Abstract
Lactate, pyruvate, acetoacetate, and 3-hydroxybutyrate are intermediary metabolites that normally occur in blood and all have an essential role in energy production. Their blood accumulation is a frequent cause of metabolic acidosis for children. Their determination in biological fluids is useful in the early detection, diagnosis and treatment follow up of abnormalities such as enzymes of pyruvate « carrefour » (PDH or PC defects), enzymes of Krebs cycle, neoglucogenesis, liver glycogenolysis, oxidation of fatty acids, ketogenesis, ketolysis or mitochondrial respiratory chain. Their relative blood concentrations are an expression of nutritional balance, providing a view of the metabolic disturbances arising in a patient and are useful tools to investigate intermediary metabolism in health and disease particularly in inherited metabolic diseases. Interpretation of those tests is provided including reference values and clinical cases from children affected with different diseases involved in their metabolism.
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Vassault, A. (2008). Lactate, Pyruvate, Acetoacetate and 3-Hydroxybutyrate. In: Blau, N., Duran, M., Gibson, K. (eds) Laboratory Guide to the Methods in Biochemical Genetics. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-76698-8_4
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DOI: https://doi.org/10.1007/978-3-540-76698-8_4
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