A case of Kartagener's syndrome presenting as respiratory and right heart failures

S. C. Yang, K. S. Lee, H. J. Yoon, D. H. Shin, S. S. Park, J. H. Lee, C. K. Park

Research output: Contribution to journalArticle

Abstract

Kartagener's syndrome, a congenital disease transmitted as an autosomal recessive illness with a prevalence of approximately 1:20,000 persons, is characterized by the triple association of situs inversus, bronchiectasis, and sinusitis. Affected persons have an incoordination of ciliary motility that leads to defective mucociliary transport, chronic bronchial infections. Kartagener's syndrome is a subset of the immotile cilia syndrome and therefore all patients with Kartagener's syndrome have immotile cilia with obvious ultrastructural defects in the ciliary axoneme. In the respiratory tract this inability presumably causes impaired clearance of mucus and inhaled particles and results in the chronic infections of the sinuses and bronchial trees that are characterized of the disease. The end-stage phenomenon in Kartagener's syndrome, respiratory or heart failure is a less common event and heart-lung transplantation is becoming an accepted therapy for patients with end-stage pulmonary disease in Kartagener's syndrome in many institutes. We report one case of Kartagener's syndrome in a 25-year-old young woman who was presented as respiratory and right heart failures, with review of literatures.

Original languageEnglish
Pages (from-to)251-256
Number of pages6
JournalTuberculosis and Respiratory Diseases
Volume43
Issue number2
DOIs
StatePublished - 1996 Jan 1

Fingerprint

Kartagener Syndrome
Heart Failure
Ciliary Motility Disorders
Heart-Lung Transplantation
Axoneme
Situs Inversus
Mucociliary Clearance
Bronchiectasis
Cilia
Sinusitis
Ataxia
Mucus
Infection
Respiratory Insufficiency
Respiratory System
Lung Diseases

Keywords

  • Kartagener's syndrome
  • heart failure
  • respiratory failure

Cite this

Yang, S. C. ; Lee, K. S. ; Yoon, H. J. ; Shin, D. H. ; Park, S. S. ; Lee, J. H. ; Park, C. K. / A case of Kartagener's syndrome presenting as respiratory and right heart failures. In: Tuberculosis and Respiratory Diseases. 1996 ; Vol. 43, No. 2. pp. 251-256.
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A case of Kartagener's syndrome presenting as respiratory and right heart failures. / Yang, S. C.; Lee, K. S.; Yoon, H. J.; Shin, D. H.; Park, S. S.; Lee, J. H.; Park, C. K.

In: Tuberculosis and Respiratory Diseases, Vol. 43, No. 2, 01.01.1996, p. 251-256.

Research output: Contribution to journalArticle

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