Chest
Clinical InvestigationsThe Relationship of Chronic Sputum Expectoration to Physiologic, Radiologic, and Health Status Characteristics in α1-Antitrypsin Deficiency (PiZ)
Section snippets
Subjects
The group of study subjects consisted of the first 127 patients attending our department for assessment. All patients had a serum α1-antitrypsin (α1-AT) concentration of < 10 μM, and the phenotype was confirmed as PiZ (inferring a genotype PiZZ or PiZnull) by isoelectric focusing in a central US laboratory (Salt Lake City, UT). Augmentation therapy with α1-AT is not licensed within the United Kingdom and, therefore, was not received by any of the study participants. Investigations and data
Results
The 117 nonsmoking PiZ patients displayed a wide range of lung function, although the median data indicated severe airflow obstruction (median FEV1, 32% predicted; total range, 10 to 130% predicted) and reduced gas transfer (median Dlco/Va ratio, 71% predicted; total range, 4 to 148% predicted), which are conditions that are consistent with the presence of emphysema. This was confirmed by HRCT scan, and the VI values demonstrated lower zone predominance (p < 0.001), which is characteristic of α1
Discussion
In this group of patients with α1-ATD, CSE was associated with more severe airflow obstruction and emphysema. In addition, these patients demonstrated worse health status, as assessed by both disease-specific and generic instruments, and the initial analysis indicated that this relationship was independent of the degree of physiologic impairment, smoking history, and age.
Of importance in our study, sputum expectoration was neither sensitive nor specific for the presence of bronchiectasis on
References (30)
- et al.
A comparison of the level of dyspnea vs disease severity in indicating the health-related quality of life of patients with COPD
Chest
(1999) - et al.
“Density mask”: an objective method to quantitate emphysema using computed tomography
Chest
(1988) - et al.
Airway inflammation in COPD assessed by sputum levels of interleukin-8
Chest
(1997) - et al.
Chronic bronchitis: prevalence, smoking habits, impact, and antismoking advice
Prev Med
(1986) - et al.
Psychological factors contributing to disproportionate disability in chronic bronchitis
J Psychosom Res
(1983) The National Mucolytic Study: results of a randomized, double-blind, placebo-controlled study of iodinated glycerol in chronic obstructive bronchitis
Chest
(1990)Augmentation therapy reduces frequency of lung infections in antitrypsin deficiency: a new hypothesis with supporting data
Chest
(2000)- et al.
Reassessment of inflammation of airways in chronic bronchitis
BMJ
(1985) - et al.
Structure of central airways in current smokers and ex-smokers with and without mucus hypersecretion: relationship to lung function
Thorax
(1987) - et al.
The effects of chronic bronchitis and chronic air-flow obstruction on lung cell populations recovered by bronchoalveolar lavage
Am Rev Respir Dis
(1985)
Airways obstruction, chronic expectoration, and rapid decline of FEV1 in smokers are associated with increased levels of sputum neutrophils
Thorax
The natural history of chronic airflow obstruction
BMJ
Association of chronic mucus hypersecretion with FEV1 decline and chronic obstructive pulmonary disease morbidity: Copenhagen City Heart Study Group
Am J Respir Crit Care Med
Relation of ventilatory impairment and of chronic mucus hypersecretion to mortality from obstructive lung disease and from all causes
Thorax
Effect of exacerbation on quality of life in patients with chronic obstructive pulmonary disease
Am J Respir Crit Care Med
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The Antitrypsin Deficiency Assessment and Programme for Treatment (ADAPT) project is supported by a noncommercial grant from Bayer plc. Dr. Stockley is a member of the α1 International Registry (AIR).