eMedicine Specialties > Pediatrics: General Medicine > Pulmonology
Primary Ciliary Dyskinesia: Follow-up
Updated: Sep 25, 2008
Follow-up
Prognosis
- The progression of lung disease varies and is affected by the time of diagnosis, the ability of medical treatment to control symptoms, and the prevention of complications that affect the quality of life.
- Some individuals have a normal or near normal lifespan. No studies have examined the impact of current symptomatic therapies on the course of disease.
Patient Education
- Genetic counseling should be offered to parents of newly diagnosed infants and children. The importance of regular health monitoring should be emphasized. Counsel patients to avoid smoke, allergens, environmental irritants, and exposure to respiratory pathogens.
- For excellent patient education resources, visit eMedicine's Procedures Center. Also, see eMedicine's patient education article Bronchoscopy.
More on Primary Ciliary Dyskinesia |
| Overview: Primary Ciliary Dyskinesia |
| Differential Diagnoses & Workup: Primary Ciliary Dyskinesia |
| Treatment & Medication: Primary Ciliary Dyskinesia |
Follow-up: Primary Ciliary Dyskinesia |
| Multimedia: Primary Ciliary Dyskinesia |
| References |
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References
Kartagener M. Zur pathogene der bronkiectasein:bronkiectasein bei situs viscerum inversus. Beitr Klin Tuberk. 1933;82:489.
Kartagner M. Zur pathogenese der bronkiectasein. I Mittelung: Bronkiectasein bei situs viscerum invesus. Beitr Klin Tuberk. 1933;83:498-501.
Afzelius BA. A human syndrome caused by immotile cilia. Science. Jul 23 1976;193(4250):317-9. [Medline].
Carson JL, Collier AM, Hu SS. Acquired ciliary defects in nasal epithelium of children with acute viral upper respiratory infections. N Engl J Med. Feb 21 1985;312(8):463-8. [Medline].
Pedersen M. Ciliary activity and pollution. Lung. 1990;168 Suppl:368-76. [Medline].
Sturgess JM, Chao J, Turner JA. Transposition of ciliary microtubules: another cause of impaired ciliary motility. N Engl J Med. Aug 7 1980;303(6):318-22. [Medline].
Chilvers MA, Rutman A, O'Callaghan C. Ciliary beat pattern is associated with specific ultrastructural defects in primary ciliary dyskinesia. J Allergy Clin Immunol. Sep 2003;112(3):518-24. [Medline].
Afzelius BA, Eliasson R. Male and female infertility problems in the immotile-cilia syndrome. Eur J Respir Dis Suppl. 1983;127:144-7. [Medline].
Pennarun G, Escudier E, Chapelin C, et al. Loss-of-function mutations in a human gene related to Chlamydomonas reinhardtii dynein IC78 result in primary ciliary dyskinesia. Am J Hum Genet. Dec 1999;65(6):1508-19. [Medline].
Guichard C, Harricane MC, Lafitte JJ, et al. Axonemal dynein intermediate-chain gene (DNAI1) mutations result in situs inversus and primary ciliary dyskinesia (Kartagener syndrome). Am J Hum Genet. Apr 2001;68(4):1030-5. [Medline].
Hornef N, Olbrich H, Horvath J, et al. DNAH5 mutations are a common cause of primary ciliary dyskinesia with outer dynein arm defects. Am J Respir Crit Care Med. Jul 15 2006;174(2):120-6. [Medline].
Bush A, Ferkol T. Movement: the emerging genetics of primary ciliary dyskinesia. Am J Respir Crit Care Med. Jul 15 2006;174(2):109-10. [Medline].
Santamaria F, Montella S, Tiddens HA, et al. Structural and functional lung disease in primary ciliary dyskinesia. Chest. Aug 2008;134(2):351-7. [Medline].
Corbelli R, Bringolf-Isler B, Amacher A, Sasse B, Spycher M, Hammer J. Nasal nitric oxide measurements to screen children for primary ciliary dyskinesia. Chest. Oct 2004;126(4):1054-9. [Medline].
Karadag B, James AJ, Gultekin E, Wilson NM, Bush A. Nasal and lower airway level of nitric oxide in children with primary ciliary dyskinesia. Eur Respir J. Jun 1999;13(6):1402-5. [Medline].
Noone PG, Leigh MW, Sannuti A, et al. Primary ciliary dyskinesia: diagnostic and phenotypic features. Am J Respir Crit Care Med. Feb 15 2004;169(4):459-67. [Medline].
Further Reading
Keywords
primary ciliary dyskinesia, PCD, immotile cilia syndrome, ICS, cilia, dyskinetic cilia syndrome, immotile cilia syndrome, Kartagener syndrome, situs inversus totalis, respiratory infections, sinusitis, otitis media, male infertility, chronic sinusitis, bronchiectasis, ciliary dyskinesia syndrome, CDS, male infertility, rhinitis, pneumonia, dextrocardia, rhinorrhea, anosmia, halitosis, hydrocephalus, atelectasis, nasal mucosal congestion, mucopurulent nasal discharge, nasal obstruction, nasal polyps
Follow-up: Primary Ciliary Dyskinesia