Serial measurements of FEV1 over 12 years in a patient with alpha-1-protease inhibitor deficiency:influence of augmentation therapy and infections.
In patients with severe alpha-1-protease inhibitor (alpha 1-Pi) deficiency forced expiratory volume in 1 s (FEV1) is an accepted parameter to monitor the progression of emphysema. In a patient with severe alpha 1-Pi deficiency (PiZZ) more than 1,000 FEV1 measurements were performed over a period of 12 years. FEV1 dramatically decreased initially (delta FEV1 > 500 ml/year), but stabilized after augmentation therapy was instituted. Three years later, the FEV1 decreased again abruptly; the deterioration was paralleled by an increasing number of severe bronchopulmonary infections. This nonlinear decline implies a positive influence of augmentation therapy and a deleterious effect of bronchopulmonary infections in the disease (p < 0.0005). Daily variation of FEV1 in infection-free intervals exceeded 30% and were thus higher than the mean decrease in FEV1 per year. In this instance, FEV1 measurements performed once or twice per year may reveal a deterioration, whereas the change of FEV1 is still within the range of spontaneous variation. More frequent measurements of FEV1 can be useful to minimize the influence of high intraindividual variation.
Department of Pneumology, University Hospital, Ruhrlandklinik, Essen-Heidhausen, FRG.
M
M
Wencker
J
J
Denker
N
N
Konietzko
D000328:Adult; D000900:Anti-Bacterial Agents; D001424:Bacterial Infections / Q000188:drug therapy / Q000503:physiopathology*; D004359:Drug Therapy, Combination / Q000627:therapeutic use; D005500:Follow-Up Studies; D005541:Forced Expiratory Volume / Q000502:physiology*; D006801:Humans; D008171:Lung Diseases / Q000188:drug therapy / Q000382:microbiology* / Q000503:physiopathology; D008297:Male; D008661:Metabolism, Inborn Errors / Q000188:drug therapy / Q000503:physiopathology; D000515:alpha 1-Antitrypsin / Q000627:therapeutic use; D019896:alpha 1-Antitrypsin Deficiency
D000900:Anti-Bacterial Agents; D000515:alpha 1-Antitrypsin