Abstract:
: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 micromol/l vs. 0.31 micromol/l, P < 0.001). Patients with homozygous SCD had a remarkably lower ratio of arginine to ADMA (50 micromol/l vs. 237, P < 0.001). ADMA correlated with markers of haemolysis, low oxygen saturation and soluble adhesion molecules. PH was associated with high levels of ADMA and related metabolites. Higher ADMA level was associated with early mortality, remaining significant in a multivariate analysis. Subjects with homozygous SCD have high systemic levels of ADMA, associated with PH and early death, implicating ADMA as a functional NOS inhibitor in these patients. These defects and others converge on the nitric oxide pathway in homozygous SCD with vasculopathy.
journal_name
Br J Haematoljournal_title
British journal of haematologyauthors
Kato GJ,Wang Z,Machado RF,Blackwelder WC,Taylor JG 6th,Hazen SLdoi
10.1111/j.1365-2141.2009.07658.xsubject
Has Abstractpub_date
2009-05-01 00:00:00pages
506-13issue
4eissn
0007-1048issn
1365-2141pii
BJH7658journal_volume
145pub_type
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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doi:10.1111/j.1365-2141.1987.tb02333.x
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journal_title:British journal of haematology
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journal_title:British journal of haematology
pub_type: 杂志文章
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pub_type: 杂志文章
doi:10.1111/j.1365-2141.1990.tb07912.x
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journal_title:British journal of haematology
pub_type: 杂志文章
doi:10.1111/j.1365-2141.1981.tb02682.x
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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journal_title:British journal of haematology
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journal_title:British journal of haematology
pub_type: 杂志文章
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journal_title:British journal of haematology
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journal_title:British journal of haematology
pub_type: 杂志文章
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