triad of chronic sinusitis, bronchiecctasis, and situs inversus
recurrent acute otitis media and nasl polyps also common
syndrome is due to an ultrastructural abnormality of the cilia in which dynein arms are absent resulting in ciliary dysfunction and mucus stasis and frequent sinopulmonary infections
situs inversus is due to the absence of the influence of cilia function on viscera development
diagnosed by bronchoscopy for cilia biopsy
References
Haddad GG. Primary ciliary dyskinesia. In: Behrman RE, Kliegman RM, Jenson HB, eds. Nelson Textbook of Pediatrics. 16th ed. Philadelphia, Pa: WB SaundersCo; 2000:1327-1328
