Rationale: Genotype–phenotype relationships are emerging in primary ciliary dyskinesia
(PCD), but little is known about lung volume changes over time. Objectives: To investigate …

Rationale: Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary
clearance, recurrent respiratory infections, progressive airway damage, and obstructive lung …

Rationale: In primary ciliary dyskinesia, factors leading to disease heterogeneity are poorly
understood. Objectives: To describe early lung disease progression in primary ciliary …

Results One hundred and thirty-five patients≥ 5 years of age were enrolled. There were
1,155 study visits, with a mean 9 visits per patient (range, 2-20); 24 participants completed …

Rationale: The relationship between clinical phenotype of childhood primary ciliary
dyskinesia (PCD) and ultrastructural defects and genotype is poorly defined. Objectives: To …

Rationale: Primary ciliary dyskinesia (PCD) is an inherited disorder characterized by
heterogeneous airway disease. Traditional lung function techniques (eg, spirometry) may …

Purpose Primary ciliary dyskinesia (PCD) is characterised by repeated upper and lower
respiratory tract infections, neutrophilic airway inflammation and obstructive airway disease …

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 …

Background: Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary
clearance that results in accumulation of mucus and bacteria in the airways. Lower …

Background No studies longitudinally, simultaneously assessed body mass index (BMI) and
spirometry in primary ciliary dyskinesia (PCD). Methods We determined BMI and spirometry …