Alpha-1 Antitrypsin Augmentation Therapy and Biomarkers of Elastin Degradation

Figures & data

Table 1.  Patient cohorts providing plasma samples for analysis of DI

Plasma Samples	MM	ZZ No Aug	ZZ Augmented
N	50	50	47
Age, years
AV	59.7 ± 11.5	61.0 ± 11.7	64.6 ± 9.7
Gender,% Male	36.0	46.9	34.7
AV
Phenotype	All subjectsPI MM	All subjectsPI ZZ	All subjectsPI ZZ
Serum alpha1-antitrypsin, μM	28.2 ± 4.2	4.4 ± 0.9	22.0 ± 14.3
AV + SD
Forced Expiratory Volume in 1 second	NotDetermined	72.0 ± 29.2	40.6 ± 21.6
AV ± S.D.

See results from these cohorts in Figure 1.

Table 2.  Patients receiving AAT augmentation by intravenous infusion

Plasma	Pre-Augmented	Post-Augmented
N	11	11
Age, years	53.6 ± 7.7	54.0 ± 8.0
Gender,% Male	55	55
Phenotype	All subjects PI ZZ	All subjects PI ZZ
Serum alpha1-antitrypsin, μM	3.8 ± 0.9	18.0 ± 5.7
AV + S.D.
Forced Expiratory Volume in 1 second	63.2 ± 20.3	63.2 ± 17.6
AV + S.D.

See results from this cohort in Figures 2 and 3.

Table 3.  Patients receiving augmentation of AAT by aerosol administration

Aerosol	Pre-Dose
N		12
Age, years		55.2 ± 2.8
Gender,% Male		66.7
Phenotype	All subjects PI ZZ
Serum alpha1-antitrypsin, μM	8.0 ± 0.8Post Dose: 8.3 ± 0.9
AV + S.D
Forced Expiratory Volume in 1 second	60.0 ± 15.6
AV + S.D.

See Results from this cohort in Figures 4 and 5.

Figure 1.  The effect of intravenous alpha-1 antitrypsin augmentation therapy on plasma levels of desmosine and isodesmosine (DI) in alpha-1 antitrypsin deficiency.

Figure 2.  The effect of intravenous alpha-1 antitrypsin augmentation therapy on levels of desmosine and isodesmosine (DI) in plasma.

Figure 3.  The effect of Intravenous alpha-1 antitrypsin augmentation therapy on levels of desmosine and isodesmosine (DI) in bronchoalveolar lavage fluid.

Figure 4.  The effect of aerosol alpha-1 antitrypsin augmentation therapy on levels of desmosine and isodesmosine (DI) in plasma.

Figure 5.  The effect of aerosol alpha-1 antitrypsin augmentation therapy on levels of desmosine and isodesmosine (DI) in bronchoalveolar lavage fluid.

Figure 6.  Correlation of DI in plasma and BAL 12 weeks after intravenous augmentation therapy.

Figure 7.  The effect of aerosol alpha-1 antitrypsin augmentation therapy on urinary desmosine and isodesmosine (DI): Ratios of free DI to total DI.

Figure 8.  The relationship of age to plasma levels of desmosine and isodesmosine (DI) in normal subjects and alpha-1 deficient subjects receiving and not receiving intravenous alpha-1 antitrypsin augmentation therapy.