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Thiamine-Responsive and Non-responsive Patients with PDHC-E1 Deficiency: A Retrospective Assessment

Pyruvate dehydrogenase complex (PDHC) deficiency is a disorder of energy metabolism that leads to a range of clinical manifestations. We sought to characterise clinical manifestations and biochemical, neuroimaging and molecular findings in thiamine-responsive and nonresponsive PDHC-deficient patient...

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Bibliografiske detaljer
Udgivet i:JIMD Rep
Main Authors: van Dongen, Sanne, Brown, Ruth M., Brown, Garry K., Thorburn, David R., Boneh, Avihu
Format: Artigo
Sprog:Inglês
Udgivet: Springer Berlin Heidelberg 2014
Fag:
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4270867/
https://ncbi.nlm.nih.gov/pubmed/24718837
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/8904_2014_293
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