22nd February 2024

Mucociliary clearance defects

• Cystic fibrosis
• Kartagener Syndrome 
• Young syndrome

Kartagener syndrome is an autosomal recessive disorder characterised by situs inversus, bronchiectasis and chronic sinusitis. Symptoms manifest from A defect in the mucociliary escalator, resulting in patients with recurrent bacterial chest infections, otitis media, rhinosinusitis and infertility. Situs inversus is often identified on chest radiographs with dextrocardia seen as an incidental finding and does not have any clinical sequalae. Other signs of dextrocardia include ECG findings with positive P-wave and inverted T-wave in Lead I, tall R wave in aVR lead and poor R wave progression from Leads V1 – V6. 

Young syndrome is characterised by infertility due to azoospermia, rhinosinusitis and bronchiectasis.