Abnormality of erythrocyte membrane n-3 long chain polyunsaturated fatty acids in sickle cell haemoglobin C (HbSC) disease is not as remarkable as in sickle cell anaemia (HbSS)
Sickle cell disease (SCD) is a group of inherited blood disorders in which clinical illness results from the presence of erythrocytes with sickled haemoglobin (HbS). Blood vessel occlusion is a fundamental pathological process in SCD. Sickle cell haemoglobin C (HbSC) disease and sickle cell anaemia...
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| container_title | Prostaglandins, leukotrienes and essential fatty acids |
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| creator | Ren, Hongmei Ghebremeskel, Kebreab Okpala, Iheanyi Ugochukwu, Cynthia C. Crawford, Michael Ibegbulam, Obike |
| description | Sickle cell disease (SCD) is a group of inherited blood disorders in which clinical illness results from the presence of erythrocytes with sickled haemoglobin (HbS). Blood vessel occlusion is a fundamental pathological process in SCD. Sickle cell haemoglobin C (HbSC) disease and sickle cell anaemia (HbSS) share some pathophysiology and clinical manifestations. However, the former is generally less severe. Erythrocytes of HbSC patients have longer life span, reduced haemolysis, and lower propensity to adhere to vascular endothelium than those of their HbSS counterparts. The structure and function of erythrocytes are strongly modulated by membrane long chain polyunsaturated fatty acids (LCPUFA). We have tested the possibility that HbSC and HbSS patients have different membrane fatty acid composition consistent with the difference in their clinical severity. Steady-state patients, 9 HbSC and 28 HbSS, and 15 HbAA were studied. The HbSC patients had a higher level of linoleic (LA,
P
<
0.05
) and docosahexaenoic (DHA,
P
<
0.05
) acids, and lower arachidonic acid (AA,
P
<
0.01
) and AA/eicosapentaenoic acid (EPA) ratio
(
P
<
0.05
)
in erythrocyte choline phosphoglycerides (CPG) compared with the HbSS group. Similarly, the level of EPA was higher and AA/EPA ratio
(
P
<
0.01
)
lower in serine phosphoglycerides of the HbSC patients. In contrast to the HbSC, the HbSS group had lower levels of EPA
(
P
<
0.001
)
, DHA
(
P
<
0.05
)
, total n-3 metabolites and total n-3 fatty acids
(
P
<
0.001
)
in erythrocyte CPG compared with the healthy HbAA controls. Moreover, the HbSS patients with disease complications compared with those without complications had reduced DHA and total n-3 fatty acids
(
P
<
0.005
)
in erythrocyte CPG. The abnormalities in erythrocyte in LCPUFA which is manifested by an increase in AA and a decrease in EPA and DHA in HbSS relative to HbSC disease observed in this study are consistent with the contrast in clinical severity between the two entities. |
| doi_str_mv | 10.1016/j.plefa.2005.10.002 |
| format | Article |
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P
<
0.05
) and docosahexaenoic (DHA,
P
<
0.05
) acids, and lower arachidonic acid (AA,
P
<
0.01
) and AA/eicosapentaenoic acid (EPA) ratio
(
P
<
0.05
)
in erythrocyte choline phosphoglycerides (CPG) compared with the HbSS group. Similarly, the level of EPA was higher and AA/EPA ratio
(
P
<
0.01
)
lower in serine phosphoglycerides of the HbSC patients. In contrast to the HbSC, the HbSS group had lower levels of EPA
(
P
<
0.001
)
, DHA
(
P
<
0.05
)
, total n-3 metabolites and total n-3 fatty acids
(
P
<
0.001
)
in erythrocyte CPG compared with the healthy HbAA controls. Moreover, the HbSS patients with disease complications compared with those without complications had reduced DHA and total n-3 fatty acids
(
P
<
0.005
)
