Kartagener's syndrome

Alternative eponyms

Related people

Hereditary syndrome with a triad of: situs inversus (transposition) of the viscera, abnormal frontal sinuses producing sinusitis and bronchiectasis, and immobility of the cilia.

Description

Hereditary syndrome comprising triad of: situs inversus (transposition) of the viscera, abnormal frontal sinuses producing sinusitis and bronchiectasis, and immobility of the cilia. Symptoms and sign are dyspnoea, productive cough, recurrent respiratory infections, colds, bouts of pneumonia, rheumatoid arthritis, renal abnormalities, turricephaly, heart defects, malformations of renal vessels, anomalous subclavian artery, dextrocardia, and other abnormalities. Palpitation, otitis media, nasal speech, conductive hearing loss. Anosmia. Clubbing of fingers.

The syndrome may begin in neonatal life. In most instances symptoms date back to childhood. Ninety percent of cases were observed before the age of 15 years. Aetiology unknown. Autosomal recessive inheritance with incomplete penetrance. The condition has been observed to occur in siblings and in identical twins. Frequency ca. 1: 40 000. It was first described by the Ukrainian physician A. K. Zivert. Kartagener reported a group of cases in 1933.

We thank Andre Trombeta and Walter E. Berdon for information submitted.

Bibliography

  • A. K. Zivert:
    Über einen Fall von Bronchiectasie bei einem Patienten mit situs inversus viscerum.
    Berliner klinische Wochenschrift, 1904, 41: 139-141.

  • M. Kartagener:
    Zur Pathogenese der Bronchiektasien: Bronchiektasien bei Situs viscerum inversus.
    Beiträge zum Klinik der Tuberkulose, 1933, 83: 489-501.

    Zur Frage der Bronchiektasien. Familiäres Vorkommen von Bronchiektasien.
    Beiträge zum Klinik der Tuberkulose, 1933, 84: 73.
  • M. Kartagener, A. Horlacher:
    Zur Pathogenese der Bronkiektasien. Situs viscerum inversus und polyposis nasi in einem Falle familiärer Bronkiektasen.
    Beiträge zum Klinik der Tuberculose, 1935, 87: 489.

  • B. A. Afzelius:
    A human syndrome caused by immotile cilia.
    Science, Washington, 1976, 193: 317-319.
  • B. A. Afzelius, H. Kolberg:
    Medfödd cilieorörlighet - en genomgång av ett norrländskt barn- och ungdomsmaterial.
    Läkartidningen, 1981, 78: 3045-3048.
  • Walter E. Berdon, Chris McManus, and Björn Afzelius:
    More on Kartagener's syndrome and the contributions of Afzelius and A.K. Siewert.<7i>
    Pediatric Radiology, Heidelberg, July 2004, 34 (7): 585-586. Letter to the editor.

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