Pyruvate dehydrogenase complex deficiency | 食品添加物合法業者資訊網
由HPavlu-Pereira著作·2020·被引用3次—Thepyruvatedehydrogenasecomplex(PDC)catalyzestheirreversibledecarboxylationofpyruvateintoacetyl-CoA.PDCdeficiencycanbecausedby ...
General informationThe broad phenotypic spectrum of PDC deficiency, common to many other genetic mitochondrial disorders, often hampers the achievement of a rapid diagnosis. Patients were suspected to be PDC deficient based on clinical signs /symptoms and biochemical data, namely elevated plasma lactate (L) and pyruvate (P) levels with low L/P ratio and/or impaired PDC activity, but only those with genetic confirmation were considered in this study. Thus, a total of 13 individuals, all of European ancestry, were diagnosed (Table 1). The prevalence of PDC deficiency in Portugal is therefore estimated to be 1/790,000, comparable to the prevalence of 1/1,000,000 reported by Orphanet, the portal of rare diseases and orphan drugs, and also to the values calculated for the French population (1/827,000 – 1/1,135,000) [8, 18].
Table 1 Genetic, biochemical, clinical and therapeutics d...Pyruvate dehydrogenase deficiency | 食品添加物合法業者資訊網
Pyruvate dehydrogenase complex deficiency | 食品添加物合法業者資訊網
Pyruvate dehydrogenase deficiency | 食品添加物合法業者資訊網
Pyruvate Dehydrogenase Deficiency (PDCD) | 食品添加物合法業者資訊網
Pyruvate Dehydrogenase Complex Deficiency | 食品添加物合法業者資訊網
Clinical and genetic spectrum of pyruvate dehydrogenase ... | 食品添加物合法業者資訊網
The spectrum of pyruvate dehydrogenase complex deficiency ... | 食品添加物合法業者資訊網
Pyruvate dehydrogenase deficiency | 食品添加物合法業者資訊網
Pyruvate Dehydrogenase Complex Deficiency | 食品添加物合法業者資訊網
Pyruvate dehydrogenase complex deficiency | 食品添加物合法業者資訊網
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