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Abstract
Phenylketonuria (PKU) and hyperphenylalaninemia (HPA) are two genetic disorders that affect the metabolism of the amino acid phenylalanine (Phe), leading to increased serum Phe concentrations, and possible negative neurologic outcomes. PKU and HPA are manageable with lifelong dietary restriction of Phe. Diet restriction has been associated with increased energy intake, and unhealthy eating habits. Some evidence shows that children with PKU tend to weigh more than children without PKU. It is possible that this excess weight may be at least partially due to diet restriction. Although the restrictive nature of dietary treatment of PKU and HPA is well known, restricted eating behaviors have not been measured in this population. This study measures restricted eating behaviors in children with PKU and HPA using the Child-Feeing Questionnaire (CFQ). Surveys were sent by mail to the parents of 64 children with PKU or HPA that attend the UW PKU clinic. 13 children with PKU and 5 unaffected siblings were identified. In this small sample, no significant difference in restriction was found between children with PKU and their unaffected siblings. This result seems to support the effectiveness of current education practices at the UW PKU clinic.
