Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007

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Improved survival at low lung function in cystic fibrosis: cohort study from 1990 to 2007

OBJECTIVES To evaluate the survival of patients with cystic fibrosis whose lung function has deteriorated to a forced expiratory volume in one second (FEV(1)) below 30% predicted in the recent treatment era and to explore factors associated with any change in survival. Design Cohort study. SETTING Adult cystic fibrosis unit in London. PARTICIPANTS 276 patients (147 (53%) male) whose FEV(1) ...

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Lung function from infancy to school age in cystic fibrosis.

The aim was to investigate pulmonary mechanics in patients with cystic fibrosis during infancy and again in early childhood to see whether infant tests predicted status at school age. Plethysmographic measurements of thoracic gas volume and airways resistance were made in 29 patients at 6 months and again at 5 years 10 months. Maximum flow at functional residual capacity was measured during inf...

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Lung function from infancy to preschool in a cohort of children with cystic fibrosis.

This study aimed to describe lung function in a cohort of children with cystic fibrosis (CF) who underwent infant pulmonary function tests (IPFTs) and preschool spirometry. Children performed up to four IPFTs (raised volume rapid thoracic compression technique) over 1 yr and five preschool spirometry tests over up to 2 yrs during participation in prospective, multicentre studies of infant and p...

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Cystic fibrosis and survival to 40 years: a study of cystic fibrosis transmembrane conductance regulator function.

Significant survival heterogeneity exists in cystic fibrosis. Our aim was to determine whether residual function of the cystic fibrosis transmembrane conductance regulator (CFTR) is present in long-term survivors with severe mutations. Nasal potential difference (PD) and sweat chloride were measured in 34 long-term survivors (aged ≥ 40 yrs) and compared with young patients (18-23 yrs) with seve...

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Lung function in infants with cystic fibrosis.

Lung function was measured in 28 infants with cystic fibrosis and repeated in 17 of the infants during the first year of life. Thoracic gas volume (TGV) and specific airway conductance (sGaw) were measured plethysmographically and maximum forced expiratory flow at functional residual capacity (VmaxFRC) was derived from the partial expiratory flow-volume curve. At the time of the initial evaluat...

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ژورنال

عنوان ژورنال: BMJ

سال: 2011

ISSN: 0959-8138,1468-5833

DOI: 10.1136/bmj.d1008