The Turkish Journal of Pediatrics
2009 , Vol 51 , Num 5
Changing Dietary Practices in Phenylketonuria
1Birmingham Children’s Hospital, Birmingham, United Kingdom, and 2Department of Nutrition and Dietetics, Hacettepe
University Faculty of Health Sciences, Ankara, Turkey
In recent years, there has been much focus on research on non-dietary
treatments in phenylketonuria (PKU). However, diet is likely to remain
the major treatment for many years to come, since it has continued to be
developed and consistent improvements have occurred. For example, with
protein substitute, studies have tried to define the optimal dose and timing
of intake; changes in palatability and presentation appear to have led to the
long-term maintenance of acceptable blood phenylalanine control in teenage
patients, and the low phenylalanine protein source glycomacropeptide is
being considered as an alternative source to non-phenylalanine amino acids.
Some countries are now adopting a simpler approach to dietary management,
allowing a wider range of lower low phenylalanine foods without measurement,
in combination with controlled phenylalanine exchange systems. Patients
with PKU who are partially responsive to sapropterin dihydrochloride still
require some dietary treatment. Long-term studies are required for examining
the combined use of sapropterin dihydrochloride and diet to determine its
impact on nutritional adequacy, growth, and blood phenylalanine variability.
Generally, whether diet is used alone or in combination with sapropterin
dihydrochloride, its true impact on quality of life and lifestyle should be
investigated. Overall, it is likely we will continue to see many changes in
the dietary treatment in the next five years, and hopefully this will lead to
a better outcome for patients.
Keywords :
phenylketonuria, dietary practices