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Two Siblings With Valproate-Related Hyperammonemia and Novel Mutations in Glutamine Synthetase (GLUL) Treated With Carglumic Acid

This case report describes 2 siblings with myoclonic epilepsy who had novel mutations in the glutamine synthetase (GLUL) gene: c.316C>T, p.(Arg106*) and c.42G>C, p.(Lys14Asn). Valproic acid improved seizure control, but was associated with hyperammonemic encephalopathy. Addition of carglumic a...

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Pubblicato in:Child Neurol Open
Autori principali: Bennett, Jennifer, Gilkes, Christy, Klassen, Karin, Kerr, Marina, Khan, Aneal
Natura: Artigo
Lingua:Inglês
Pubblicazione: SAGE Publications 2020
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC7585879/
https://ncbi.nlm.nih.gov/pubmed/33150193
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1177/2329048X20967880
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