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.
Wencker
M
M
Department of Pneumology, University Hospital, Ruhrlandklinik, Essen-Heidhausen, FRG.
Denker
J
J
Konietzko
N
N
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