Neutrophil elastase in patients with homozygous β‐thalassemia and pseudoxanthoma elasticum‐like syndrome

2000 
In this study we Investigated the possible role of neutrophil (PMN) elastase and Its natural inhibitor, α1-proteinase inhibitor (α1-PI) In the pathogenesis of the pseudoxanthoma elasticum (PXE)-like syndrome which is found in patients with homozygous #.thalassemia. We studied 30 β-thalassemia homozygotes with the PXE-like syndrome [PXE(+) group], 20 β-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 0.05). Using logistlc regression, we studied the prognostic value for PXE of the following Independent variables: number of transfusions, chelatlon therapy, mean hemoglobin concentration, PMN elastase concentration, α1-Pl concentration, chronic transaminase elevation, and positivity for antl-HCV. None of the above variabies was found to have significant prognostic value for the PXE. Plasma PMN elastase concentration is elevated in all β-thalassemla homozygotes; its role in the pathogenesis of the PXE-like syndrome in β-thalassemla can not be establlshed, but our findings suggest that neutrophils of β-thalassemia patients are activated, since PMN elastase is a marker of neutrophil activation.
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