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Genetic and Rare Diseases Information Center (GARD)

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Kartagener syndrome


Other Names for this Disease
  • Dextrocardia bronchiectasis and sinusitis
  • Siewert syndrome
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Your Question

I have Kartagener syndrome. As I get older, it seems as if my symptoms are getting worse. I am on a long-term regimen of antibiotics and wondered if there were other options for management of this condition or resources where I can obtain support for a healthy lifestyle.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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?

Kartagener syndrome is characterized by the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus.[4][5] Most children have chronic year-round cough, and chronic sinusitis and nasal congestion. These symptoms often persist into adulthood.[1] Recurrent chronic sinusitis may lead to sinus pressure headaches. Chronic bronchitis and recurrent pneumonia are also common.[5] Chronic/recurrent ear infections are apparent in most young children with Kartagener syndrome, but becomes less apparent by school age. In many infants and young children, chronic otitis media is associated with transient hearing loss that may affect speech development. If untreated, infections of the middle ear may result in irreversible hearing loss.[1][5]

Individuals also have situs inversus totalis, a mirror-image reversal of all organs. The reversed organs do not usually pose a problem.[4][1] Males with Kartagener syndrome may be infertile secondary to impaired sperm motility because the flagella of the sperm and cilia often (but not always) have the same defects.[1][5] Some women with Kartagener syndrome have normal fertility, but others have impaired fertility and an increased risk for ectopic pregnancy because of impaired ciliary function in the fallopian tubes.[1][6]
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.[4] 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.[5] Because of their susceptibility to pneumonia and infections, people with Kartagener syndrome should get immunized, avoid tobacco smoke, and exercise regularly to prevent sickness.[4] 
  
Last updated: 8/22/2011

Where can I learn more about current research for Kartagener syndrome, including studies which may be looking at this condition in different age groups and those working towards new and better ways to treat the symptoms?

ClinicalTrials.gov lists trials that are studying or have studied Kartagener syndrome. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies. 

The Genetic Disorders Of Mucociliary Clearance Consortium is a network of four U.S. centers (University of North Carolina at Chapel Hill, Washington University in St. Louis, University of Washington, and University of Colorado) that are collaborating in the diagnostic testing, genetic studies, and clinical trials in patients with impairments in mucociliary clearance, focusing on primary ciliary dyskinesia, cystic fibrosis, and pseudohypoaldosteronism. Ultimately, this group hopes to better define the clinical pathogenesis of these important airway diseases, improve or expand diagnostic testing, and develop new and effective treatments. To read more about this consortium and their involvement with primary ciliary dyskinesia and Kartagener syndrome, click on the above link.

Last updated: 1/5/2009

What is the prognosis for individuals with Kartagener syndrome?

Chronic childhood infections can be very debilitating, but the range and severity of clinical symptoms is wide. Fortunately, the disease usually becomes less problematic near the end of the patient's second decade, and many patients have near normal adult lives.[5]
Last updated: 1/5/2009

Where can I find support for individuals with Kartagener syndrome?

The following organizations provide information and support to individuals and families affected by Kartagener syndrome. These groups may be able to provide you with guidance regarding maintaining a healthy lifestyle. 

The PCD Foundation is dedicated to increasing awareness of inherited ciliary disorders and to providing education and advocacy for affected individuals and their caregivers.

PCD (Primary Ciliary Dyskinesia) Foundation
29252 N. 22nd. Lane
Phoenix AZ 85085
Phone: 623-215-2032 or 612-396-1179
Fax: 623-215-6670
Email: info@pcdfoundation.org
Web: www.pcdfoundation.org

The Primary Ciliary Dyskinesia Family Support Group was formed to provide support to adults with primary ciliary dyskinesias and parents of children known to have the condition.

Primary Ciliary Dyskinesia Family Support Group
67 Evendons Lane
Wokingham
Berks RG41 4AD
United Kingdom
Phone: 0118 9770258
Web: www.pcdsupport.org.uk

The Kartagener's Syndrome and Primary Ciliary Dyskinesia Foundation is a German organization formed for people with Primary Ciliary Dyskinesia and Kartagener´s Syndrome, and their families. They are in contact with individuals with this diagnosis all over the world.

Kartagener's Syndrome and Primary Ciliary Dyskinesia Foundation
Email: nc-fuerleru@netcologne.de
Web: www.kartagener-syndrome.org

You may also benefit from contacting the American Lung Association.

American Lung Association
61 Broadway 6th Floor
New York NY 10006
Phone: 800-LUNGUSA (800-586-4871)
Primary Ciliary Dyskinesia (including Kartagener syndrome)
Last updated: 1/5/2009

References