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

被引：10

作者：

Lin, H. J. ^{[1
]}

Kwong, A. M. ^{[1
]}

Carter, J. M. ^{[2
,3
]}

Ferreira, B. F. ^{[1
]}

Austin, M. F. ^{[1
]}

Devarajan, K. ^{[4
]}

Coleman, R. J. ^{[5
]}

Feuchtbaum, L. B. ^{[2
]}

Lorey, F. ^{[2
]}

Jonas, A. J. ^{[1
]}

机构：

[1] Harbor UCLA Med Ctr, Dept Pediat, Div Med Genet, Torrance, CA 90502 USA

[2] Calif Dept Publ Hlth, Genet Dis Screening Program, Richmond, CA USA

[3] Publ Hlth Fdn Enterprises, City Of Industry, CA USA

[4] St Francis Med Ctr, Dept Neonatol, Lynwood, CA USA

[5] Univ Calif Irvine, Dept Pediat, Orange, CA 92668 USA

来源：

JOURNAL OF PERINATOLOGY | 2011年 / 31卷 / 07期

关键词：

newborn screening; PKU; total parenteral nutrition; AMINO-ACIDS; PRETERM INFANT; PEDIATRICS; WEIGHT; GROWTH; TIME;

D O I：

10.1038/jp.2010.207

中图分类号：

R71 [妇产科学];

学科分类号：

100211 ;

摘要：

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

引用

页码：507 / 510

页数：4

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