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Hyperhomocysteinemia is associated with low plasma pyridoxine levels in children with sickle cell disease.
MedLine Citation:
PMID:  12142786     Owner:  NLM     Status:  MEDLINE    
Elevated plasma homocysteine levels have been shown to be a risk factor for endothelial cell damage and thrombosis, which are implicated in sickle cell disease (SCD)-related vaso-occlusion. The aim of this study was to determine the prevalence of hyperhomocysteinemia in SCD. Fasting and postmethionine load (PML) homocysteine, red cell folate, and the MTHFR C677T mutation were determined in 77 patients with SCD and 110 African-American controls. Plasma methylmalonic acid and pyridoxine levels were determined in 54 patients and all controls. For analysis, the subjects were divided into two age groups (2-10 years and 10.1-21 years). In both age groups, median PML homocysteine levels were significantly elevated in patients with SCD compared with controls. Fasting homocysteine levels were elevated in patients with SCD versus controls only in those older than 10 years. Hyperhomocysteinemia was noted in 38% of patients versus 7% in controls. Folate levels were higher among patients than controls and showed a significant negative correlation with PML homocysteine levels in patients with SCD. Pyridoxine levels in patients with SCD were significantly lower than in controls and showed a negative correlation with PML homocysteine levels. Among patients with SCD, pyridoxine deficiency was more common (62%) among those with hyperhomocysteinemia compared with those with normal homocysteine levels (30%). Homozygosity for the MTHFR C677T mutation was rare. These data suggest that children with SCD have significant hyperhomocysteinemia, associated with pyridoxine and relative folate deficiencies.
Vinod V Balasa; Karen A Kalinyak; Judy A Bean; Davis Stroop; Ralph A Gruppo
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  24     ISSN:  1077-4114     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:    2002 Jun-Jul
Date Detail:
Created Date:  2002-07-26     Completed Date:  2002-08-16     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  374-9     Citation Subset:  IM    
Cincinnati Comprehensive Sickle Cell Center of the Division of Hematology/Oncology, Children's Hospital Medical Center, Ohio 45229, USA.
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MeSH Terms
Anemia, Sickle Cell / complications*,  therapy
Case-Control Studies
Child, Preschool
Folic Acid / blood*
Homocysteine / blood*
Hyperhomocysteinemia / blood,  etiology*
Methionine / diagnostic use
Methylenetetrahydrofolate Reductase (NADPH2)
Oxidoreductases Acting on CH-NH Group Donors / genetics
Pyridoxine / blood*,  deficiency
Risk Factors
Vitamin B 12 / blood
Grant Support
Reg. No./Substance:
454-28-4/Homocysteine; 59-30-3/Folic Acid; 63-68-3/Methionine; 65-23-6/Pyridoxine; 68-19-9/Vitamin B 12; EC 1.5.-/Oxidoreductases Acting on CH-NH Group Donors; EC Reductase (NADPH2)

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