Background Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine
metabolism caused by deficiency in the enzyme phenylalanine hydroxylase that converts …

Introduction: Phenylketonuria (PKU) is an inborn metabolic disorder that affects
phenylalanine catabolism. Early diagnosis and prompt treatment initiation are crucial for …

In addition to the numerous immunological and nutritional benefits that breast milk offers to
infants, its proportion in the diet must be limited or even excluded in the case of inborn errors …

Background Phenylketonuria (PKU) treatment consists of life-long protein restriction and
Phe-free medical foods for adequate nutritional intake and growth. A relationship between …

Phenylketonuria (PKU) management aims to control phenylalanine (Phe) intakes. In
newborns and infants this implies possible titration of Human milk (HM) with …

Commercial formula milk (FM) constitutes the best alternative to fulfill the nutritional
requirements of infants when breastfeeding is precluded. Here, we present the comparative …

Breastfeeding or standard infant formulas, alongside phenylalanine (Phe)-free protein
substitutes, constitute the dietary management for infants with PKU to guarantee protein …

Human milk (HM) offers important nutritional benefits. However, except for phenylketonuria
(PKU), there are little data on optimal levels of consumption of HM and a special formula free …

Background In phenylketonuria (PKU), weaning is considered more challenging when
compared to feeding healthy infants. The primary aim of weaning is to gradually replace …

This article highlights the significance of inborn errors of metabolism and focuses specifically
on phenylketonuria (PKU), a well-known inheritance disorder caused by the deficiency or …