Abstract
Most of inherited metabolic disorders are rare, but a number of these disorders increases and so does a number of diagnosed patients due to a newborn screening and a development of new analytic methods. Inherited metabolic disorders are mostly serious.
Nutrition management has a significant role in a therapy of a part of these disorders, especially in so called small molecules disorders. It involves a regulated intake of a substrate, which metabolism is limited in a patient due to an enzymatic or a protein deficiency or functional disruption.
Supplementation of lacking metabolic products and essential nutrients is also a part of a nutrition management. The goals of a nutrition management are to reach and maintain a normal level of all metabolites and a normal growth and development of a patient.
This article reviews the basic principles of a nutrition management of most often occuring inherited metabolic disorders, which are managible by a diet.