The fraternal twin sisters are presenting with episodes of fasting hypoglycemia, hypoketonemia, and muscle weakness occurring only during periods of calorific deprivation. One twin dies at 5 months of age. The surviving twin develops a cardiomyopathy, which is diagnosed at 3 years of age. Carnitine esters are elevated in muscle tissue and in serum. Which of the following is the most likely diagnosis?
a-L-Iduronidase deficiency Acid maltase deficiency (a 1,4-glucosidase deficiency) Carnitine uptake deficiency Long-chain acyl CoA dehydrogenase (LCAD) deficiency