Primary ciliary dysfunction

PRIMARY CILIARY DYSFUNCTION
Originally described in respiratory tract and sperm of pts with KARTAGENER SYNDROME (situs inversus, sinusitis, and bronchiectasis), but situs inversus only present in 50% pts with dyskinetic cilia.
Autosomal recessive inheritance.
Nasal nitric oxide test is used as screening test.
Electron microscopy of nasal or bronchial biopsy is confirmatory.
Cell culture and high speed videomicroscopy analysis are other confirmatory tests.
Treatment is symptomatic.