Primary ciliary dyskinesia in the genomics age
Primary ciliary dyskinesia is a genetically and clinically heterogeneous syndrome. Impaired
function of motile cilia causes failure of mucociliary clearance. Patients typically present with …
function of motile cilia causes failure of mucociliary clearance. Patients typically present with …
[HTML][HTML] Non-cystic fibrosis bronchiectasis in children and adolescents: Neglected and emerging issues
M Poeta, M Maglione, M Borrelli, F Santamaria - Pediatrics & Neonatology, 2020 - Elsevier
Pediatric non-cystic fibrosis (CF) bronchiectasis is characterized by endobronchial
suppuration, airway neutrophilic inflammation and poor mucus clearance and is associated …
suppuration, airway neutrophilic inflammation and poor mucus clearance and is associated …
Structural and functional lung impairment in primary ciliary dyskinesia. Assessment with magnetic resonance imaging and multiple breath washout in comparison to …
S Nyilas, G Bauman, O Pusterla, G Sommer… - Annals of the …, 2018 - atsjournals.org
Rationale: Primary ciliary dyskinesia (PCD) is an inherited disorder characterized by
heterogeneous airway disease. Traditional lung function techniques (eg, spirometry) may …
heterogeneous airway disease. Traditional lung function techniques (eg, spirometry) may …
Image Phenotyping of Preterm-Born Children Using Hyperpolarized 129Xe Lung Magnetic Resonance Imaging and Multiple-Breath Washout
Rationale: Preterm birth is associated with low lung function in childhood, but little is known
about the lung microstructure in childhood. Objectives: We assessed the differential …
about the lung microstructure in childhood. Objectives: We assessed the differential …
Magnetic resonance imaging of pulmonary and paranasal sinus abnormalities in children with primary ciliary dyskinesia compared to children with cystic fibrosis
L Wucherpfennig, F Wuennemann… - Annals of the …, 2024 - atsjournals.org
Rationale: Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are characterized by
inherited impaired mucociliary clearance leading to chronic progressive lung disease as …
inherited impaired mucociliary clearance leading to chronic progressive lung disease as …
[HTML][HTML] Primary Ciliary Dyskinesia: A Clinical Review
KA Despotes, MA Zariwala, SD Davis, TW Ferkol - Cells, 2024 - mdpi.com
Primary ciliary dyskinesia (PCD) is a rare, genetically heterogeneous, motile ciliopathy,
characterized by neonatal respiratory distress, recurrent upper and lower respiratory tract …
characterized by neonatal respiratory distress, recurrent upper and lower respiratory tract …
[HTML][HTML] Lung function in children with primary ciliary dyskinesia
VA Ferraro, RJ Castaldo, V Tonazzo, S Zanconato… - Children, 2023 - mdpi.com
Background: Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary
clearance that results in accumulation of mucus and bacteria in the airways. Lower …
clearance that results in accumulation of mucus and bacteria in the airways. Lower …
[HTML][HTML] Lower airway clinical outcome measures for use in primary ciliary dyskinesia research: a scoping review
F Gahleitner, J Thompson, CL Jackson… - ERJ Open …, 2021 - Eur Respiratory Soc
Objectives Disease-specific, well-defined and validated clinical outcome measures are
essential in designing research studies. Poorly defined outcome measures hamper pooling …
essential in designing research studies. Poorly defined outcome measures hamper pooling …
Structural and Functional Lung Impairment in PCD: Assessment with MRI and Multiple Breath Washout in Comparison to Spirometry.
SM Nyilas, G Bauman, O Pusterla… - Annals of the …, 2018 - boris.unibe.ch
RATIONALE Primary ciliary dyskinesia (PCD) is an inherited disorder characterized by
heterogeneous airway disease. Traditional lung function techniques (eg spirometry) may …
heterogeneous airway disease. Traditional lung function techniques (eg spirometry) may …
[HTML][HTML] Simultaneous multiple breath washout and oxygen-enhanced magnetic resonance imaging in healthy adults
Lung function testing and lung imaging are commonly used techniques to monitor
respiratory diseases, such as cystic fibrosis (CF). The nitrogen (N 2) multiple-breath washout …
respiratory diseases, such as cystic fibrosis (CF). The nitrogen (N 2) multiple-breath washout …