Pulmonary clearance (Pcl) of aerosolized 99mTc-DTPA was studied in fourteen patients with primary ciliary dyskinesia (PCD), (median age 23.5 yrs, range 12-44 yrs) and nine normal individuals (median age 23 yrs, range 18-27 yrs). All had never smoked. Regional Pcl was studied for arbitrarily defined central and peripheral regions of the lung using a gamma camera method, whilst total Pcl was stud...

Nitric oxide is continually synthesised in the respiratory epithelium and is upregulated in response to infection or inflammation. Primary ciliary dyskinesia (PCD) is characterised by recurrent sinopulmonary infections due to impaired mucociliary clearance. Despite chronic infections, nasal nitric oxide in such patients is markedly reduced and is used as a screening test for this condition. The...

Non-cystic fibrosis bronchiectasis again becomes a major health problem due to inappropriate antibiotic use and increasing frequency of protracted bacterial bronchitis. The aim was to determine the changes in etiology of bronchiectasis. Patients who admitted to Behçet Uz Children Hospital between 2005 and 2015 (n=110) were retrospectively examined. The etiology of bronchiectasis was detected as...

Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive disease of abnormalities of ciliary structure and function. The result is impaired mucociliary clearance, causing a variety of respiratory symptoms, and likely progression to bronchiectasis in most cases. Situs anomalies are present in nearly 50% of cases.

Ciliary motion defects cause defective mucociliary transport (MCT) in primary ciliary dyskinesia (PCD). Current diagnostic tests do not assess how MCT is affected by perturbation of ciliary motion. In this study, we sought to use micro-optical coherence tomography (μOCT) to delineate the mechanistic basis of cilia motion defects of PCD genes by functional categorization of cilia motion. Trachea...

BACKGROUND The diagnosis of primary ciliary dyskinesia (PCD) requires the analysis of ciliary function and ultrastructure. Diagnosis can be complicated by secondary effects on cilia such as damage during sampling, local inflammation or recent infection. To differentiate primary from secondary abnormalities, re-analysis of cilia following culture and re-differentiation of epithelial cells at an ...

OBJECTIVE To assess the results of tympanoplasty in children with primary ciliary dyskinesia complicated by tympanic perforation or cholesteatoma with hearing loss and/or recurrent otorrhea. DESIGN Retrospective study. Postoperative follow-up of 26.3 months in the type 1 tympanoplasty group and 46 months in the child with cholesteatoma. SETTING Hospitalized care, referral center. PATIENTS...

Affiliations: Institute of Social and Preventive Medicine, University of Bern, Bern, Switzerland. Paediatric Respiratory Medicine, Children’s University Hospital of Bern, University of Bern, Bern, Switzerland. Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK. NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton ...