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Decline in FEV 1 in patients with PiZ alpha‐1‐antitrypsin deficiency: The Australian experience
Author(s) -
BURDON JONATHAN G. W.,
BRENTON SUE,
HOCKING VANESSA,
KNIGHT KENNETH R.,
AYAD MONNA,
COOK LUISA,
JANS EDWARD D.
Publication year - 2002
Publication title -
respirology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.857
H-Index - 85
eISSN - 1440-1843
pISSN - 1323-7799
DOI - 10.1046/j.1440-1843.2002.00363.x
Subject(s) - medicine , spirometry , alpha 1 antitrypsin deficiency , lung function , lung disease , demography , lung , pediatrics , asthma , sociology
Objective: Alpha‐1‐antitrypsin (α 1 antitrypsin) deficiency is a rare hereditary disorder which characteristically presents with emphysema at an early age. The aim of the present study was to determine whether the rate of decline of lung function in α 1 antitrypsin‐deficient subjects in Australia was similar to that found elsewhere. Methodology: Patients registered with the Australian Alpha‐1‐Antitrypsin Replacement Program were studied. All patients ( n = 50) had a serum α 1 antitrypsin concentration of < 0.3 g/L and had had spirometry measured over at least 2 years. They were compared with a group of normal volunteers (hospital staff, n = 107) with normal α 1 antitrypsin levels and phenotypes and with no clinical history of lung disease. All had spirometry measured for periods ranging from 2 to 6 years. The rate of decline of forced expiratory volume in 1 s (FEV 1 ) for each subject was calculated by least squares linear regression using FEV 1 against the time from entry into the study. Results: The group mean (± SD) rate of decline in FEV 1 was significantly greater ( P < 0.01) in the α 1 antitrypsin‐deficient patients (88 ± 71 mL/year) than for the normal controls (–15 ± 48 mL/year). There was no difference in decline in FEV 1 when the data was analysed for gender and for index versus non‐index cases. Conclusion: The results confirm previous reports of an accelerated rate of decline of FEV 1 in patients with α 1 antitrypsin deficiency. Our results indicate that the rate of decline of lung function in α 1 antitrypsin deficient subjects in Australia is similar to that found in reported series from elsewhere.