Kartagener症候群の症例報告および電子顕微鏡による超微細構造の観察
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概要
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The Kartagener's syndrcme is cne of the rare congenital diseases. It has three symptoms; chrcnic sinusitis, bronchiectasis and situs inversus. Four cases of this syndrome were firstly reported by Kartagener in 1933. Since then, many cases have been reported clinically but electrcn micrcsccpic study has nct reperted in Japan. Recently we had a case of this syndrome of 43-year-old female. We took biopsy specimens from the mucous membrane of nose, trachea and left main bronchus. These specimens were studied by electron micrcscopy, and several findings were obtained. 1) In the epithelial cells of paranasal cavity, no cilia but many fine microvilli were observed. The secretory mucus filled the lumen surrounded by the epithelial cell lines. 2) In the trachea, on the other hand, cilia was rarely seen and most of them showed defect of dynein arms and discrder of nuicrotubule arrangement and orientation in cross section view. 3) While in the bronchus, the same findings as paranasal cavity was observed. The absence of cilia in the paranasal sinus and left bronchus may cause loss of protective function of the respiratory truct, and may result the storage of mucus in the lumen, which will lead to brcnchiectasis and sinusitis. The abnormality of cilia, the defect of dynein arm of microtubular doublets and disorder of orientation and arrangement of them may cause the immotile cilia syndrome and sterility in male. Further, it may have a relation to the symptom of situs inversus.
- 特定非営利活動法人 日本気管食道科学会の論文
特定非営利活動法人 日本気管食道科学会 | 論文
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