Long-Term Augmentation Therapy in Twenty Patients with Severe Alpha-1-Antitrypsin Deficiency – Three-Year Follow-Up
| Autor: | Martin Schwaiblmair, G. Fruhmann, Claus Vogelmeier |
|---|---|
| Rok vydání: | 1997 |
| Předmět: |
Adult
Male Pulmonary and Respiratory Medicine congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty Pediatrics Serine Proteinase Inhibitors medicine.medical_treatment Pulmonary function testing alpha 1-Antitrypsin Deficiency medicine Humans Prospective Studies Infusions Intravenous Prospective cohort study Lung function Aged Chemotherapy Alpha 1-antitrypsin deficiency Dose-Response Relationship Drug business.industry Respiratory disease Follow up studies Middle Aged medicine.disease Respiratory Function Tests respiratory tract diseases Surgery Treatment Outcome α1 antitrypsin Pulmonary Emphysema alpha 1-Antitrypsin Female Deficiency Diseases business Follow-Up Studies |
| Zdroj: | Respiration. 64:10-15 |
| ISSN: | 1423-0356 0025-7931 |
| DOI: | 10.1159/000196636 |
| Popis: | The purpose of this uncontrolled, prospective study was to evaluate the influence of long-term augmentation therapy with plasma-derived alpha 1-antitrypsin (AAT) on lung function parameters in patients with severe emphysema caused by AAT deficiency. Twenty patients (mean age 48 years) received AAT infusions once weekly for up to 36 months. No adverse effects were observed. At the beginning of the study, mean (+/- SEM) FEV1 was 1.35 +/- 0.12 liters and mean TLCO was 54 +/- 4% of predicted. After 36 months of treatment, mean FEV1 was 1.25 +/- 0.12 liters (p = n.s) and the TLCO was 52 +/- 4% predicted (p = n.s). Similar values were obtained before and after therapy for FVC (2.79 +/- 0.23 vs. 2.82 +/- 0.21 liters), MEF50 (0.72 +/- 0.09 vs. 0.68 +/- 0.08 liters/s), RV (4.60 +/0 0.44 vs 4.45 +/- 0.311) and TLC (7.72 +/- 0.49 vs. 7.38 +/- 0.42 l). The calculated annual loss of FEV1 (35.6 ml/year) was smaller than in historical untreated patients with AAT deficiency. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|