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.

Links

Related articles

• Chronic lung diseases

References

• BENEŠ, Jiří. Studijní materiály [online]. [cit. 2010]. <http://jirben.wz.cz>.

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