Dietary guidelines for inborn errors of metabolism

Among the most vexing decisions in the management of metabolic diseases that require restriction of dietary components is the determination of adequate and sufficient diet. In metabolic diseases such as urea cycle disorders or organic acidemias, under-restriction of protein can result in hyperammonemia or acidosis whereas over-restriction can lead to poor growth. Each condition may have unique dietary allowances and patients can be variably affected. The field of metabolism is re-examining the rigid guidelines of metabolic formulas that have allowed these patients to survive.

http://ift.tt/2wxnzJr