Parenteral Nutrition in a Premature Infant with Phenylketonuria

Parenteral Nutrition in a Premature Infant with Phenylketonuria

Parenteral nutrition (PN) is now an important facet of the management of the extremely premature infant. However, its effects on those with inherited metabolic disease have not been well documented. We report an infant with classical phenylketonuria (PKU) who had unusually high serum phenylalanine a...

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Veröffentlicht in:JPEN. Journal of parenteral and enteral nutrition 1984-01, Vol.8 (1), p.42-44
Hauptverfasser: Cole, David E.C., Landry, Daniel A.
Format: Artikel
Sprache:eng
Schlagworte:
Amino Acids - administration & dosage
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Biological and medical sciences
Emergency and intensive care: neonates and children. Prematurity. Sudden death
Female
Humans
Infant, Newborn
Infant, Premature, Diseases - therapy
Intensive care medicine
Medical sciences
Parenteral Nutrition
Phenylalanine - administration & dosage
Phenylalanine - blood
Phenylketonurias - blood
Phenylketonurias - therapy
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Zusammenfassung:Parenteral nutrition (PN) is now an important facet of the management of the extremely premature infant. However, its effects on those with inherited metabolic disease have not been well documented. We report an infant with classical phenylketonuria (PKU) who had unusually high serum phenylalanine at 12 days of life (5200 μmol; >3.2 SD above our mean for PKU at 10-15 days of age) while on parenteral nutrition, despite a relatively high tolerance for phenylalanine on oral feeds at 3-4 months of life (97-128 mg/ kg/day; normal for PKU:
ISSN:0148-6071
1941-2444
DOI:10.1177/014860718400800142