Parenteral nutrition in patients with inborn errors of metabolism - a therapeutic problem

BACKGROUND: Parenteral nutri - tion is now a standard part of supportive treat - ment in pediatric departments. We describe four cases in which parenteral nutrition was extremely difficult due to coincidence with inborn errors of metabolism. The first two cases was fatty acid be - ta-oxidation disorders associated with necrotizing enterocolitis and congenital heart disease. Thus, limitations of intravenous lipid intake made it diffi - cult to maintain a good nutritional status. The third case was phenylketonuria associated with a facial region tumour (rhabdomyosarcoma), in which par - enteral nutrition was complicated because of a high phenylalanine content in the amino acid for - mulas for parenteral nutrition. The fourth patient was a child with late-diagnosed tyrosinemia type 1, complicated with encephalopathy - during in - tensive care treatment the patient needed nutri - tional support, including parenteral nutrition - we observed amino acid formula problems similar to those in the phenylketonuria patient. Parenteral nutrition in children with inborn er - rors of metabolism is a rare, but very important therapeutic problem. Total parenteral nutrition formulas are not prepared for this group of dis - eases.