Abstract | CONTEXT: OBJECTIVE: DESIGN: SETTING: Urban tertiary care center and community clinics in the United States between February 2001 and March 2005. PARTICIPANTS: Two hundred twenty-eight patients with sickle cell disease, aged 18 to 74 years, and 36 control participants. MAIN OUTCOME MEASURES: RESULTS: Plasma arginase activity was significantly elevated in patients with sickle cell disease, with highest activity found in patients with secondary pulmonary hypertension. Arginase activity correlated with the arginine- ornithine ratio, and lower ratios were associated with greater severity of pulmonary hypertension and with mortality in this population (risk ratio, 2.5; 95% confidence interval [CI], 1.2-5.2; P = .006). Global arginine bioavailability, characterized by the ratio of arginine to ornithine plus citrulline, was also strongly associated with mortality (risk ratio, 3.6; 95% CI, 1.5-8.3; P<.001). Increased plasma arginase activity was correlated with increased intravascular hemolytic rate and, to a lesser extent, with markers of inflammation and soluble adhesion molecule levels. CONCLUSIONS:
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Authors | Claudia R Morris, Gregory J Kato, Mirjana Poljakovic, Xunde Wang, William C Blackwelder, Vandana Sachdev, Stanley L Hazen, Elliott P Vichinsky, Sidney M Morris Jr, Mark T Gladwin |
Journal | JAMA
(JAMA)
Vol. 294
Issue 1
Pg. 81-90
(Jul 06 2005)
ISSN: 1538-3598 [Electronic] United States |
PMID | 15998894
(Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, N.I.H., Intramural, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
- Amino Acids
- Nitric Oxide
- Arginine
- Ornithine
- Arginase
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Topics |
- Adult
- Aged
- Amino Acids
(blood)
- Anemia, Sickle Cell
(blood, complications, enzymology, mortality)
- Arginase
(blood, metabolism)
- Arginine
(metabolism)
- Case-Control Studies
- Female
- Humans
- Hypertension, Pulmonary
(etiology)
- Male
- Middle Aged
- Nitric Oxide
(blood)
- Ornithine
(blood)
- Proportional Hazards Models
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