Treatment
The aim of treatment for the patient with a UCD is to
maintain normal plasma ammonia and amino acid
concentrations with nutritional management and drug
therapy. Nutritional therapy for a UCD consists of a
protein-restricted diet supplemented with an EAA
mixture high in branched-chain amino acids (BCAAs)
to ensure adequate intakes of essential and conditionally
essential amino acids. Enough protein-free energy
must be administered both to permit the normal
turnover of protein necessary at all ages and to prevent
net catabolism of body proteins. Monitoring of nutritional
status becomes even more critical when both
dialysis and medications are used to lower plasma ammonia concentrations, as dialysis may also decrease
the concentrations of nutrients in the body and
increase catabolism (Pupim et al 2004). The purpose
of this paper is to describe current nutritional management
practices for UCDs, including acute treatment,
treatment during surgical procedures, and
management of the chronic phase of these disorders.