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[Pathophysiology of acute thoracic syndrome].

Authors
Type
Published Article
Journal
Pathologie Biologie
0369-8114
Publisher
Elsevier
Publication Date
Volume
47
Issue
1
Pages
26–30
Identifiers
PMID: 10081776
Source
Medline
License
Unknown

Abstract

Pulmonary complications are the leading cause of morbidity and mortality in sickle cell disease patients. Acute chest syndrome (ACS), in which chest pain and dyspnea, occurs in combination with a recent chest radiograph abnormality, raises both diagnostic and therapeutic challenges. The pathogenesis of ACS involves alterations in blood rheology, increased coagulability, and, above all, increased adhesion of sickle cells to the vascular endothelium and nitric oxide-mediated dysregulation of vascular reactivity. Sickle cell disease thus impacts all the cells in the vascular environment. Recently gained insights into pathophysiology offer hope that new treatments for preventing and treating acute and chronic pulmonary complications will soon become available.

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