Clinical Expressions of Immotile Cilia Syndrome

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Abstract
Three distinct morphologic types of immotile cilia disorder in man have been recognized. Patients with the disorder have variable clinical manifestations of disease of the upper and lower respiratory tract. Patients (21) with immotile cilia syndrome were evaluated from the standpoint of history, clinical presentation, radiologic changes, pulmonary function tests and mucociliary clearance. Results were compared to the pathologic changes noted on ultrastructural examination of the cilia. Although clinical severity does not correlate with the type of abnormality found, a profile of clinical signs and symptoms suggestive of immotile cilia syndrome emerged. The triad of productive cough, sinusitis and otitis was a consistent finding in all. Situs inversus occurred in only half of the patients.