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Kartagener syndrome (sinusitis-bronchiectasis-situs inversus syndrome; bronchiectasis-dextrocardia-sinusitis; kartagener triad) 6


Kartagener Syndrome (Sinusitis-Bronchiectasis-Situs Inversus Syndrome; Bronchiectasis-Dextrocardia-Sinusitis; Kartagener Triad) 555h75f 555h75f 555h75f 555h75f 555h75f 6

General: Autosomal recessive; onset in early infancy; occasionally dominant; finding of various structural defects in patients with this condition suggests that there are several genetic determinants.

Ocular: Myopia; glaucoma; conjunctival melanosis; iris coloboma; tortuous and dilated retinal vessels; retinal pigmentary degeneration; pseudopapillitis.

Clinical: Immotile cilia; situs inversus; bronchiectasis; sinusitis; various cardiovascular and renal abnormalities; dyspnea; productive cough; recurrent respiratory infections; palpitation; otitis media; nasal speech; conductive hearing loss; nasal polyps; situs inversus viscerum with hepatic dullness on left side.

Kartagener M. Zuir Pathogenese der Bronchiektasien, Bronchrektasien bei Situs Inversus. Beitr Klin Tbk

Magalini SI, Scrascia E. Dictionary of Medical Syndromes, 2nd ed. Philadelphia: JB Lippincott, 1981.

McKusick VA. Mendelian Inheritance in Man; A Catalog of Human Genes and Genetic Disorders, 12th ed. Baltimore: The Johns Hopkins University Press, 1998.

Online Mendelian Inheritance in Man, OMIM. McKusick-Nathans Institute for Genetic Medicine, Johns Hopkins University and National Center for Biotechnology Information, National Library of Medicine, February 12, 2007. World Wide Web URL: https://www.ncbi.nlm.nih.gov/omim/.

Sturgess JM, et al. Genetic aspects of immotile cilia syndrome. Am J Med Genet 1986; 25:149-l60.




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