in erythrocyte CPG. The abnormalities in erythrocyte in LCPUFA which is manifested by an increase in AA and a decrease in EPA and DHA in HbSS relative to HbSC disease observed in this study are consistent with the contrast in clinical severity between the two entities.]]></description><identifier>ISSN: 0952-3278</identifier><identifier>EISSN: 1532-2823</identifier><identifier>DOI: 10.1016/j.plefa.2005.10.002</identifier><identifier>PMID: 16314081</identifier><language>eng</language><publisher>Kidlington: Elsevier Ltd</publisher><subject>Adult ; Aged ; Anemia, Sickle Cell - blood ; Anemia, Sickle Cell - complications ; Anemias. Hemoglobinopathies ; Biological and medical sciences ; Diseases of red blood cells ; Erythrocyte Membrane - chemistry ; Erythrocytes - chemistry ; Fatty Acids, Omega-3 - blood ; Fatty Acids, Unsaturated - analysis ; Fatty Acids, Unsaturated - blood ; Fundamental and applied biological sciences. Psychology ; Hematologic and hematopoietic diseases ; Hemoglobin SC Disease - blood ; Hemoglobins - analysis ; Humans ; Leukocyte Count ; Medical sciences ; Middle Aged ; Phosphatidylcholines - blood ; Phosphatidylethanolamines - blood ; Phosphatidylserines - blood ; Triglycerides - blood ; Vertebrates: endocrinology</subject><ispartof>Prostaglandins, leukotrienes and essential fatty acids, 2006, Vol.74 (1), p.1-6</ispartof><rights>2005 Elsevier Ltd</rights><rights>2006 INIST-CNRS</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c387t-59f8f369de37cd276dc8acfbbc4287e3e6c0d59c61a9110f5ff8e8c2ae56df6a3</citedby><cites>FETCH-LOGICAL-c387t-59f8f369de37cd276dc8acfbbc4287e3e6c0d59c61a9110f5ff8e8c2ae56df6a3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktohtml>$$Uhttps://dx.doi.org/10.1016/j.plefa.2005.10.002$$EHTML$$P50$$Gelsevier$$H</linktohtml><link.rule.ids>314,780,784,3550,4024,27923,27924,27925,45995</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=17377190$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/16314081$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Ren, Hongmei</creatorcontrib><creatorcontrib>Ghebremeskel, Kebreab</creatorcontrib><creatorcontrib>Okpala, Iheanyi</creatorcontrib><creatorcontrib>Ugochukwu, Cynthia C.</creatorcontrib><creatorcontrib>Crawford, Michael</creatorcontrib><creatorcontrib>Ibegbulam, Obike</creatorcontrib><title>Abnormality of erythrocyte membrane n-3 long chain polyunsaturated fatty acids in sickle cell haemoglobin C (HbSC) disease is not as remarkable as in sickle cell anaemia (HbSS)</title><title>Prostaglandins, leukotrienes and essential fatty acids</title><addtitle>Prostaglandins Leukot Essent Fatty Acids</addtitle><description><![CDATA[Sickle cell disease (SCD) is a group of inherited blood disorders in which clinical illness results from the presence of erythrocytes with sickled haemoglobin (HbS). Blood vessel occlusion is a fundamental pathological process in SCD. Sickle cell haemoglobin C (HbSC) disease and sickle cell anaemia (HbSS) share some pathophysiology and clinical manifestations. However, the former is generally less severe. Erythrocytes of HbSC patients have longer life span, reduced haemolysis, and lower propensity to adhere to vascular endothelium than those of their HbSS counterparts. The structure and function of erythrocytes are strongly modulated by membrane long chain polyunsaturated fatty acids (LCPUFA). We have tested the possibility that HbSC and HbSS patients have different membrane fatty acid composition consistent with the difference in their clinical severity. Steady-state patients, 9 HbSC and 28 HbSS, and 15 HbAA were studied. The HbSC patients had a higher level of linoleic (LA,
P
<
0.05
) and docosahexaenoic (DHA,
P
<
0.05
) acids, and lower arachidonic acid (AA,
P
<
0.01
) and AA/eicosapentaenoic acid (EPA) ratio
(
P
<
0.05
)
in erythrocyte choline phosphoglycerides (CPG) compared with the HbSS group. Similarly, the level of EPA was higher and AA/EPA ratio
(
P
<
0.01
)
lower in serine phosphoglycerides of the HbSC patients. In contrast to the HbSC, the HbSS group had lower levels of EPA
(
P
<
0.001
)
, DHA
(
P
<
0.05
)
, total n-3 metabolites and total n-3 fatty acids
(
P
<
0.001
)
in erythrocyte CPG compared with the healthy HbAA controls. Moreover, the HbSS patients with disease complications compared with those without complications had reduced DHA and total n-3 fatty acids
(
P
<
0.005
)
