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Diseases

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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Symptoms

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What are the signs and symptoms of Kartagener syndrome?

Kartagener syndrome is characterized by the clinical triad of chronic sinusitis, bronchiectasis, and situs inversus.[1][2] Most children have chronic year-round cough, and chronic sinusitis and nasal congestion. These symptoms often persist into adulthood.[3] Recurrent chronic sinusitis may lead to sinus pressure headaches. Chronic bronchitis and recurrent pneumonia are also common.[2] 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.[3][2]

Individuals also have situs inversus totalis, a mirror-image reversal of all organs. The reversed organs do not usually pose a problem.[1][3] 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.[3][2] 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.[3][4]
Last updated: 8/22/2011

The Human Phenotype Ontology provides the following list of signs and symptoms for Kartagener syndrome. If the information is available, the table below includes how often the symptom is seen in people with this condition. You can use the MedlinePlus Medical Dictionary to look up the definitions for these medical terms.

Signs and Symptoms Approximate number of patients (when available)
Recurrent respiratory infections 90%
Abnormal respiratory motile cilium morphology -
Abnormality of the cornea -
Absent frontal sinuses -
Anosmia -
Asplenia -
Autosomal recessive inheritance -
Bronchiectasis -
Chronic otitis media -
Chronic rhinitis -
Chronic sinusitis -
Communicating hydrocephalus -
Conductive hearing impairment -
Headache -
Heterogeneous -
Immotile cilia -
Male infertility -
Nasal polyposis -
Pneumonia -
Sinusitis -
Situs inversus totalis -

Last updated: 11/3/2014

The Human Phenotype Ontology (HPO) has collected information on how often a sign or symptom occurs in a condition. Much of this information comes from Orphanet, a European rare disease database. The frequency of a sign or symptom is usually listed as a rough estimate of the percentage of patients who have that feature.

The frequency may also be listed as a fraction. The first number of the fraction is how many people had the symptom, and the second number is the total number of people who were examined in one study. For example, a frequency of 25/25 means that in a study of 25 people all patients were found to have that symptom. Because these frequencies are based on a specific study, the fractions may be different if another group of patients are examined.

Sometimes, no information on frequency is available. In these cases, the sign or symptom may be rare or common.


References
  1. Primary Ciliary Dyskinesia. American Lung Association. 2011; http://www.lungusa.org/lung-disease/primary-ciliary-dyskinesia/. Accessed 7/12/2011.
  2. Bent JP, Mosenifar Z. Kartagener Syndrome. eMedicine. April 2009; http://emedicine.medscape.com/article/299299-overview. Accessed 7/12/2011.
  3. 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.
  4. Kartagener Syndrome. Online Mendelian Inheritance in Man (OMIM). June 2011; http://omim.org/entry/244400. Accessed 7/12/2011.


Other Names for this Disease
  • Dextrocardia bronchiectasis and sinusitis
  • 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.