Ciliary ultrastructure in two sisters with Kartagener's syndrome

Med Mol Morphol. 2007 Mar;40(1):34-9. doi: 10.1007/s00795-007-0354-y. Epub 2007 Mar 29.

Abstract

Kartagener's syndrome (KS) is a clinical variant of primary ciliary dyskinesia involving situs inversus associated with chronic airway infections. We studied two sisters; the elder one had dextrocardia and scoliosis, and the younger one had situs inversus of the lung, liver, and stomach as well as dextrocardia. Both patients had chronic sinusitis and chronic bronchitis with bronchiectasis. In both cases, the ciliary defect associated with this syndrome is the absence of inner dynein arms.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Bronchiectasis / pathology
  • Bronchitis, Chronic / pathology
  • Cilia / ultrastructure*
  • Dextrocardia / pathology
  • Female
  • Humans
  • Kartagener Syndrome / pathology*
  • Liver / pathology
  • Lung / pathology
  • Lung / ultrastructure
  • Microscopy, Electron, Transmission
  • Respiratory Mucosa / pathology
  • Respiratory Mucosa / ultrastructure*
  • Scoliosis / pathology
  • Siblings
  • Sinusitis / pathology
  • Situs Inversus / pathology
  • Stomach / pathology