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1-antitrypsin deficiency: relationship to lung function and health status
1 Antitrypsin Deficiency Assessment and Programme for Treatment (ADAPT) project, Lung Investigation Unit, Queen Elizabeth Hospital, Birmingham, UK and 2 School of Maths and Statistics, Birmingham University, Birmingham, UK
CORRESPONDENCE: R.A. Stockley, Dept of Medicine, Queen Elizabeth Hospital, University Hospital Birmingham NHS Trust, Birmingham, B15 2TH, UK. Fax: 44 1216978256
Keywords: 
1-antitrypsin deficiency, computed tomography, health status, obstructive lung disease, pulmonary emphysema, respiratory function tests
Received: June 22, 2000
Accepted January 18, 2001
The development of computed tomography (CT) has enabled emphysema to be assessed noninvasively. Objective quantification of lung density correlates well with lung function in patients with chronic obstructive pulmonary disease and has been shown to be a sensitive tool for monitoring disease progression.
In order to determine the clinical impact of changes seen on high-resolution computed tomography (HRCT), the relationship between the objective quantification of emphysema on HRCT, lung function and health status in 111 patients with
The degree of HRCT scan abnormality correlated well (p<0.001 for all comparisons) with forced expiratory volume in one second (r=–0.60––0.75), specific airway conductance (r=–0.67–0.76), residual volume/total lung capacity (r=0.46–0.58) and transfer factor of the lung for carbon monoxide (r=–0.64––0.81). In addition, the CT scans correlated (p<0.001) with health status as assessed by the St. George's Respiratory Questionnaire (SGRQ total: r=–0.38–0.50) and the Short-Form health survey (e.g. physical functioning: r=–0.39–0.54).
In summary, other workers have shown high-resolution computed tomography to be a sensitive indicator of disease progression. This study confirms the relationship between high-resolution computed tomography and lung physiology, and suggests the relationship is even stronger in patients with predominantly lower zone pan-lobular emphysema than in usual chronic obstructive pulmonary disease. High-resolution computed tomography also relates to patients disability and impairment as defined by health status questionnaires and, therefore, should be considered as an alternative outcome measure particularly in a1-antitrypsin deficiency.
1-antitrypsin deficiency was examined (PiZ).
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