Nutrition Management for the Preterm Infant With PAH Deficiency

被引：0

作者：

Barr, Emily M. ^{[1
]}

Weihe, Tarine U. ^{[1
]}

Hillsman, Lori A. ^{[1
]}

Rowzer, Kasey D. ^{[1
]}

McGinty, Lindsey ^{[1
]}

Heese, Bryce A. ^{[2
]}

Gannon, Jennifer L. ^{[2
]}

机构：

[1] Childrens Mercy, Dept Nutr Serv, 2401 Gillham Rd, Kansas City, MO 64108 USA

[2] Childrens Mercy, Div Clin Genet, Kansas City, MO 64108 USA

来源：

TOPICS IN CLINICAL NUTRITION | 2020年 / 35卷 / 02期

关键词：

nutrition; PAH deficiency; phenylalanine; phenylketonuria; PKU; prematurity; preterm; REQUIREMENTS; CALCIUM;

D O I：

10.1097/TIN.0000000000000211

中图分类号：

R15 [营养卫生、食品卫生]; TS201 [基础科学];

学科分类号：

100403 ;

摘要：

The nutrition guidelines for management of phenylketonuria (PKU or phenylalanine hydroxlyase [PAH] deficiency) are well developed. One missing component is the phenylalanine (Phe) requirements for the preterm infant with PAH deficiency. It has been found that preterm infants at a postmenstrual age (PMA) of 30 and 32 weeks have required a Phe intake as high as 100 mg/kg, well above the current clinical guidelines available for the term infant. Three case presentations illustrate modifications that the clinician may need to consider when caring for the preterm infant with PAH deficiency during the weeks leading up to 40 weeks of PMA.

引用

页码：178 / 187

页数：10

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