Possible oxidative stress involvement in congenital dyserythropoietic anemia type 1
Congenital dyserythropoietic anemia type 1 (CDA1) patients may suffer from iron overload, associated with oxidative damage. The aim of this study was to evaluate possible involvement of oxidative stress in the pathogenesis of CDA1. : Blood samples from 10 children diagnosed as CDA1 patients from fiv...
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| Veröffentlicht in: | The hematology journal : the official journal of the European Haematology Association 2001, Vol.2 (3), p.196-199 |
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| container_title | The hematology journal : the official journal of the European Haematology Association |
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| creator | Mazor, D Kapelushnik, J Shalev, H Meyerstein, N |
| description | Congenital dyserythropoietic anemia type 1 (CDA1) patients may suffer from iron overload, associated with oxidative damage. The aim of this study was to evaluate possible involvement of oxidative stress in the pathogenesis of CDA1.
: Blood samples from 10 children diagnosed as CDA1 patients from five Bedouin families, were studied. In this study, activities of superoxide dismutase and catalase were evaluated as well as methemoglobin, plasma total thiols, plasma total antioxidant capacity and glycerol lysis time.
Normal values were found for superoxide dismutase, methemoglobin, trolox equivalent antioxidant capacity and total plasma thiols in CDA1 patients. However average catalase levels were significantly reduced (P |
| doi_str_mv | 10.1038/sj.thj.6200080 |
| format | Article |
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: Blood samples from 10 children diagnosed as CDA1 patients from five Bedouin families, were studied. In this study, activities of superoxide dismutase and catalase were evaluated as well as methemoglobin, plasma total thiols, plasma total antioxidant capacity and glycerol lysis time.
Normal values were found for superoxide dismutase, methemoglobin, trolox equivalent antioxidant capacity and total plasma thiols in CDA1 patients. However average catalase levels were significantly reduced (P<0.001) and glycerol lysis test was significantly prolonged (P<0.001). Ferritin levels, which were slightly increased in all patients, positively correlated with catalase values (r = 0.74, P = 0.022).
Oxidative stress has not been proven in CDA1 pediatric patients. Some indications of oxidative damage exist, but it may not be directly related to the mechanism of anemia.</description><identifier>ISSN: 1466-4860</identifier><identifier>DOI: 10.1038/sj.thj.6200080</identifier><identifier>PMID: 11920245</identifier><language>eng</language><publisher>England</publisher><subject>Adolescent ; Anemia, Dyserythropoietic, Congenital - enzymology ; Anemia, Dyserythropoietic, Congenital - genetics ; Anemia, Dyserythropoietic, Congenital - metabolism ; Antioxidants - analysis ; Arabs - genetics ; Catalase - blood ; Child ; Child, Preschool ; Consanguinity ; Humans ; Infant ; Methemoglobinemia - etiology ; Models, Biological ; Oxidation-Reduction ; Oxidative Stress ; Sulfhydryl Compounds - blood ; Superoxide Dismutase - blood</subject><ispartof>The hematology journal : the official journal of the European Haematology Association, 2001, Vol.2 (3), p.196-199</ispartof><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c206t-f127e9cbd001e4758f6cbaec19b35d16c0303588696ebc5ed7f1916e10695943</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><link.rule.ids>314,776,780,4009,27902,27903,27904</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/11920245$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Mazor, D</creatorcontrib><creatorcontrib>Kapelushnik, J</creatorcontrib><creatorcontrib>Shalev, H</creatorcontrib><creatorcontrib>Meyerstein, N</creatorcontrib><title>Possible oxidative stress involvement in congenital dyserythropoietic anemia type 1</title><title>The hematology journal : the official journal of the European Haematology Association</title><addtitle>Hematol J</addtitle><description>Congenital dyserythropoietic anemia type 1 (CDA1) patients may suffer from iron overload, associated with oxidative damage. The aim of this study was to evaluate possible involvement of oxidative stress in the pathogenesis of CDA1.
: Blood samples from 10 children diagnosed as CDA1 patients from five Bedouin families, were studied. In this study, activities of superoxide dismutase and catalase were evaluated as well as methemoglobin, plasma total thiols, plasma total antioxidant capacity and glycerol lysis time.
Normal values were found for superoxide dismutase, methemoglobin, trolox equivalent antioxidant capacity and total plasma thiols in CDA1 patients. However average catalase levels were significantly reduced (P<0.001) and glycerol lysis test was significantly prolonged (P<0.001). Ferritin levels, which were slightly increased in all patients, positively correlated with catalase values (r = 0.74, P = 0.022).
