Primary ciliary dyskinesia: Difference between revisions
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* Toto upozornění se vkládá šablonou {{subst:Vložený článek}} | * Toto upozornění se vkládá šablonou {{subst:Vložený článek}} | ||
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Older name: immobile cilia syndrome; OMIM: [http://omim.org/entry/244400 244400] | |||
* Older name: immobile cilia syndrome; OMIM: [http://omim.org/entry/244400 244400] | |||
* Pathogenesis - [[AR | AR hereditary]] disease, generalized disorder of motility of cilia and flagella of all cells; | * Pathogenesis - [[AR | AR hereditary]] disease, generalized disorder of motility of cilia and flagella of all cells; | ||
** the movement is completely absent or the movement is uncoordinated; | ** the movement is completely absent or the movement is uncoordinated; | ||
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== Links == | == Links == | ||
=== Related articles === | === Related articles === |
Revision as of 16:23, 10 December 2021
- 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
References
- BENEŠ, Jiří. Studijní materiály [online]. [cit. 2010]. <http://jirben.wz.cz>.