Metabolism
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Muscle weakness, heart failure and profound hypoglycaemia in a young girl
Objectives
By the end of this exercise you should be able to:
Explain how carnitine deficiency affects fatty acid metabolism, and how impaired fatty acid metabolism leads to non-ketotic fasting hypoglycaemia
Explain how impaired fatty acid metabolism leads to ready fatigue and muscle weakness, and cardiomyopathy, leading to heart failure
Explain how measurement of plasma enzymes can be useful in the diagnosis of liver and muscle disease
Describe the role of carnitine in the uptake of fatty acids into mitochondria for oxidation, and the roles of carnitine acyltransferase and the carnitine / acyl carnitine transport protein