavirus disease 2019 (COVID-19) pandemic, there has been a decline in pediatric emergency department visits. Our aim was to assess pattern foreign body aspiration (FBA) during first year COVID-19, comparison prior years. Methods: In this retrospective multicenter study, we compared number children that presented with FBA COVID-19 (March 1st, 2020 February 28, 2021), annual average years 2016-2019. We also lockdown periods post-lockdown and percentage...
Abstract Background: Duchenne Muscular Dystrophy (DMD) is the most common form of inherited muscle disease in childhood. DMD patients have severe growth retardation due to several reasons including chronic treatment with glucocorticoid (GC). Data regarding efficacy and safety Recombinant Growth Hormone (rGH) very limited. The aim this study evaluate hormone 4 boys induced failure. Methods Results: prepubertal on high dose GC (Deflazecort or Prednisone) at an age range between 12-13.6 years...