Endogenous nitric oxide synthase inhibitors in sickle cell disease: abnormal levels and correlations with pulmonary hypertension, desaturation, haemolysis, organ dysfunction and death

Summary Pulmonary hypertension (PH) in patients with sickle cell disease (SCD) is linked to intravascular haemolysis, impaired nitric oxide bioavailability, renal dysfunction, and early mortality. Asymmetric dimethylarginine (ADMA), an endogenous inhibitor of nitric oxide synthases (NOS), is associa...

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Bibliographic Details
Published in:British journal of haematology 2009-05, Vol.145 (4), p.506-513
Main Authors: Kato, Gregory J., Wang, Zeneng, Machado, Roberto F., Blackwelder, William C., Taylor 6th, James G., Hazen, Stanley L.
Format: Article
Language:English
Subjects:
Anemia, Sickle Cell - blood
Anemia, Sickle Cell - mortality
Anemias. Hemoglobinopathies
arginine
Arginine - analogs & derivatives
Arginine - blood
asymmetric dimethylarginine
Biological and medical sciences
Case-Control Studies
Citrulline - blood
Diseases of red blood cells
Follow-Up Studies
Hematologic and hematopoietic diseases
Hemolysis
Humans
Hypertension, Pulmonary - blood
Hypertension, Pulmonary - mortality
Kidney Diseases - blood
Kidney Diseases - mortality
Linear Models
Medical sciences
Nitric Oxide Synthase - antagonists & inhibitors
omega-N-Methylarginine - blood
Oxygen - blood
Pneumology
Proportional Hazards Models
pulmonary hypertension
Pulmonary hypertension. Acute cor pulmonale. Pulmonary embolism. Pulmonary vascular diseases
Risk
sickle cell disease
Survival Rate
symmetric dimethylarginine
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Summary:Summary Pulmonary hypertension (PH) in patients with sickle cell disease (SCD) is linked to intravascular haemolysis, impaired nitric oxide bioavailability, renal dysfunction, and early mortality. Asymmetric dimethylarginine (ADMA), an endogenous inhibitor of nitric oxide synthases (NOS), is associated with vascular disease in other populations. We determined the plasma concentrations for several key arginine metabolites and their relationships to clinical variables in 177 patients with SCD and 29 control subjects: ADMA, symmetric dimethylarginine (SDMA), NG‐monomethyl L‐arginine (L‐NMMA), N‐omega‐hydroxy‐L‐arginine (NOHA), arginine and citrulline. The median ADMA was significantly higher in SCD than controls (0·94  μmol/l vs. 0·31 μmol/l, P 
ISSN:0007-1048
1365-2141
DOI:10.1111/j.1365-2141.2009.07658.x