Nutrition Management for the Preterm Infant With PAH Deficiency

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.