CASE REVIEWNutrition Management for the Preterm Infant With PAH DeficiencyBarr, Emily M. MS, RD; Weihe, Tarine U. MS, RD; Hillsman, Lori A. MS, RD; Rowzer, Kasey D. MS, RD; McGinty, Lindsey MS, RD; Heese, Bryce A. MD; Gannon, Jennifer L. MDAuthor Information Department of Nutrition Services (Mss Barr, Weihe, Hillsman, Rowzer, and McGinty) and Division of Clinical Genetics (Drs Heese and Gannon), Children's Mercy, Kansas City, Missouri. Correspondence: Emily M. Barr, MS, RD, Department of Nutrition Services, Children's Mercy, 2401 Gillham Rd, Kansas City, MO 64108 (email@example.com). The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article. Topics in Clinical Nutrition: April/June 2020 - Volume 35 - Issue 2 - p 178-187 doi: 10.1097/TIN.0000000000000211 Buy Metrics Abstract 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. © 2020 Wolters Kluwer Health, Inc. All rights reserved.