Behavioural factors related to metabolic control in patients with phenylketonuria
- 23 July 2004
- journal article
- Published by Wiley in Journal of Inherited Metabolic Disease
- Vol. 28 (5), 627-637
- https://doi.org/10.1007/s10545-005-0014-0
Abstract
Background. The objective of this study was to determine the importance of parental factors possibly related to dietary control in early and continuously treated patients with phenylketonuria (PKU). Methods. A questionnaire was disseminated among parents of 238 patients with PKU born after the nationwide introduction of newborn screening for PKU (1 September 1974) until 31 December 1995. The questionnaire was based on a behavioural model measuring people’s attitudes, subjective norms, and self-efficacy. Dietary control was defined on the basis of mean phenyl- alanine (Phe) concentration of the PKU patients measured between 1 January 1994 and 31 December 1996. Results. Response rate was 71%. Attitudes: children of parents who believed that their child adheres well to the diet, even if his or her Phe concentrations are sometimes too high, had lower Phe concentrations than children of parents who disagree with this statement (adjusted difference −103 μmol/L, p < 0.001). Subjective norm: Phe concentrations were higher when parents answered that their relatives did not approve when their child deviates from the diet (p = 0.004). Self-efficacy: children of parents who reported difficulties in having their child eat the synthetic protein substitute three times a day had higher Phe concentrations than those of parents who did not have such difficulties (adjusted difference 156 μmol/L, p = 0.007). Conclusion. More attention should be given to parents having their child eat the synthetic protein substitute at least three times a day and to teaching parents to keep strictly to the diet without being too rigid. These factors were strongly associated to dietary control and may be amenable to change.Keywords
This publication has 25 references indexed in Scilit:
- Plasma phenylalanine in patients with phenylketonuria self-managing their dietArchives of Disease in Childhood, 2005
- Blood phenylalanine control in adolescents with phenylketonuriaInternational Journal of Adolescent Medicine and Health, 2004
- Sustained attention and inhibition of cognitive interference in treated phenylketonuria: associations with concurrent and lifetime phenylalanine concentrationsNeuropsychologia, 2002
- Prefrontal Dysfunction in Early and Continuously Treated PhenylketonuriaDevelopmental Neuropsychology, 1999
- Factors affecting the variation in plasma phenylalanine in patients with phenylketonuria on diet.Archives of Disease in Childhood, 1996
- Predictors of mean phenylalanine levels during the first five years of life in patients with phenylketonuria who were treated earlyActa Paediatrica, 1994
- Psychological and social findings in adolescents with phenylketonuriaEuropean Journal of Pediatrics, 1992
- The theory of planned behaviorOrganizational Behavior and Human Decision Processes, 1991
- Children with PhenylketonuriaJournal of Developmental & Behavioral Pediatrics, 1991
- Behavior disturbance in 8-year-old children with early treated phenylketonuria: Report from the MRC/DHSS phenylketonuria registerThe Journal of Pediatrics, 1988