Iron Status in Children With Recurrent Episodes of Tumefactive Cerebral Demyelination
Iron is a vital element in the multifactorial initiation of myelination. It is required for cholesterol and lipid biosynthesis, both key components of myelin. Iron also plays an important role in energy production by mitochondrial oxidative metabolism which occurs in myelin-producing oligodentrocyte...
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Veröffentlicht in: | Journal of child neurology 2010-11, Vol.25 (11), p.1401-1407 |
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creator | van Toorn, Ronald Schoeman, Johan F. Solomons, Regan Rensburg, Megan A. van Rensburg, Susan J. |
description | Iron is a vital element in the multifactorial initiation of myelination. It is required for cholesterol and lipid biosynthesis, both key components of myelin. Iron also plays an important role in energy production by mitochondrial oxidative metabolism which occurs in myelin-producing oligodentrocytes at a higher rate than in any other cell. Iron deficiency can, therefore, result in decreased oligodendrocyte survival and defective myelination. This led us to investigate iron status in 2 consecutive children with multiple sclerosis who presented with recurrent episodes of tumefactive demyelination. Testing revealed nonanemic iron deficiency in both patients. Discontinuation of iron supplementation in both children resulted in recurrent decreased iron parameters which can indicate mutations in proteins responsible for regulation of iron uptake. Further studies are warranted to explore the association of low iron in children presenting with recurrent episodes of tumefactive demyelination. |
doi_str_mv | 10.1177/0883073810366179 |
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It is required for cholesterol and lipid biosynthesis, both key components of myelin. Iron also plays an important role in energy production by mitochondrial oxidative metabolism which occurs in myelin-producing oligodentrocytes at a higher rate than in any other cell. Iron deficiency can, therefore, result in decreased oligodendrocyte survival and defective myelination. This led us to investigate iron status in 2 consecutive children with multiple sclerosis who presented with recurrent episodes of tumefactive demyelination. Testing revealed nonanemic iron deficiency in both patients. Discontinuation of iron supplementation in both children resulted in recurrent decreased iron parameters which can indicate mutations in proteins responsible for regulation of iron uptake. Further studies are warranted to explore the association of low iron in children presenting with recurrent episodes of tumefactive demyelination.</description><identifier>ISSN: 0883-0738</identifier><identifier>EISSN: 1708-8283</identifier><identifier>DOI: 10.1177/0883073810366179</identifier><identifier>PMID: 20395637</identifier><language>eng</language><publisher>Los Angeles, CA: SAGE Publications</publisher><subject>Cerebral Cortex - pathology ; Child, Preschool ; Children ; Cholesterol ; Demyelinating Diseases - blood ; Demyelinating Diseases - pathology ; Demyelination ; Hemoglobins - metabolism ; Humans ; Infant ; Iron ; Iron - blood ; Lipids ; Magnetic Resonance Imaging ; Male ; Mitochondria ; Multiple sclerosis ; Mutation ; Myelination ; Nutrient deficiency ; Oligodendrocytes ; Oxidative metabolism ; Recurrence ; Supplementation</subject><ispartof>Journal of child neurology, 2010-11, Vol.25 (11), p.1401-1407</ispartof><rights>The Author(s) 2010</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c368t-4b1bc3afb84b62410727dbb4ca117ce296971787648c81d137449e58959016c13</citedby><cites>FETCH-LOGICAL-c368t-4b1bc3afb84b62410727dbb4ca117ce296971787648c81d137449e58959016c13</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://journals.sagepub.com/doi/pdf/10.1177/0883073810366179$$EPDF$$P50$$Gsage$$H</linktopdf><linktohtml>$$Uhttps://journals.sagepub.com/doi/10.1177/0883073810366179$$EHTML$$P50$$Gsage$$H</linktohtml><link.rule.ids>314,780,784,21810,27915,27916,43612,43613</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/20395637$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>van Toorn, Ronald</creatorcontrib><creatorcontrib>Schoeman, Johan F.</creatorcontrib><creatorcontrib>Solomons, Regan</creatorcontrib><creatorcontrib>Rensburg, Megan A.</creatorcontrib><creatorcontrib>van Rensburg, Susan J.</creatorcontrib><title>Iron Status in Children With Recurrent Episodes of Tumefactive Cerebral Demyelination</title><title>Journal of child neurology</title><addtitle>J Child Neurol</addtitle><description>Iron is a vital element in the multifactorial initiation of myelination. It is required for cholesterol and lipid biosynthesis, both key components of myelin. Iron also plays an important role in energy production by mitochondrial oxidative metabolism which occurs in myelin-producing oligodentrocytes at a higher rate than in any other cell. Iron deficiency can, therefore, result in decreased oligodendrocyte survival and defective myelination. This led us to investigate iron status in 2 consecutive children with multiple sclerosis who presented with recurrent episodes of tumefactive demyelination. Testing revealed nonanemic iron deficiency in both patients. Discontinuation of iron supplementation in both children resulted in recurrent decreased iron parameters which can indicate mutations in proteins responsible for regulation of iron uptake. Further studies are warranted to explore the association of low iron in children presenting with recurrent episodes of tumefactive demyelination.