in erythrocyte CPG. The abnormalities in erythrocyte in LCPUFA which is manifested by an increase in AA and a decrease in EPA and DHA in HbSS relative to HbSC disease observed in this study are consistent with the contrast in clinical severity between the two entities.]]></description><subject>Adult</subject><subject>Aged</subject><subject>Anemia, Sickle Cell - blood</subject><subject>Anemia, Sickle Cell - complications</subject><subject>Anemias. Hemoglobinopathies</subject><subject>Biological and medical sciences</subject><subject>Diseases of red blood cells</subject><subject>Erythrocyte Membrane - chemistry</subject><subject>Erythrocytes - chemistry</subject><subject>Fatty Acids, Omega-3 - blood</subject><subject>Fatty Acids, Unsaturated - analysis</subject><subject>Fatty Acids, Unsaturated - blood</subject><subject>Fundamental and applied biological sciences. Psychology</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Hemoglobin SC Disease - blood</subject><subject>Hemoglobins - analysis</subject><subject>Humans</subject><subject>Leukocyte Count</subject><subject>Medical sciences</subject><subject>Middle Aged</subject><subject>Phosphatidylcholines - blood</subject><subject>Phosphatidylethanolamines - blood</subject><subject>Phosphatidylserines - blood</subject><subject>Triglycerides - blood</subject><subject>Vertebrates: endocrinology</subject><issn>0952-3278</issn><issn>1532-2823</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2006</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNp9kc1uEzEUhS0EomnhCZCQN6B2McE_mb9FF1UEFKkSi8LaumNfN04942A7SPNWPCJOE6kLJFaWjr9zdO89hLzjbMkZbz5tlzuPFpaCsbooS8bEC7LgtRSV6IR8SRasr0UlRdudkfOUtqwQnK9ekzPeSL5iHV-QPzfDFOII3uWZBksxznkTg54z0hHHIcKEdKok9WF6oHoDbqK74Of9lCDvI2Q01EIuZtDOJFq-k9OPHqlG7-kGcAwPPgxFX9PL2-F-fUWNSwgJqUt0CplCohFHiI8wFBv8kwFTCXHw5L6_ekNeWfAJ357eC_Lzy-cf69vq7vvXb-ubu0rLrs1V3dvOyqY3KFttRNsY3YG2w6BXomtRYqOZqXvdcOg5Z7a2tsNOC8C6MbYBeUE-HnN3MfzaY8pqdOkwTzlI2CfVMsEkb-oCyiOoY0gpolW76Mo6s-JMHYpSW_VUlDoUdRBLDcX1_hS_H0Y0z55TMwX4cAIgafC2FKFdeuZa2ba8Z4W7PnJYjvHbYVRJO5w0GhdRZ2WC--8gfwHVZrSx</recordid><startdate>2006</startdate><enddate>2006</enddate><creator>Ren, Hongmei</creator><creator>Ghebremeskel, Kebreab</creator><creator>Okpala, Iheanyi</creator><creator>Ugochukwu, Cynthia C.</creator><creator>Crawford, Michael</creator><creator>Ibegbulam, Obike</creator><general>Elsevier Ltd</general><general>Elsevier</general><scope>IQODW</scope><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>2006</creationdate><title>Abnormality of erythrocyte membrane n-3 long chain polyunsaturated fatty acids in sickle cell haemoglobin C (HbSC) disease is not as remarkable as in sickle cell anaemia (HbSS)</title><author>Ren, Hongmei ; Ghebremeskel, Kebreab ; Okpala, Iheanyi ; Ugochukwu, Cynthia C. ; Crawford, Michael ; Ibegbulam, Obike</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c387t-59f8f369de37cd276dc8acfbbc4287e3e6c0d59c61a9110f5ff8e8c2ae56df6a3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2006</creationdate><topic>Adult</topic><topic>Aged</topic><topic>Anemia, Sickle Cell - blood</topic><topic>Anemia, Sickle Cell - complications</topic><topic>Anemias. Hemoglobinopathies</topic><topic>Biological and medical sciences</topic><topic>Diseases of red blood cells</topic><topic>Erythrocyte Membrane - chemistry</topic><topic>Erythrocytes - chemistry</topic><topic>Fatty Acids, Omega-3 - blood</topic><topic>Fatty Acids, Unsaturated - analysis</topic><topic>Fatty Acids, Unsaturated - blood</topic><topic>Fundamental and applied biological sciences. Psychology</topic><topic>Hematologic and hematopoietic diseases</topic><topic>Hemoglobin SC Disease - blood</topic><topic>Hemoglobins - analysis</topic><topic>Humans</topic><topic>Leukocyte Count</topic><topic>Medical sciences</topic><topic>Middle Aged</topic><topic>Phosphatidylcholines - blood</topic><topic>Phosphatidylethanolamines - blood</topic><topic>Phosphatidylserines - blood</topic><topic>Triglycerides - blood</topic><topic>Vertebrates: endocrinology</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Ren, Hongmei</creatorcontrib><creatorcontrib>Ghebremeskel, Kebreab</creatorcontrib><creatorcontrib>Okpala, Iheanyi</creatorcontrib><creatorcontrib>Ugochukwu, Cynthia C.</creatorcontrib><creatorcontrib>Crawford, Michael</creatorcontrib><creatorcontrib>Ibegbulam, Obike</creatorcontrib><collection>Pascal-Francis</collection><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>Prostaglandins, leukotrienes and essential fatty acids</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Ren, Hongmei</au><au>Ghebremeskel, Kebreab</au><au>Okpala, Iheanyi</au><au>Ugochukwu, Cynthia C.