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KMID : 0361019890320040717
Korean Journal of Otolaryngology - Head and Neck Surgery
1989 Volume.32 No. 4 p.717 ~ p.722
Immotile Cilia Syndrome
CH Lee
JH Kim/EG Hwang
Abstract
We have recently experienced 3 cases of immotile cilia syndrome within a family. They had chronic recurrent episodes of productive cough and hyperrhinorrhea. All of 3 cases had same ciliary defects(type ld : partial outer and inner arm defects) which were confirmed by electron microscopic biopsy at the inferior turbinate mucosa. The immotile cilia syndrome is defined as a congenital disorder of cilia associated with ultrastructural defects having specific and genetically determined abnormalities. The cilia with ultrastructural defects retain some abnormal ciliary motility which suggest that this disorder be termed ""dyskinetic ciliary syndrome"" rather than immotile cilia syndrome. Diagnosis is ultimately confirmed by nasal electronmicroscopic biopsy and treatment is based on appropriate drainage procedures. Prognosis for a normal life is excellent.
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