Literature review for - Flourish Paediatrics

phenylalanine free formula was altered according to the infant’s blood-spot phenylalanine
level. Blood-spot phenylalanine level was monitored twice weekly to age 3 months then
measured once weekly. The overall median mean duration of breastfeeding supplemented
with phenylalanine-free formula was 27.4 weeks. (range 0.7-65.7 weeks). All infants had
acceptable metabolic control and growth indicating that breastfeeding plus a phenylalaninefree
formula provides safe and acceptable management for infants newly diagnosed with
PKU (Sweeney, Netting et al. 2009).
The management of PKU in Europe
In Europe there are no consensus guidelines for the optimal dietary care of PKU infants. The
recommendations for breastfeeding PKU infants vary across countries. In Germany a fixed
quantity of breast milk, is recommended followed by Phe-free formula until satiety, whereas
in Belgium, Norway, Poland, Denmark and the UK recommendations are for a fixed quantity
of formula prior to breastfeeding until satiety. The quantity of infant protein substitute is
adjusted according to the infant’s blood Phe concentrations. The centre in the Netherlands
advises an alternating breastfeeding schedule, where the frequency of breastfeeding depends
on the infant’s blood Phe concentration (van Rijn, Bekhof et al. 2003; Ahring, Belanger-
Quintana et al. 2009).
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