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Pyruvate dehydrogenase deficiency (PDH), a deficiency in the E1 (pyruvate dehydrogenase) component of the pyruvate dehydrogenase complex, is one of the.

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Presentation on theme: "Pyruvate dehydrogenase deficiency (PDH), a deficiency in the E1 (pyruvate dehydrogenase) component of the pyruvate dehydrogenase complex, is one of the."— Presentation transcript:

1 Pyruvate dehydrogenase deficiency (PDH), a deficiency in the E1 (pyruvate dehydrogenase) component of the pyruvate dehydrogenase complex, is one of the most common genetic defects of mitochondrial energy metabolism. PDH deficiency is an extremely heterogeneous condition, both in clinical presentation and in the severity of the biochemical abnormality, with a clinical spectrum ranging from fatal lactic acidosis in the newborn period to a chronic neurodegenerative condition with gross structural abnormalities in the central nervous system. Source: Newborn, Atlas of Pediatric EEG Citation: Laoprasert P. Atlas of Pediatric EEG; 2011 Available at: Accessed: October 26, 2017 Copyright © 2017 McGraw-Hill Education. All rights reserved

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