Oxidative stress has not been proven in CDA1 pediatric patients. Some indications of oxidative damage exist, but it may not be directly related to the mechanism of anemia.</description><subject>Adolescent</subject><subject>Anemia, Dyserythropoietic, Congenital - enzymology</subject><subject>Anemia, Dyserythropoietic, Congenital - genetics</subject><subject>Anemia, Dyserythropoietic, Congenital - metabolism</subject><subject>Antioxidants - analysis</subject><subject>Arabs - genetics</subject><subject>Catalase - blood</subject><subject>Child</subject><subject>Child, Preschool</subject><subject>Consanguinity</subject><subject>Humans</subject><subject>Infant</subject><subject>Methemoglobinemia - etiology</subject><subject>Models, Biological</subject><subject>Oxidation-Reduction</subject><subject>Oxidative Stress</subject><subject>Sulfhydryl Compounds - blood</subject><subject>Superoxide Dismutase - blood</subject><issn>1466-4860</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2001</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNpFkD1PwzAQhj2AaPlYGZEntoS7fDjJiCq-pEog0d1KnAt1lMTBdivy7wlqJKa74blX7z2M3SKECHH-4NrQ79tQRACQwxlbYyJEkOQCVuzSuRYABWZwwVaIRQRRkq7Z54dxTlcdcfOj69LrI3HnLTnH9XA03ZF6Gvy8c2WGLxq0LzteT47s5PfWjEaT14qXA_W65H4aieM1O2_KztHNMq_Y7vlpt3kNtu8vb5vHbaAiED5oMMqoUFU996IkS_NGqKokhUUVpzUKBTHEaZ6LQlClUqqzBgsUhCCKtEjiK3Z_ih2t-T6Q87LXTlHXzWXMwckMoyJNonwGwxOo7PyspUaOVvelnSSC_DMnXStnc3IxNx_cLcmHqqf6H1-0xb8Jdm3p</recordid><startdate>2001</startdate><enddate>2001</enddate><creator>Mazor, D</creator><creator>Kapelushnik, J</creator><creator>Shalev, H</creator><creator>Meyerstein, N</creator><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>7X8</scope></search><sort><creationdate>2001</creationdate><title>Possible oxidative stress involvement in congenital dyserythropoietic anemia type 1</title><author>Mazor, D ; Kapelushnik, J ; Shalev, H ; Meyerstein, N</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c206t-f127e9cbd001e4758f6cbaec19b35d16c0303588696ebc5ed7f1916e10695943</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2001</creationdate><topic>Adolescent</topic><topic>Anemia, Dyserythropoietic, Congenital - enzymology</topic><topic>Anemia, Dyserythropoietic, Congenital - genetics</topic><topic>Anemia, Dyserythropoietic, Congenital - metabolism</topic><topic>Antioxidants - analysis</topic><topic>Arabs - genetics</topic><topic>Catalase - blood</topic><topic>Child</topic><topic>Child, Preschool</topic><topic>Consanguinity</topic><topic>Humans</topic><topic>Infant</topic><topic>Methemoglobinemia - etiology</topic><topic>Models, Biological</topic><topic>Oxidation-Reduction</topic><topic>Oxidative Stress</topic><topic>Sulfhydryl Compounds - blood</topic><topic>Superoxide Dismutase - blood</topic><toplevel>online_resources</toplevel><creatorcontrib>Mazor, D</creatorcontrib><creatorcontrib>Kapelushnik, J</creatorcontrib><creatorcontrib>Shalev, H</creatorcontrib><creatorcontrib>Meyerstein, N</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>The hematology journal : the official journal of the European Haematology Association</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Mazor, D</au><au>Kapelushnik, J</au><au>Shalev, H</au><au>Meyerstein, N</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Possible oxidative stress involvement in congenital dyserythropoietic anemia type 1</atitle><jtitle>The hematology journal : the official journal of the European Haematology Association</jtitle><addtitle>Hematol J</addtitle><date>2001</date><risdate>2001</risdate><volume>2</volume><issue>3</issue><spage>196</spage><epage>199</epage><pages>196-199</pages><issn>1466-4860</issn><abstract>Congenital dyserythropoietic anemia type 1 (CDA1) patients may suffer from iron overload, associated with oxidative damage. The aim of this study was to evaluate possible involvement of oxidative stress in the pathogenesis of CDA1.
: Blood samples from 10 children diagnosed as CDA1 patients from five Bedouin families, were studied. In this study, activities of superoxide dismutase and catalase were evaluated as well as methemoglobin, plasma total thiols, plasma total antioxidant capacity and glycerol lysis time.
Normal values were found for superoxide dismutase, methemoglobin, trolox equivalent antioxidant capacity and total plasma thiols in CDA1 patients. However average catalase levels were significantly reduced (P<0.001) and glycerol lysis test was significantly prolonged (P<0.001). Ferritin levels, which were slightly increased in all patients, positively correlated with catalase values (r = 0.74, P = 0.022).
Oxidative stress has not been proven in CDA1 pediatric patients. Some indications of oxidative damage exist, but it may not be directly related to the mechanism of anemia.</abstract><cop>England</cop><pmid>11920245</pmid><doi>10.1038/sj.thj.6200080</doi><tpages>4</tpages></addata></record> |
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| subjects | Adolescent Anemia, Dyserythropoietic, Congenital - enzymology Anemia, Dyserythropoietic, Congenital - genetics Anemia, Dyserythropoietic, Congenital - metabolism Antioxidants - analysis Arabs - genetics Catalase - blood Child Child, Preschool Consanguinity Humans Infant Methemoglobinemia - etiology Models, Biological Oxidation-Reduction Oxidative Stress Sulfhydryl Compounds - blood Superoxide Dismutase - blood |
| title | Possible oxidative stress involvement in congenital dyserythropoietic anemia type 1 |
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