| Methemoglobinemia and ascorbate deficiency in hemoglobin E β thalassemia: metabolic and clinical implications. | |
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MedLine Citation:
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PMID: 22885163 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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During investigations of the phenotypic diversity of hemoglobin (Hb) E β thalassemia, a patient was encountered with persistently high levels of methemoglobin associated with a left-shift in the oxygen dissociation curve, profound ascorbate deficiency, and clinical features of scurvy; these abnormalities were corrected by treatment with vitamin C. Studies of erythropoietin production before and after treatment suggested that, as in an ascorbate-deficient murine model, the human hypoxia induction factor pathway is not totally dependent on ascorbate levels. A follow-up study of 45 patients with HbE β thalassemia showed that methemoglobin levels were significantly increased and that there was also a significant reduction in plasma ascorbate levels. Haptoglobin levels were significantly reduced, and the high frequency of the 2.2 haptoglobin genotype may place an additional pressure on ascorbate as a free-radical scavenger in this population. There was, in addition, a highly significant correlation between methemoglobin levels, splenectomy, and factors that modify the degree of globin-chain imbalance. Because methemoglobin levels are modified by several mechanisms and may play a role in both adaptation to anemia and vascular damage, there is a strong case for its further study in other forms of thalassemia and sickle-cell anemia, particularly when splenic function is defective. |
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Authors:
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Angela Allen; Christopher Fisher; Anuja Premawardhena; Dayananda Bandara; Ashok Perera; Stephen Allen; Timothy St Pierre; Nancy Olivieri; David Weatherall |
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Publication Detail:
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Type: Case Reports; Journal Article; Research Support, Non-U.S. Gov't Date: 2012-08-10 |
Journal Detail:
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Title: Blood Volume: 120 ISSN: 1528-0020 ISO Abbreviation: Blood Publication Date: 2012 Oct |
Date Detail:
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Created Date: 2012-10-15 Completed Date: 2013-01-17 Revised Date: 2013-05-20 |
Medline Journal Info:
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Nlm Unique ID: 7603509 Medline TA: Blood Country: United States |
Other Details:
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Languages: eng Pagination: 2939-44 Citation Subset: AIM; IM |
Affiliation:
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MRC Molecular Haematology Unit, Weatherall Institute of Molecular Medicine, University of Oxford, John Radcliffe Hospital, Oxford, United Kingdom. |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adult Ascorbic Acid / metabolism* Ascorbic Acid Deficiency / etiology*, metabolism, pathology Family Female Hemoglobin E / metabolism* Humans Male Methemoglobin / metabolism* Methemoglobinemia / etiology*, metabolism, pathology Young Adult beta-Thalassemia / complications*, metabolism |
| Grant Support | |
ID/Acronym/Agency:
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//Medical Research Council; //Wellcome Trust |
| Chemical | |
Reg. No./Substance:
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50-81-7/Ascorbic Acid; 9008-37-1/Methemoglobin; 9034-61-1/Hemoglobin E |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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