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Family conditions and dietary control in phenylketonuria

  • Original Article
  • Published:
Journal of Inherited Metabolic Disease

Summary

Objective

This investigation is an attempt to describe coping with phenylketonuria (PKU) in order to understand some aspects underlying good compliance.

Methods

The coping concept was applied to PKU in two questionnaires. Self- and parental ratings were combined with assessments of phenylalanine levels and the severity of the disease. All Swedish patients with PKU born in 1980–91, a total of 53 children and youths with their parents, were invited to participate in the study and 41 (77%) of them did so.

Results

The patients turned out to have good compliance with the diet. The main result was that patients with separated or divorced parents were more likely to have higher phenylalanine levels and this association was not diminished by adjustment for the potential confounding factors.

Conclusion

Patients’ need for support must be judged individually according to different family conditions.

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Author information

Authors and Affiliations

  1. Department of Neuroscience, Pharmacology, Uppsala University, BMC, Box 593, SE-751 24, Uppsala, Sweden

    G. M. Olsson

  2. Clinical Epidemiology Unit, Department of Medicine, Karolinska University Hospital, Karolinska Institute, Stockholm, Sweden

    S. M. Montgomery

  3. Clinical Research Centre, Örebro University Hospital, Örebro, Sweden

    S. M. Montgomery

  4. Department of Primary Care and Social Medicine, Imperial College, London, UK

    S. M. Montgomery

  5. Department of Paediatrics, Karolinska University Hospital at Huddinge, Stockholm, Sweden

    G. M. Olsson & J. Alm

Authors
  1. G. M. Olsson
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  2. S. M. Montgomery
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  3. J. Alm
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Corresponding author

Correspondence to G. M. Olsson.

Additional information

Communicating editor: John Walter

Competing interests: None declared

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Olsson, G.M., Montgomery, S.M. & Alm, J. Family conditions and dietary control in phenylketonuria. J Inherit Metab Dis 30, 708–715 (2007). https://doi.org/10.1007/s10545-007-0493-2

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  • DOI: https://doi.org/10.1007/s10545-007-0493-2

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