Kartagener syndrome

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Disease Overview

Kartagener syndrome is a type of primary ciliary dyskinesia that is also characterized by situs inversus totalis (mirror-image reversal of internal organs). The signs and symptoms vary but may include neonatal respiratory distress; frequent lung, sinus and middle ear infections beginning in early childhood; and infertility.[8196][8197][8198] It can be cause by changes (mutations) in many different genes that are inherited in an autosomal recessive manner. Although scientists have identified many of the genes associated with Kartagener syndrome, the genetic cause of some cases is unknown.[8199][8197] There is no cure for Kartagener syndrome. Treatment varies based on the signs and symptoms present in each person but may include airway clearance therapy and antibiotics.[8196][8197][8198]


Synonyms

  • Dextrocardia bronchiectasis and sinusitis
  • Siewert syndrome
  • Dextrocardia-bronchiectasis-sinusitis syndrome
  • Primary ciliary dyskinesia and situs inversus
  • Immotile cilia syndrome, Kartagener type
  • Primary ciliary dyskinesia, Kartagener type

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National Organization for Rare Disorders