Extremely high phenylalanine levels in a newborn on parenteral nutrition: phenylketonuria in the neonatal intensive care unit

作者：Lin H J^*; Kwong A M; Carter J M; Ferreira B F; Austin M F; Devarajan K; Coleman R J; Feuchtbaum L B; Lorey F; Jonas A J

来源：Journal of Perinatology, 2011, 31(7): 507-510.

DOI：10.1038/jp.2010.207

• newborn screening
• PKU
• total parenteral nutrition

A 1890-g newborn on total parenteral nutrition (TPN) had phenylalanine levels reaching 4164 mu M indicating phenylketonuria (PKU). Review of 64 PKU cases from the California Newborn Screening Program disclosed another newborn diagnosed while on TPN. Phenylalanine levels rose five times faster with TPN, as estimated from rates in these infants. Thus, TPN use is associated with very high phenylalanine levels in newborns with PKU. When starting TPN soon after birth (for example, on day 1), early detection of PKU-by newborn screening 12 to 24 h after infusions are begun-should be helpful in limiting exposures to toxic levels of phenylalanine. Journal of Perinatology (2011) 31, 507-510; doi:10.1038/jp.2010.207

• 出版日期2011-7
• 单位UCLA