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If carnitine supplements are not promptly started, patients with primary carnitine deficiency can present with an acute metabolic decompensation early in life, or later in life with skeletal and cardiac myopathy or sudden death from arrhythmia [82, 100, 102]
Duffy Shepulski Michael K
This may be the direct result of improved intramitochondrial homeostasis induced by L-carnitine in removing accumulating acyl moieties
