One case of Kartagener's syndrome with extracemtral microtubule in cilia / 대한내과학회지

ABSTRACT

Kartagener's syndrome is an autosomal recessively inherited condition characterized by triad of situs inversus, bronchiectasis, and chronic sinusitis. And recently it was classified as a subclass of dyskinetic cilia syndrome, which has a defect in mucociliary transport resulting from immotile or dyskinetic beating of cilia. Electron microscopic examination of the cilia from sperm tails, nasal and bronchial epithelium of patients reveals the partial or the complete absence of dynein arms or radial spoke, or microtubule disposition. This patient had all the triad of Kartagener's syndrome and showed two extracentral microtubules on the electron microscopic examination of the nasal mucosa. Most patients have dynein arms defect, but it is rare to have extracentral microtubules. So we report one case of Kartagener's syndrome with extracentral microtubules confirmed by electron microscopy.