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- 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