</au><au>Crawford, Michael</au><au>Ibegbulam, Obike</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Abnormality of erythrocyte membrane n-3 long chain polyunsaturated fatty acids in sickle cell haemoglobin C (HbSC) disease is not as remarkable as in sickle cell anaemia (HbSS)</atitle><jtitle>Prostaglandins, leukotrienes and essential fatty acids</jtitle><addtitle>Prostaglandins Leukot Essent Fatty Acids</addtitle><date>2006</date><risdate>2006</risdate><volume>74</volume><issue>1</issue><spage>1</spage><epage>6</epage><pages>1-6</pages><issn>0952-3278</issn><eissn>1532-2823</eissn><abstract><![CDATA[Sickle cell disease (SCD) is a group of inherited blood disorders in which clinical illness results from the presence of erythrocytes with sickled haemoglobin (HbS). Blood vessel occlusion is a fundamental pathological process in SCD. Sickle cell haemoglobin C (HbSC) disease and sickle cell anaemia (HbSS) share some pathophysiology and clinical manifestations. However, the former is generally less severe. Erythrocytes of HbSC patients have longer life span, reduced haemolysis, and lower propensity to adhere to vascular endothelium than those of their HbSS counterparts. The structure and function of erythrocytes are strongly modulated by membrane long chain polyunsaturated fatty acids (LCPUFA). We have tested the possibility that HbSC and HbSS patients have different membrane fatty acid composition consistent with the difference in their clinical severity. Steady-state patients, 9 HbSC and 28 HbSS, and 15 HbAA were studied. The HbSC patients had a higher level of linoleic (LA,
P
<
0.05
) and docosahexaenoic (DHA,
P
<
0.05
) acids, and lower arachidonic acid (AA,
P
<
0.01
) and AA/eicosapentaenoic acid (EPA) ratio
(
P
<
0.05
)
in erythrocyte choline phosphoglycerides (CPG) compared with the HbSS group. Similarly, the level of EPA was higher and AA/EPA ratio
(
P
<
0.01
)
lower in serine phosphoglycerides of the HbSC patients. In contrast to the HbSC, the HbSS group had lower levels of EPA
(
P
<
0.001
)
, DHA
(
P
<
0.05
)
, total n-3 metabolites and total n-3 fatty acids
(
P
<
0.001
)
in erythrocyte CPG compared with the healthy HbAA controls. Moreover, the HbSS patients with disease complications compared with those without complications had reduced DHA and total n-3 fatty acids
(
P
<
0.005
)
in erythrocyte CPG. The abnormalities in erythrocyte in LCPUFA which is manifested by an increase in AA and a decrease in EPA and DHA in HbSS relative to HbSC disease observed in this study are consistent with the contrast in clinical severity between the two entities.]]></abstract><cop>Kidlington</cop><pub>Elsevier Ltd</pub><pmid>16314081</pmid><doi>10.1016/j.plefa.2005.10.002</doi><tpages>6</tpages></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 0952-3278 |
| ispartof | Prostaglandins, leukotrienes and essential fatty acids, 2006, Vol.74 (1), p.1-6 |
| issn | 0952-3278 1532-2823 |
| language | eng |
| recordid | cdi_proquest_miscellaneous_70203165 |
| source | MEDLINE; Elsevier ScienceDirect Journals Complete |
| subjects | Adult Aged Anemia, Sickle Cell - blood Anemia, Sickle Cell - complications Anemias. Hemoglobinopathies Biological and medical sciences Diseases of red blood cells Erythrocyte Membrane - chemistry Erythrocytes - chemistry Fatty Acids, Omega-3 - blood Fatty Acids, Unsaturated - analysis Fatty Acids, Unsaturated - blood Fundamental and applied biological sciences. Psychology Hematologic and hematopoietic diseases Hemoglobin SC Disease - blood Hemoglobins - analysis Humans Leukocyte Count Medical sciences Middle Aged Phosphatidylcholines - blood Phosphatidylethanolamines - blood Phosphatidylserines - blood Triglycerides - blood Vertebrates: endocrinology |
| title | Abnormality of erythrocyte membrane n-3 long chain polyunsaturated fatty acids in sickle cell haemoglobin C (HbSC) disease is not as remarkable as in sickle cell anaemia (HbSS) |
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