</description><subject>Cerebral Cortex - pathology</subject><subject>Child, Preschool</subject><subject>Children</subject><subject>Cholesterol</subject><subject>Demyelinating Diseases - blood</subject><subject>Demyelinating Diseases - pathology</subject><subject>Demyelination</subject><subject>Hemoglobins - metabolism</subject><subject>Humans</subject><subject>Infant</subject><subject>Iron</subject><subject>Iron - blood</subject><subject>Lipids</subject><subject>Magnetic Resonance Imaging</subject><subject>Male</subject><subject>Mitochondria</subject><subject>Multiple sclerosis</subject><subject>Mutation</subject><subject>Myelination</subject><subject>Nutrient deficiency</subject><subject>Oligodendrocytes</subject><subject>Oxidative metabolism</subject><subject>Recurrence</subject><subject>Supplementation</subject><issn>0883-0738</issn><issn>1708-8283</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2010</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><recordid>eNqFkM1LAzEQxYMotlbvniQ3T6uZTZqPo9SvQkFQi8clm53VyH7UZFfof--WVg-CeBqG-b03vEfIKbALAKUumdacKa6BcSlBmT0yBsV0olPN98l4c0429xE5ivGdMaanhh2SUcq4mUquxmQ5D21Dnzrb9ZH6hs7efFUEbOiL797oI7o-DFtHb1Y-tgVG2pb0ua-xtK7zn0hnGDAPtqLXWK-x8o3tfNsck4PSVhFPdnNClrc3z7P7ZPFwN59dLRLHpe4SkUPuuC1zLXKZCmAqVUWeC2eHdA5TI40CpZUU2mkogCshDE61GVKAdMAn5HzruwrtR4-xy2ofHVaVbbDtY2amQgIYqf8lhy9MgOBqINmWdKGNMWCZrYKvbVhnwLJN69nv1gfJ2c68z2ssfgTfNQ9AsgWifcXsve1DM9Tyt-EXtJWIKA</recordid><startdate>20101101</startdate><enddate>20101101</enddate><creator>van Toorn, Ronald</creator><creator>Schoeman, Johan F.</creator><creator>Solomons, Regan</creator><creator>Rensburg, Megan A.</creator><creator>van Rensburg, Susan J.</creator><general>SAGE Publications</general><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><scope>7TK</scope></search><sort><creationdate>20101101</creationdate><title>Iron Status in Children With Recurrent Episodes of Tumefactive Cerebral Demyelination</title><author>van Toorn, Ronald ; Schoeman, Johan F. ; Solomons, Regan ; Rensburg, Megan A. ; van Rensburg, Susan J.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c368t-4b1bc3afb84b62410727dbb4ca117ce296971787648c81d137449e58959016c13</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2010</creationdate><topic>Cerebral Cortex - pathology</topic><topic>Child, Preschool</topic><topic>Children</topic><topic>Cholesterol</topic><topic>Demyelinating Diseases - blood</topic><topic>Demyelinating Diseases - pathology</topic><topic>Demyelination</topic><topic>Hemoglobins - metabolism</topic><topic>Humans</topic><topic>Infant</topic><topic>Iron</topic><topic>Iron - blood</topic><topic>Lipids</topic><topic>Magnetic Resonance Imaging</topic><topic>Male</topic><topic>Mitochondria</topic><topic>Multiple sclerosis</topic><topic>Mutation</topic><topic>Myelination</topic><topic>Nutrient deficiency</topic><topic>Oligodendrocytes</topic><topic>Oxidative metabolism</topic><topic>Recurrence</topic><topic>Supplementation</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>van Toorn, Ronald</creatorcontrib><creatorcontrib>Schoeman, Johan F.</creatorcontrib><creatorcontrib>Solomons, Regan</creatorcontrib><creatorcontrib>Rensburg, Megan A.</creatorcontrib><creatorcontrib>van Rensburg, Susan J.</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><collection>Neurosciences Abstracts</collection><jtitle>Journal of child neurology</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>van Toorn, Ronald</au><au>Schoeman, Johan F.</au><au>Solomons, Regan</au><au>Rensburg, Megan A.</au><au>van Rensburg, Susan J.</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Iron Status in Children With Recurrent Episodes of Tumefactive Cerebral Demyelination</atitle><jtitle>Journal of child neurology</jtitle><addtitle>J Child Neurol</addtitle><date>2010-11-01</date><risdate>2010</risdate><volume>25</volume><issue>11</issue><spage>1401</spage><epage>1407</epage><pages>1401-1407</pages><issn>0883-0738</issn><eissn>1708-8283</eissn><abstract>Iron is a vital element in the multifactorial initiation of myelination. It is required for cholesterol and lipid biosynthesis, both key components of myelin. Iron also plays an important role in energy production by mitochondrial oxidative metabolism which occurs in myelin-producing oligodentrocytes at a higher rate than in any other cell. Iron deficiency can, therefore, result in decreased oligodendrocyte survival and defective myelination. This led us to investigate iron status in 2 consecutive children with multiple sclerosis who presented with recurrent episodes of tumefactive demyelination. Testing revealed nonanemic iron deficiency in both patients. Discontinuation of iron supplementation in both children resulted in recurrent decreased iron parameters which can indicate mutations in proteins responsible for regulation of iron uptake. Further studies are warranted to explore the association of low iron in children presenting with recurrent episodes of tumefactive demyelination.</abstract><cop>Los Angeles, CA</cop><pub>SAGE Publications</pub><pmid>20395637</pmid><doi>10.1177/0883073810366179</doi><tpages>7</tpages></addata></record> |
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subjects | Cerebral Cortex - pathology Child, Preschool Children Cholesterol Demyelinating Diseases - blood Demyelinating Diseases - pathology Demyelination Hemoglobins - metabolism Humans Infant Iron Iron - blood Lipids Magnetic Resonance Imaging Male Mitochondria Multiple sclerosis Mutation Myelination Nutrient deficiency Oligodendrocytes Oxidative metabolism Recurrence Supplementation |
title | Iron Status in Children With Recurrent Episodes of Tumefactive Cerebral Demyelination |
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