Breastfeeding in phenylketonuric and hyperphenylalaninemic infants : how and why

Phenylketonuria is a metabolic disorder caused by deficiency of the enzyme phenylalanine hydroxylase, which converts phenylalanine (phe) to tyrosine (tyr). In PKU children neurological and behavioral disorders can be prevented by the early introduction of a strict dietary regimen aimed to reduce blood phe levels to nontoxic concentrations, in particular for central nervous system (CNS); in hyperphenylalaninemic (HPA) patients a dietary treatment isn't indicated. In accordance with the WHO/UNICEF recommendation, breast feeding should be encouraged also in phenylketonuric infants. [...] Results and conclusion: mothers could theoretically keep on breast feeding their babies ensuring a close follow up by regular blood phe measurements in order to modify, if required, the dietary schedules. We may conclude that breast feeding duration rates in PKU/HPA infants are notably lower than in health population. We speculate that the diagnosis of a chronic disorder requiring a strict clinical and dietary management may negatively affect the maternal attitude towards breast feeding. After diagnosis, mothers need psychological and technical support to maintain breast feeding in order to gain the expected advantages on infant.