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- Title
Evolution of Novel Small-Molecule Therapeutics Targeting Sickle Cell Vasculopathy.
- Authors
Kato, Gregory J.; Gladwin, Mark T.
- Abstract
The article presents a review of the pathophysiology of sickle vasculopathy and looks at the results of clinical trials of novel small-molecule therapeutics in patients with sickle cell disease. The case of a 34 year old African American woman with homozygous sickle cell disease is presented. Her medical history which involved chronic severe hemolytic anemia with complications of pulmonary artery systolic hypertension and leg ulcers is presented. A discussion on the scavenging of nitric oxide by cell-free plasma hemoglobin is included. A discussion of intravascular hemolysis and decreased nitric oxide bioavailability marked by lactate dehydrogenase is also included. The involvement of endogenous nitric oxide synthase inhibitors with pulmonary hypertension is addressed as well.
- Subjects
SICKLE cell anemia treatment; PATHOLOGICAL physiology; PULMONARY hypertension; HEMOLYSIS &; hemolysins; DEHYDROGENASES; NITRIC oxide; LEG ulcers
- Publication
JAMA: Journal of the American Medical Association, 2008, Vol 300, Issue 22, p2638
- ISSN
0098-7484
- Publication type
Article
- DOI
10.1001/jama.2008.598