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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

My son has Kartagener syndrome and is having several medical problems. I need to know as much as possible about this condition.

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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 is primary ciliary dyskinesia?

Primary ciliary dyskinesia is a genetic disease which affects the upper and lower airways of the lungs. Through an inherited defective gene, the cilia (tiny hair-like structures that move mucus out of the respiratory passages) in individuals affected by this condition are either immotile (unable to move) or dysmotile (they move incorrectly). As a result, the body is not able to keep the lungs and sinuses clear of fluids, which can lead to congestion, infection, and various other complications.[4][5] Because cilia are also present in the brain and reproductive organs, individuals with primary ciliary dyskinesia may also have chronic headaches, hydrocephalus and infertility.[4]  
Last updated: 5/10/2011

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][6] 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.[6] 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][6]

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][6] 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][7]
Last updated: 8/22/2011

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.[8] Kartagener syndrome is inherited in an autosomal recessive manner. Recent studies have shown that Kartagener syndrome is linked to a region on chromosome 15q.[9] 
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.[6] 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

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.[6]
Last updated: 1/5/2009