Carnitine deficiency may be associated with sudden death.
Pathogenesis: carnitine deficiency is associated with impaired energy production from long-chain fatty acids. This is important for the function of cardiac and skeletal muscle.
Risk factors for sudden death:
(1) untreated carnitine deficiency (failure to provide L-carnitine)
(2) physiologic stress
(3) cardiomyopathy of carnitine deficiency
(4) cardiac arrhythmias (prolonged or short QT interval, ventricular fibrillation, bradycardia, atrial arrhythmias, tall peaked T waves in the mid-precordial leads)
(5) exposure to drugs that reduce carnitine levels
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