Longitudinal study of lung function in a cohort of primary ciliary dyskinesia
摘要：
Patients with () have pronounced stasis of their respiratory and therefore recurrent lower airway , which raises concerns for the of lung function. Twenty four patients with have been studied prospectively with a standardized regime in our clinic for 2-16 yrs with clinic visits, including spirometry 2-4 times per year, daily physiotherapy and monthly sputum cultures with subsequent specific antibiotic treatment. Lung function was significantly lower in the 12 PCD patients entering the cohort as adults when compared to the patients entering as children (forced vital capacity (FVC) 70 versus 85% predicted; forced expiratory volume in one second (FEV1) 59 versus 72% pred). The lung damage did not relate to the type of . During the subsequent surveillance of the groups for a median of 14 and 7 yrs, respectively, the lung function remained stable in most patients. It is concluded that is accompanied by a progressive deterioration in lung function if undertreated, but lung function can be maintained with appropriate antibiotic treatment and regular physiotherapy. This emphasizes the need for early diagnosis of .
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DOI：
10.1183/09031936.97.10102376
被引量：
年份：
1997
Taylor & Francis
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万方医学
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