<strong>Chest</strong> & <strong>Heart</strong> Journal Vol. <strong>35</strong>, <strong>No</strong>.-2, July <strong>2011</strong>improvement in life expectancy by chestphysiotherapy & judicious use <strong>of</strong> antibiotics. Genetherapy may be an option <strong>of</strong> treatment in nearfuture.So in Kartagenar’s syndrome as involvement &severity are quite variable.Persons with PCD live an active life. <strong>The</strong> rate <strong>of</strong>decline <strong>of</strong> lung function is much slower than thatin cystic fibrosis. But delayed diagnosis <strong>and</strong>improper treatment results poorer outcome. Sowe must give due priority for evaluation <strong>of</strong>bronchiectasis to exclude PCD.References:1. Afzelius BA. A human syndrome caused byimmotile cilia. Science 1976; 193:317–319.2. Mossberg B, Afzelius BA, Eliasson R,Camner P. On the pathogenesis <strong>of</strong>obstructive lung disease: a study on theimmotile cilia syndrome. Sc<strong>and</strong> J Respir Dis1978; 59:55–65.3. Afzelius BA. Inheritance <strong>of</strong> r<strong>and</strong>omness. MedHypotheses 1996; 47:23–26.4. <strong>No</strong>one PG, Bali D, Carson JL, Sannuti A,Gipson CL, Ostrowski LE, Bromberg PA,Boucher RC, Knowles MR. Discordant organlaterality in monozygotic twins with primaryciliary dyskinesia. Am J Med Genet 1999;82:155–160.5. Torgersen J. Situs inversus, asymmetry, <strong>and</strong>twinning. Is J Hum Genet 1950; 2:361–370.6. Katsuhara K, Kawamoto S, Wakabayashi T,Belsky JL. Situs inversus totalis <strong>and</strong>Kartagener’s syndrome in a Japanesepopulation. <strong>Chest</strong> 1972; 61:56–61.7. de Iongh RU, Rutl<strong>and</strong> J. Ciliary defects inhealthy subjects, bronchiectasis, <strong>and</strong> primaryciliary dyskinesia. Am J Respir Crit Care Med1995; 151:1559–1567.8. Bush A, Cole P, Hariri M, Mackay I, PhillipsG, O’Callaghan C, Wilson R, Warner JO.Primary ciliary dyskinesia: diagnosis <strong>and</strong>st<strong>and</strong>ards <strong>of</strong> care. Eur Respir J 1998; 12:982–988.9. Escudier E, Couprie M, Duriez B, Roudot-Thoraval F, Millepied MC, Pruliere-EscabasseV, Labatte L, Coste A. Computer-assistedanalysis helps detect inner dynein armabnormalities. Am J Respir Crit Care Med2002; 166:1257–1262.10. Coren ME, Meeks M, Morrison I, BuchdahlRM, Bush A. Primary ciliary dyskinesia: ageat diagnosis <strong>and</strong> symptom history. ActaPaediatr 2002; 91:667–669.11. Siewert A. Uber einen fall vonbronchiektasien bei einen patienten mit situsinversus viscerum. Klin Wochenschr 1904;41:139.12. Kartagener M. Zur pathogenese derbronchiektasien bei situs visceruminversus.Beitr Klin Tuberk 1933; 83:489.13. Pederson H, Rebbe H. Absence <strong>of</strong> arms inthe axoneme <strong>of</strong> immobile humanspermatozoa. Biol Report 1975:12:541.14. Pederson H, Mygind N. Absence <strong>of</strong> axonemalarms in nasal mucosa cilia in Kartagener’ssyndrome. Nature 1976; 262:494.15. Afzelius BA.A human syndrome caused byimmotile cilia. Science 1976; 193:317.16. Eliasson R, Mossberg B, Cammer P, AfzeliusBA. <strong>The</strong> immotile cilia syndrome: acongenital ciliary abnormality as an etiologicfactor in chronic airway infections <strong>and</strong> malesterility. N Engl J Med 1977; 297:1.17. Chodhari, R; Mitchison, Hm; Meeks, M(March 2004). “Cilia, primary ciliarydyskinesia <strong>and</strong> molecular genetics”.Paediatric respiratory reviews 5 (1): 69–76.doi:10.1016/j.prrv.2003.09.005.PMID 15222957.18. Gene Reviews. “Primary Ciliary Dyskinesia”.http://www.genetests.org/servlet/access?db=geneclinics& site= gt & id= 8888890&key=ujnJJL1T9E6XN&gry= &fcn= y&fw=8zBM&filename = /pr<strong>of</strong>iles/pcd/index.html.Retrieved 2007-11-16 s.19. Zariwala, Ma; Knowles, Mr; Omran, H (2007).“Genetic defects in ciliary structure <strong>and</strong>function”. Annual review <strong>of</strong> physiology 69:423–50. doi:10.1146/annurev. physiol.69.040705.141301. PMID 17059<strong>35</strong>8.138
A Man with Chronic Cough – Evaluated As Kartagenar’s SyndromeMK Uddin et al.20. Afzelius BA. Immotile Cilia syndrome past,present <strong>and</strong> prospects for the future. Thorax1998:53:894.21. Lundberg JO, Weitzberg E, <strong>No</strong>rdvall SL,Kuylenstierna R, Lundberg JM, Alving K.Primarily nasal origin <strong>of</strong> exhaled nitric oxide<strong>and</strong> absence in Kartagener’s syndrome. EurRespir J 1994; 7:1501–1504.22. Wodehouse T, Kharitonov SA, Mackay IS,Barnes PJ, Wilson R, Cole PJ. Nasal nitricoxide measurements for the screening <strong>of</strong>primary ciliary dyskinesia. Eur Respir J 2003;21:43–47.23. Zariwala M, <strong>No</strong>one PG, Sannuti A, Minnix S,Zhou Z, Leigh MW, Hazucha M, Carson JL,Knowles MR. Germline mutations in anintermediate chain dynein cause primaryciliary dyskinesia. Am J Respir Cell Mol Biol2001; 25:577–583.24. Narang I, Ersu R, Wilson NM, Bush A. Nitricoxide in chronic airway inflammation inchildren: diagnostic use <strong>and</strong> pathophysiologicsignificance. Thorax 2002; 57:586–589.25. Wendy A. Stannard Et el. Diagnostic Testing<strong>of</strong> Patients Suspected <strong>of</strong> Primary CiliaryDyskinesia. Am J Respir Crit Care Med 181.pp. 307-314, (2010)139
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