title: Parenteral nutrition in patients with inborn errors of metabolism - a therapeutic problem.
abstract: Parenteral nutrition is now a standard part of supportive treatment 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 beta-oxidation disorders associated with necrotizing enterocolitis and congenital heart disease. Thus, limitations of intravenous lipid intake made it difficult to maintain a good nutritional status. The third case was phenylketonuria associated with a facial region tumour (rhabdomyosarcoma), in which parenteral nutrition was complicated because of a high phenylalanine content in the amino acid formulas for parenteral nutrition. The fourth patient was a child with late-diagnosed tyrosinemia type 1, complicated with encephalopathy - during intensive care treatment the patient needed nutritional support, including parenteral nutrition - we observed amino acid formula problems similar to those in the phenylketonuria patient. Parenteral nutrition in children with inborn errors of metabolism is a rare, but very important therapeutic problem. Total parenteral nutrition formulas are not prepared for this group of diseases.
authors.affiliation: Department of Pediatric Gastroenterology and Metabolism, I Chair of Pediatrics, Poznan University of Medical Sciences, Szpitalna, Poznan, Poland. lukasz@jerozolima.poznan.pl.
authors.forename: L
authors.initials: L
authors.lastname: Kaluzny
authors.forename: M
authors.initials: M
authors.lastname: Szczepanik
authors.forename: Z
authors.initials: Z
authors.lastname: Siwinska-Mrozek
authors.forename: M
authors.initials: M
authors.lastname: Borkowska-Klos
authors.forename: W
authors.initials: W
authors.lastname: Cichy
authors.forename: J
authors.initials: J
authors.lastname: Walkowiak
mesh_terms: D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008661:Metabolism, Inborn Errors / Q000628:therapy*; D010288:Parenteral Nutrition / Q000379:methods*