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. 1981 Jun;56(6):432–435. doi: 10.1136/adc.56.6.432

Immotile cilia syndrome: a new cause of neonatal respiratory distress.

A Whitelaw, A Evans, B Corrin
PMCID: PMC1627459  PMID: 6973323

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.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Eliasson R., Mossberg B., Camner P., Afzelius B. A. The immotile-cilia syndrome. A congenital ciliary abnormality as an etiologic factor in chronic airway infections and male sterility. N Engl J Med. 1977 Jul 7;297(1):1–6. doi: 10.1056/NEJM197707072970101. [DOI] [PubMed] [Google Scholar]
  2. Fischer T. J., McAdams J. A., Entis G. N., Cotton R., Ghory J. E., Ausdenmoore R. W. Middle ear ciliary defect in Kartagener's syndrome. Pediatrics. 1978 Oct;62(4):443–445. [PubMed] [Google Scholar]
  3. Hartline J. V., Zelkowitz P. S. Kartagener's syndrome in childhood. Am J Dis Child. 1971 Apr;121(4):349–352. doi: 10.1001/archpedi.1971.02100150123020. [DOI] [PubMed] [Google Scholar]
  4. Holmes L. B., Blennerhassett J. B., Austen K. F. A reappraisal of Kartagener's syndrome. Am J Med Sci. 1968 Jan;255:13–28. [PubMed] [Google Scholar]
  5. Monnet P. Situs inversus et bronchopneumopathies au long cours, dès la période néonatale. Arch Fr Pediatr. 1978 Jun-Jul;35(6):607–619. [PubMed] [Google Scholar]
  6. NICHAMIN S. J. Kartagener's syndrome in a newborn infant. J Am Med Assoc. 1956 Jul 7;161(10):966–968. doi: 10.1001/jama.1956.62970100004009b. [DOI] [PubMed] [Google Scholar]
  7. SCHOEMPERLEN C. B., CAREY S. L. KARTAGENER'S SYNDROME. Am Rev Respir Dis. 1963 Nov;88:698–702. doi: 10.1164/arrd.1963.88.5.698. [DOI] [PubMed] [Google Scholar]

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