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Article: Adrenoleukodystrophy in a Chinese boy

TitleAdrenoleukodystrophy in a Chinese boy
Authors
KeywordsAdrenoleukodystrophy
Erucic acid
Gammaglobulin
Oleic acid
Very long chain fatty acid
Issue Date1992
PublisherElsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/braindev
Citation
Brain And Development, 1992, v. 14 n. 4, p. 276-277 How to Cite?
AbstractWe report the first Chinese boy with adrenoleukodystrophy (ALD) who presented with hyperpigmentation, behavioral change and demyelination shown in magnetic resonance imaging of the brain. ALD was confirmed by the elevation of very long chain fatty acid in the serum and biochemical evidence of adrenal insufficiency. A trial of special diet with restriction of very long chain fatty acid and addition of glyceryl trierucate/glycerol trioleate oil (GTEO or Lorenzo's oil) failed to prevent clinical deterioration. The child had progressive visual loss and spastic tetraparesis despite dietary manipulation, adrenal steroid replacement and intravenous gammaglobulin treatment.
Persistent Identifierhttp://hdl.handle.net/10722/143491
ISSN
2015 Impact Factor: 1.785
2015 SCImago Journal Rankings: 0.840
ISI Accession Number ID

 

DC FieldValueLanguage
dc.contributor.authorWong, Ven_HK
dc.date.accessioned2011-12-12T03:51:08Z-
dc.date.available2011-12-12T03:51:08Z-
dc.date.issued1992en_HK
dc.identifier.citationBrain And Development, 1992, v. 14 n. 4, p. 276-277en_HK
dc.identifier.issn0387-7604en_HK
dc.identifier.urihttp://hdl.handle.net/10722/143491-
dc.description.abstractWe report the first Chinese boy with adrenoleukodystrophy (ALD) who presented with hyperpigmentation, behavioral change and demyelination shown in magnetic resonance imaging of the brain. ALD was confirmed by the elevation of very long chain fatty acid in the serum and biochemical evidence of adrenal insufficiency. A trial of special diet with restriction of very long chain fatty acid and addition of glyceryl trierucate/glycerol trioleate oil (GTEO or Lorenzo's oil) failed to prevent clinical deterioration. The child had progressive visual loss and spastic tetraparesis despite dietary manipulation, adrenal steroid replacement and intravenous gammaglobulin treatment.en_HK
dc.languageengen_US
dc.publisherElsevier BV. The Journal's web site is located at http://www.elsevier.com/locate/braindeven_HK
dc.relation.ispartofBrain and Developmenten_HK
dc.subjectAdrenoleukodystrophyen_HK
dc.subjectErucic aciden_HK
dc.subjectGammaglobulinen_HK
dc.subjectOleic aciden_HK
dc.subjectVery long chain fatty aciden_HK
dc.subject.meshAdrenoleukodystrophy/diet therapy/drug therapy/*pathologyen_US
dc.subject.meshBrain/pathologyen_US
dc.subject.meshChild, Preschoolen_US
dc.subject.meshErucic Acids/therapeutic useen_US
dc.subject.meshFludrocortisone/therapeutic useen_US
dc.subject.meshHumansen_US
dc.subject.meshHydrocortisone/therapeutic useen_US
dc.subject.meshMagnetic Resonance Imagingen_US
dc.subject.meshMaleen_US
dc.subject.meshOleic Acids/therapeutic useen_US
dc.subject.meshgamma-Globulins/therapeutic useen_US
dc.titleAdrenoleukodystrophy in a Chinese boyen_HK
dc.typeArticleen_HK
dc.identifier.emailWong, V:vcnwong@hku.hken_HK
dc.identifier.authorityWong, V=rp00334en_HK
dc.description.naturelink_to_subscribed_fulltext-
dc.identifier.pmid1279999-
dc.identifier.scopuseid_2-s2.0-0026709327en_HK
dc.identifier.volume14en_HK
dc.identifier.issue4en_HK
dc.identifier.spage276en_HK
dc.identifier.epage277en_HK
dc.identifier.isiWOS:A1992JL67300018-
dc.publisher.placeNetherlandsen_HK
dc.identifier.scopusauthoridWong, V=7202525632en_HK

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