Primary ciliary dyskinesia




 * Older name: immobile cilia syndrome; OMIM: 244400
 * Pathogenesis - AR hereditary disease, generalized disorder of motility of cilia and flagella of all cells;
 * the movement is completely absent or the movement is uncoordinated;
 * was originally described in Cartagener's Triassic (bronchiectasis, sinusitis, situs viscerum inversus).

Clinical picture

 * Impaired self-cleaning ability of the epithelium, productive expectoration and bronchiectasis occur.
 * Other symptoms - sinusitis, chronic rhinitis, rhinorrhea, nasal polyps, recurrent otitis] otitis, conduction disorder hearing u, tympanic perforation, DC obstruction, recurrent pneumonia, infertility.
 * Situs viscerum inversus is only about 50%.
 * Diagnosis - complicated confirmation, it is necessary to know the result of electron microscopy and high-speed video microscopy (HSVM).

Therapy

 * Symptomatic, similar to other bronchiectasis.

Related articles

 * Chronic lung diseases