The Relationship between Plasma Alpha-1-Antitrypsin Polymers and Lung or Liver Function in ZZ Alpha-1-Antitrypsin-Deficient Patients
Abstract
:1. Introduction
2. Materials and Methods
2.1. Patients
2.2. Polymer ELISA
2.3. Western Blot Analyses
2.4. Statistical Analysis
3. Results
3.1. The Relationship between Plasma Z-Aat Polymers and Lung Function in Dutch Cohort
3.2. The Relationship between Plasma Z-AAT Polymers and Liver Function in the Alpha1-Liver Aachen Cohort
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Characteristics | ZZ-AATD | SZ-AATD | Rare AATD |
---|---|---|---|
N male/female | 26/30 | 2/3 | 2/4 |
Age (mean ± SD) | 56 ± 7 | 51 ± 5 | 49 ± 9 |
FEV1 % predicted (mean ± SD) | 58 ± 23 | 63 ± 17 | 53 ± 19 |
Kco % predicted (mean ± SD) | 61 ± 27 | 60 ± 11 | 56 ± 18 |
Serum ALT > ULN (%) | 0 | 0 | 0 |
Serum AST > ULN (%) | 0 | 0 | 0 |
Serum GGT > ULN (%) | 0 | 0 | 0 |
Characteristics | Pi*ZZ (n = 40), Mean ± SD |
---|---|
Age (years) | 51.3 ± 15.3 |
Women (%) | 15 (37.5) |
BMI (kg/m2) | 26.0 ± 4.2 |
BMI ≥ 30 (%) | 7 (17.5) |
Diabetes mellitus (%) | 1 (2.5) |
Liver status | . |
Median stiffness (kPa) | 7.8 ± 11.0 |
LSM ≥ 7.1 kPa (%) | 7 (17.5) |
LSM ≥ 10 kPa (%) | 4 (10) |
CAP (dB/m) | 274.8 ± 50.8 |
ALT ≥ ULN (%) | 9 (22.5) |
AST ≥ ULN (%) | 6 (15) |
GGT ≥ ULN (%) | 13 (32.5) |
ALP ≥ ULN (%) | 6 (15) |
GLDH ≥ ULN (%) | 9 (22.5) |
Bilirubin (mg/dL) | 0.65 ± 0.40 |
INR | 1.07 ± 0.21 |
Triglycerides (mg/dL) | 100.7 ± 63.8 |
HbA1c (%) | 5.1 ± 0.3 |
Ferritin | 185.1 ± 165.1 |
AAT serum level (mg/dL) | 40.6 ± 30.4 |
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Sark, A.D.; Fromme, M.; Olejnicka, B.; Welte, T.; Strnad, P.; Janciauskiene, S.; Stolk, J. The Relationship between Plasma Alpha-1-Antitrypsin Polymers and Lung or Liver Function in ZZ Alpha-1-Antitrypsin-Deficient Patients. Biomolecules 2022, 12, 380. https://doi.org/10.3390/biom12030380
Sark AD, Fromme M, Olejnicka B, Welte T, Strnad P, Janciauskiene S, Stolk J. The Relationship between Plasma Alpha-1-Antitrypsin Polymers and Lung or Liver Function in ZZ Alpha-1-Antitrypsin-Deficient Patients. Biomolecules. 2022; 12(3):380. https://doi.org/10.3390/biom12030380
Chicago/Turabian StyleSark, Annelot D., Malin Fromme, Beata Olejnicka, Tobias Welte, Pavel Strnad, Sabina Janciauskiene, and Jan Stolk. 2022. "The Relationship between Plasma Alpha-1-Antitrypsin Polymers and Lung or Liver Function in ZZ Alpha-1-Antitrypsin-Deficient Patients" Biomolecules 12, no. 3: 380. https://doi.org/10.3390/biom12030380