Ty- jour t1- I型Gaucher病JF中的肺功能异常 - 欧洲呼吸杂志au -bar ziv，y au -hadas -halpern，i au -melzer，e au -au -cahan，c au -branski，d au -zimran，a y1-1996/02/01 ur -http：http：//erj.ersjournals。com/content/9/2/340.Abstract N2-本研究的目的是确定I型高彻氏病患者肺功能和放射学异常的流行，并分析肺参与与临床型与临床之间的关系严重程度得分。前瞻性评估了1992 - 1993年期间，所有参加以色列耶路撒冷Shaare Zedek医疗中心的Gaucher诊所的患者均进行了评估。每个患者都有肺功能检查，胸部射线照相，器官受累程度和基因型分析的临床评估。在研究中包括的95例患者中（平均+/- SD年龄29 +/- 15年），有68％的患者具有一定的肺功能异常，最常见的是碳一氧化碳（KCO）的FRC和转移系数，发现为45％。和42％的患者。在大约三分之一的患者中，在22％的患者中降低了22％的患者肺活量（TLC），并强迫呼气流动。在18％的患者中，发现了气动症的迹象（剩余体积升高（RV）或RV/TLC）。 Males had a higher incidence of reduced expiratory flow than females, (forced expiratory volume in one second (FEV1) was reduced in 36% of males vs 5% of females). Chest radiographic abnormalities were found in 17% of the patients, although only 4% had severe changes. Patients with abnormal pulmonary function had a significantly higher severity score index than those with normal pulmonary function tests. There was no association between abnormal pulmonary function and genotype or age. In conclusion, abnormal pulmonary function is common among type I Gaucher patients. Pulmonary function tests show airways obstruction, with reduced expiratory flows, reduction in lung volumes and alveolar-capillary diffusion abnormality. The rate of progression and the clinical significance need to be determined. ER -