Abstract
Kartagener's syndrome is a condition that consists of situs inversus, bronchiectasis, and sinusitis. Some patients have respiratory symptoms that date from early infancy, and electron microscopical examination has shown that adults with this condition lack dynein arms in ciliary microtubules. It has been suggested that an inherited defect in ciliary ultrastructure, the immotile cilia, is the basis for the syndrome. We report 6 patients who presented within the first 24 hours of life with tachypnoea, retraction, rales, and dextrocardia. Five of them had situs inversus. They all responded to physiotherapy after antibiotics had failed to improve their condition. Electron microscopical examination of the cilia in 2 of the infants confirmed deficiency of dynein arms. All 6 infants had recurrent respiratory infections. Four went on to develop recurrent otitis media, 2 had sinusitis, and 1 now has bronchiectasis. The immotile cilia syndrome should be added to the list of causes of respiratory distress in newborn infants. The condition is worth diagnosing because of the good response to physiotherapy, and because of the likelihood of recurrent respiratory problems.
References
Jul 7, 1977·The New England Journal of Medicine·R EliassonB A Afzelius
Apr 1, 1971·American Journal of Diseases of Children·J V Hartline, P S Zelkowitz
Jul 7, 1956·Journal of the American Medical Association·S J NICHAMIN
Citations
Mar 29, 2000·Pediatric Pulmonology·M Meeks, A Bush
Mar 1, 1995·European Journal of Pediatrics·M LosaS Fanconi
May 12, 2005·Archives de pédiatrie : organe officiel de la Sociéte française de pédiatrie·K Bessaci-KabouyaP Morville
Mar 1, 1993·Acta Paediatrica·E ZammarchiA Cama
Apr 1, 1987·Journal of Veterinary Internal Medicine·W B MorrisonC E Farnum
Mar 1, 1988·Archives of Disease in Childhood·R M BuchdahlJ O Warner
Oct 17, 2006·BMC Pulmonary Medicine·Simon Whalley, I C McManus
Apr 1, 2012·Journal of Clinical Neonatology·Sonia L Molchan, Daniel P Hsu
Jul 1, 1986·Chest·J RametP Clement
Sep 4, 2007·Clinical Radiology·K JainA Bush
Dec 5, 2006·Seminars in Perinatology·Thomas Ferkol, Margaret Leigh
Jan 1, 1985·British Journal of Diseases of the Chest·M Greenstone, P J Cole
Jan 1, 1985·CRC Critical Reviews in Biochemistry·B A Afzelius
Jul 20, 2004·The Journal of Histochemistry and Cytochemistry : Official Journal of the Histochemistry Society·Jukka S LeinonenHannu J Rajaniemi
Mar 11, 2003·Prenatal Diagnosis·Marja W WesselsPatrick J Willems
Jul 1, 1993·Pediatric Pulmonology·V G EngesaethA Bush
Jun 1, 1989·Clinical Otolaryngology and Allied Sciences·L A Smallman
Dec 4, 2003·Journal of Perinatology : Official Journal of the California Perinatal Association·Tanzeema HossainHelen Christou
Mar 31, 2006·Cell Motility and the Cytoskeleton·Stephen M King
Jan 1, 1982·Archives of Oto-rhino-laryngology·H G Wiesemann, U Stephan
May 1, 1983·The Laryngoscope·G S ParkerB Bacher-Wetmore
Mar 7, 2021·Children·Evans Machogu, Benjamin Gaston