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Virtual Pediatric Hospital: Paediapaedia: Kartagener Syndrome (Immotile Cilia Syndrome) Paediapaedia: Chest and Airway Diseases

Kartagener Syndrome (Immotile Cilia Syndrome)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Classic triad of situs inversus, sinusitis, and bronchiectasis. Hearing loss and infertility may also be present.

Etiology/Pathophysiology:
Due to deficiency of dynein arms of cilia which leads to immotility of respiratory, auditory, and sperm cilia.

Pathology:
Electron microscopy reveals the deficient dynein arms of the cilia.

Imaging Findings:
Situs inversus, bronchiectasis, sinusitis.

DDX:

References:
See References Chapter.

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