Neutrophil elastase in patients with homozygous ?-thalassemia and pseudoxanthoma elasticum-like syndrome
✍ Scribed by Samarkos, M.; Aessopos, A.; Fragodimitri, C.; Karagiorga, M.; Kalotychou, V.; Voskaridou, E.; Kavouklis, E.; Loukopoulos, D.
- Publisher
- John Wiley and Sons
- Year
- 2000
- Tongue
- English
- Weight
- 44 KB
- Volume
- 63
- Category
- Article
- ISSN
- 0361-8609
No coin nor oath required. For personal study only.
✦ Synopsis
In this study we investigated the possible role of neutrophil (PMN) elastase and its natural inhibitor, ␣1-proteinase inhibitor (␣1-PI) in the pathogenesis of the pseudoxanthoma elasticum (PXE)-like syndrome which is found in patients with homozygous -thalassemia. We studied 30 -thalassemia homozygotes with the PXE-like syndrome [PXE(+) group], 20 -thalassemia homozygotes without this syndrome [PXE(-) group] and 15 healthy controls. Plasma PMN elastase concentration in the PXE(+) and in the PXE(-) group was 136.4 ± 89 and 163.8 ± 126 µg/L, respectively (P > 0.05). In the control group, the concentration was 42.9 ± 16.8 µg/L (P < 0.01 for the comparison with both patients' groups). The plasma ␣1-PI concentration in the PXE(+) and in the PXE(-) group was 2.28 ± 0.75 and 2.6 ± 0.96 g/L, respectively (P > 0.05). Using logistic regression, we studied the prognostic value for PXE of the following independent variables: number of transfusions, chelation therapy, mean hemoglobin concentration, PMN elastase concentration, ␣1-PI concentration, chronic transaminase elevation, and positivity for anti-HCV. None of the above variables was found to have significant prognostic value for the PXE. Plasma PMN elastase concentration is elevated in all -thalassemia homozygotes; its role in the pathogenesis of the PXE-like syndrome in -thalassemia can not be established, but our findings suggest that neutrophils of -thalassemia patients are activated, since PMN elastase is a marker of neutrophil activation. Am.