Kartagener syndrome

Description

Kartagener syndrome (KS) is an inherited disorder (autosomal recessive ) characterised by the clinical triad of chronic sinusitis; bronchiectasis; and situs inversus. It is is a subset of primary ciliary dyskinesia with abnormal ciliary structure/function associated with impaired ciliary motility


History

1904 – AK Siewert from Kiev (Ukraine) described a patient who ‘since birth had the unusual combination of symptoms of bronchiectasis and situs inversus totalis‘ [1904;41:139-141]

1933Kartagener described the triad of situs inversus, chronic rhinosinusitis, and chronic bronchitis with bronchiectasis in four patients [1933;83(4):489–501]

1962Kartagener then published additional cases with the triad he observed for a total of 334 cases [1962; 79: 193-207]

1976 – Afzelius studied four male subjects with immotile sperm, and observed that three of the four had chronic bronchitis, sinusitis and no mucociliary transport (measured by tracheobronchial clearance). The cilia on the cells of these patients were observed to lack dyenin arms on electron microscopy

1977 – Eliasson et al. found that patients with this syndrome had absent mucociliary transport in the nose and bronchi; the cilia lacked dynein arms; and male patients were infertile with immotile spermatozoa, lacking dynein arms. They proposed the disease be renamed ‘immotile-cilia syndrome‘.

1980 – Independent groups of researchers demonstrated that in most patients with Kartagener syndrome, ciliated cells from both the nose and bronchido demonstrate some degree of ciliary motility


Associated Persons


Alternative names

  • Siewert (Zivert) syndrome
  • Siewert-Kartagener syndrome
  • Kartagener-Afzelius syndrome
  • Primary ciliary dyskinesia (PCD)
  • Immotile cilia syndrome [Eliasson, 1977]

References


eponymictionary CTA

eponymictionary

the names behind the name

Doctor in Australia. Keen interest in internal medicine, medical education, and medical history.

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