Prevention of maternal PKU dietetic management of pregnant PKU women with P-AM maternal

Objectives: Since 1975 mass screening is obligatory in Hungary, young women reached the age of potential conception in the mid-eighties. Our aim is to prepare PKU patients for planned pregnancy, for the strict diet before conception and during pregnancy by which phe level of their blood is under control and they can have healthy babies.

Method and results: Altogether 8 PKU women, 5 HPA women and 18 children were studied. After non-planned pregnancy, two women who refused any of diet gave birth 2–2 microcephal babies, moreover two spontaneous abortus took place. Phe levels of the mothers' blood during pregnancy were between 450 and 1500 mmol/l. After planned pregnancy 5 healthy babies were born where strict protein and phe diet was on already three months before conception with P-AM maternal nutritive addition. Phe levels of the mothers' blood were between 60 and 120 mmol/l. Among HPA patients, whose blood levels were controlled regularly and found appropriate, 7 healthy babies were born without any diet treatment.

Conclusions: PHU and HPA women on strict diet are able to give birth healthy offspring nevertheless continuous personal contact repeated many times a week is indispensable for it. Beside control of the phe level of blood, control of all proteins, tyrosine, and iron supply is also indispensable. Among the amino-acid mixtures, P-AM maternal provided the tyrosine and peptide supply the most properly however, for the tolerance of it, dietetic practices had to also be applied (frozen in ice cubes against of morning indisposition).

In summary, babies of PKU women on adequate diet have no disadvantages in their development compared to other children and their somato-menthal (IQ 98–118) development.