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Article: Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) triggered by valproate therapy

TitleMitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) triggered by valproate therapy
Authors
KeywordsMELAS syndrome
Mitochondrial encephalomyopathy
Mitochondrial mutation
Valproate
Issue Date1997
PublisherSpringer Verlag. The Journal's web site is located at http://link.springer.de/link/service/journals/00431/index.htm
Citation
European Journal Of Pediatrics, 1997, v. 156 n. 7, p. 562-564 How to Cite?
AbstractWe report in this study a patient who developed repeated convulsions as a result of valproate therapy. MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) was subsequently diagnosed and a nucleotide 3243 A→G mutation was detected in the mitochondrial DNA. This mutation predisposes the patient to the detrimental effects of valproate on oxidative phosphorylation. Conclusion: We support the suggestion of Ponchaut et al. [14] that valproate should not be given to patients suspected of having mitochondrial diseases. In addition, for patients whose seizures worsen with valproate therapy, an inborn error of mitochondrial metabolism should be suspected. The underlying mitochondrial DNA defects should be sought for family screening and genetic counselling.
Persistent Identifierhttp://hdl.handle.net/10722/148073
ISSN
2023 Impact Factor: 3.0
2023 SCImago Journal Rankings: 0.984
ISI Accession Number ID
References

 

DC FieldValueLanguage
dc.contributor.authorLam, CWen_US
dc.contributor.authorLau, CHen_US
dc.contributor.authorWilliams, JCen_US
dc.contributor.authorChan, YWen_US
dc.contributor.authorWong, LJCen_US
dc.date.accessioned2012-05-29T06:10:42Z-
dc.date.available2012-05-29T06:10:42Z-
dc.date.issued1997en_US
dc.identifier.citationEuropean Journal Of Pediatrics, 1997, v. 156 n. 7, p. 562-564en_US
dc.identifier.issn0340-6199en_US
dc.identifier.urihttp://hdl.handle.net/10722/148073-
dc.description.abstractWe report in this study a patient who developed repeated convulsions as a result of valproate therapy. MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes) was subsequently diagnosed and a nucleotide 3243 A→G mutation was detected in the mitochondrial DNA. This mutation predisposes the patient to the detrimental effects of valproate on oxidative phosphorylation. Conclusion: We support the suggestion of Ponchaut et al. [14] that valproate should not be given to patients suspected of having mitochondrial diseases. In addition, for patients whose seizures worsen with valproate therapy, an inborn error of mitochondrial metabolism should be suspected. The underlying mitochondrial DNA defects should be sought for family screening and genetic counselling.en_US
dc.languageengen_US
dc.publisherSpringer Verlag. The Journal's web site is located at http://link.springer.de/link/service/journals/00431/index.htmen_US
dc.relation.ispartofEuropean Journal of Pediatricsen_US
dc.subjectMELAS syndrome-
dc.subjectMitochondrial encephalomyopathy-
dc.subjectMitochondrial mutation-
dc.subjectValproate-
dc.subject.meshAnticonvulsants - Adverse Effectsen_US
dc.subject.meshChilden_US
dc.subject.meshDna, Mitochondrial - Analysisen_US
dc.subject.meshElectron Transport Complex Iv - Drug Effectsen_US
dc.subject.meshHumansen_US
dc.subject.meshMelas Syndrome - Chemically Induced - Diagnosis - Geneticsen_US
dc.subject.meshMaleen_US
dc.subject.meshOxidative Phosphorylation - Drug Effectsen_US
dc.subject.meshPedigreeen_US
dc.subject.meshValproic Acid - Adverse Effects - Pharmacologyen_US
dc.titleMitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) triggered by valproate therapyen_US
dc.typeArticleen_US
dc.identifier.emailLam, CW:ching-wanlam@pathology.hku.hken_US
dc.identifier.authorityLam, CW=rp00260en_US
dc.description.naturelink_to_subscribed_fulltexten_US
dc.identifier.doi10.1007/s004310050663en_US
dc.identifier.pmid9243242en_US
dc.identifier.scopuseid_2-s2.0-0030758778en_US
dc.relation.referenceshttp://www.scopus.com/mlt/select.url?eid=2-s2.0-0030758778&selection=ref&src=s&origin=recordpageen_US
dc.identifier.volume156en_US
dc.identifier.issue7en_US
dc.identifier.spage562en_US
dc.identifier.epage564en_US
dc.identifier.isiWOS:A1997XJ24000015-
dc.publisher.placeGermanyen_US
dc.identifier.issnl0340-6199-

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