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Kartagener syndrome


Other Names for this Disease

  • Dextrocardia - bronchiectasis - sinusitis
  • Dextrocardia bronchiectasis and sinusitis
  • Immotile cilia syndrome, Kartagener type
  • Primary ciliary dyskinesia, Kartagener type
  • Siewert syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is Kartagener syndrome?

What are the signs and symptoms of Kartagener syndrome?

What causes Kartagener syndrome?

How might Kartagener syndrome be treated?

What is Kartagener syndrome?

Kartagener syndrome is a type of primary ciliary dyskinesia associated with situs inversus (mirror-image reversal of internal organs).[1] Kartagener syndrome is inherited in an autosomal recessive fashion and is characterized by the triad of situs inversus, bronchiectasis and sinusitis.[2] About 50% of individuals with primary ciliary dyskinesia have Kartagener syndrome.[3]
Last updated: 7/12/2011

What are the signs and symptoms of Kartagener syndrome?

Karagener syndrome is characterized by primary ciliary dyskinesia and situs inversus totalis. In people affected by situs inversus totalis, the internal organs including the heart, liver, spleen and intestine are on the opposite side of the body. Although the internal organs are abnormally placed, this condition typically does not cause any health problems.[4][5]

The signs and symptoms of primary ciliary dyskinesia vary, but may include:[4][5][6]
Last updated: 2/1/2015

What causes Kartagener syndrome?

Kartagener syndrome is caused by an inherited defective gene which causes the cilia to be either immotile (unable to move) or dysmotile (they move incorrectly). As a result, mucous cannot be cleared from the lungs, paranasal sinuses and ears. Bacteria and other irritants in the mucous lead to frequent respiratory infections.[7] Kartagener syndrome is inherited in an autosomal recessive manner. Recent studies have shown that Kartagener syndrome is linked to a region on chromosome 15q.[8] 
Last updated: 8/22/2011

How might Kartagener syndrome be treated?

There is currently no cure for Kartagener syndrome, but affected individuals can be treated for their symptoms.[9] Antibiotics can be used to treat respiratory infections and may be given on a long-term basis as a means of avoiding further infection. Tubes may be inserted into the ears of young children to assist in the avoidance of recurrent ear infections.[10] Because of their susceptibility to pneumonia and infections, people with Kartagener syndrome should get immunized, avoid tobacco smoke, and exercise regularly to prevent sickness.[9] 
  
Last updated: 8/22/2011

References
  1. Zariwala MA, Knowles MR, Leigh MW. Primary Ciliary Dyskinesia. GeneReviews. October 6, 2009; http://www.ncbi.nlm.nih.gov/books/NBK1122/. Accessed 7/12/2011.
  2. Casanova MS, Tuji FM, Yoo HJ, Haiter-Neto F. Kartagener syndrome. Dentomaxillofac Radiol. September 2006; http://www.ncbi.nlm.nih.gov/pubmed/16940490. Accessed 7/12/2011.
  3. Primary ciliary dyskinesia. Genetics Home Reference (GHR). August 2010; http://ghr.nlm.nih.gov/condition/primary-ciliary-dyskinesia. Accessed 7/12/2011.
  4. Primary Ciliary Dyskinesia. NORD. June 2012; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/526/viewAbstract.
  5. Maimoona A Zariwala, PhD, FACMG, Michael R Knowles, MD, and Margaret W Leigh, MD. Primary Ciliary Dyskinesia. GeneReviews. February 2013; http://www.ncbi.nlm.nih.gov/books/NBK1122/.
  6. John P Bent lll, MD. Kartagener Syndrome. Medscape Reference. February 2014; http://emedicine.medscape.com/article/299299-overview.
  7. Zariwala M. Primary Ciliary Dyskinesia. National Organization for Rare Disorders (NORD). 2008; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Primary%20Ciliary%20Dyskinesia. Accessed 10/13/2008.
  8. Geremek M, Schoenmaker F, Zietkiewicz E, Pogorzelski A, Diehl S, Wijmenga C, Witt M. Eur J Hum Genet. June 2008; http://www.ncbi.nlm.nih.gov/pubmed/18270537. Accessed 10/13/2008.
  9. Primary Ciliary Dyskinesia. American Lung Association. 2011; http://www.lungusa.org/lung-disease/primary-ciliary-dyskinesia/. Accessed 7/12/2011.
  10. Bent JP, Mosenifar Z. Kartagener Syndrome. eMedicine. April 2009; http://emedicine.medscape.com/article/299299-overview. Accessed 7/12/2011.


Other Names for this Disease
  • Dextrocardia - bronchiectasis - sinusitis
  • Dextrocardia bronchiectasis and sinusitis
  • Immotile cilia syndrome, Kartagener type
  • Primary ciliary dyskinesia, Kartagener type
  • Siewert syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.