Bronchoalveolar lavage(BAL) studies of inhaled aerolised α1-antitrypsin# (α1-AT) in patients with cystic fibrosis (CF) and healthy volunteers
| Subjects n | Nebuliser | α1-AT aerosol | Sample | Elastase μM | α1-AT μM | [Ref.] | |||||
| Dose mg | DD to lungs¶ mg (% dose) | Type | Time after last inhalation h | Before | After | Before | After | ||||
| Healthy | 29 | Pari LL/LC Plus | 200 | 40 (20) | BALF, ELF | Kinetics | 3.1±0.9 | 8.4±2.9 | 3.4±2.0 | 10.4±7.5 after 0.5 h | 135 |
| CF | 12 | Ultravent 10 | 1.5–3 mg·kg body weight−1 (∼90–180 mg) b.i.d. for 1 week | 18–36 (10–20) | BALF, ELF | 4 | 8.2±5.9 | 0 if >8 μM α1-AT in ELF | 4.4±0.5 | 8.8±2.9+, 12.4±8.5§ | 98 |
| CF | 22 | Pari LL | 100, 200, 350 b.i.d.for 4 weeks | 20, 40, 70 (20) | BALF, ELF | 12 | 5.1±1.6 | 0 in all but one patient; inhibitory capacity 2.1±2.3 | 3.8±0.7 | 13.3±1.8 | 136 |
| CF | 8 | Pari LC Plus | 100 b.i.d.for 8 weeks | 20 (20) | BALF, ELF | 12 | 0.22±0.10ƒ | 0.06±0.03## | 3.6±1.2%¶¶ | 9.8±2.8%¶¶ | 137, 138 |
Pari LL and Pari LC Plus are manufactured by Pari, Starnberg, Germany. Ultravent 10 is manufactured by Mallinckrodt Medical, St Louis, MO, USA. DD: delivered dose; BALF: BAL fluid; ELF: epithelial lining fluid. #: prolastin; ¶: per inhalation; + with lower dose; §: with higher dose; ƒ: conversion from 5.8±2.7 U·mL−1 given in original; ##: conversion from 1.6±0.7 U·mL−1 given in original; ¶¶: percentage of α1-AT spot volume assessed from area and density of spot. 1 μM = 52 μg·mL−1 and 1 mg = 0.019 μmol α1-AT; 1 μM = 30 μg·mL−1 and 1 mg = 0.033 μmol human leukocyte elastase (assuming all enzyme is active).