Abstract
Significant advances in the understanding of the biochemical and molecular basis of inherited metabolic disorders have led to major improvements in their diagnosis, management, and treatment. With expansion of newborn screening and identification of children prior to their initial crisis, there is hope for a more optimistic outcome for the individuals with inherited metabolic disorders. Individual diet management is central to the treatment of most metabolic disorders. This article reviews the basic principles of management and steps necessary to minimize the frequency, duration, and severity of catabolic crisis in infants and children, with an emphasis on the use of total parenteral nutrition.