Abstract
Low levels of plasma carnitine and reduced urinary carnitine excretion with persistently elevated plasma bilirubin levels, reactive hypoglycemia and generalized skeletal muscle weakness are described in a patient requiring long‐term total parenteral nutrition (TPN). Intravenous administration of L‐carnitine at 400 mg/day for 7 days and subsequently a maintenance dose of 60 mg/day corrected the plasma carnitine deficiency and reactive hypoglycemia and was associated with a return to normal plasma bilirubin levels and a restoration of skeletal muscle strength.