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Diet History Is a Reliable Predictor of Suboptimal Docosahexaenoic Acid Levels in Adult Patients with Phenylketonuria

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JIMD Reports, Volume 21

Part of the book series: JIMD Reports ((JIMD,volume 21))

Abstract

Background: Omega-3 long-chain polyunsaturated fatty acids (n3LCPUFA) levels are reduced in phenylketonuria (PKU). Recent care guidelines recommend essential fatty acid status is monitored in patients with PKU but access to such testing is limited. We hypothesized that information obtained on diet history would identify PKU adults with suboptimal levels of n3LCPUFA.

Methods: A 12-month single site prospective study was completed including 35 adults (age 18–46) attending a clinic for adults with inborn errors of metabolism. Levels of n3LCPUFA were correlated with estimated intake using a published food frequency questionnaire. n3LCPUFA levels were tested at a commercial laboratory and values > one SD below the laboratory mean value were considered suboptimal.

Results: Mean levels of docosahexaenoic acid (DHA) were lower and levels of eicosapentaenoic acid (EPA) and alpha-linoleic acid (ALA) higher in subjects with PKU than in laboratory controls. n3LCPUFA levels correlated with estimated intake (p <0.002). Diet history had a positive predictive value of 93% and negative predictive value of 90% to identify subjects with suboptimal n3LCPUFA levels.

Conclusions: Diet history is sufficient to predict adult subjects who may have low DHA levels and can be used to target testing or supplementation to those at risk. DHA levels are low despite high levels of ALA suggesting that supplementation, if indicated, should be with preformed DHA rather than with its precursors.

Competing interests: None declared

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Author information

Authors and Affiliations

  1. Adult Metabolic Diseases Clinic, Vancouver Hospital, Level 4 – 2775 Laurel Street, Vancouver, BC, Canada, V5Z 1M9

    T. Bosdet, J. Branov, C. Selvage & S. Sirrs

  2. Brain Research Center, University of British Columbia, 2211 Wesbrook Mall, Vancouver, BC, Canada, V6T 2B5

    M. Yousefi

Authors
  1. T. Bosdet
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  2. J. Branov
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  3. C. Selvage
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  4. M. Yousefi
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  5. S. Sirrs
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Corresponding author

Correspondence to J. Branov .

Editor information

Editors and Affiliations

  1. Division of Human Genetics, Medical University Innsbruck, Innsbruck, Austria

    Johannes Zschocke

  2. Division of Metabolism and Children’s Research Centre, University Children’s Hospital Zurich, Zurich, Switzerland

    Matthias Baumgartner

  3. Tulane University Medical School, New Orleans, Louisiana, USA

    Eva Morava

  4. Division of Child and Adolescent Neurology, Mayo Clinic, Rochester, Minnesota, USA

    Marc Patterson

  5. Clinical and Molecular Genetics Unit, UCL Institute of Child Health, London, United Kingdom

    Shamima Rahman

  6. Center for Child and Adolescent Medicine, Heidelberg University Hospital, Heidelberg, Germany

    Verena Peters

Additional information

Communicated by: Anita MacDonald, PhD, BSc

Appendices

Compliance with Ethics Guidelines

Taryn Bosdet, Jennifer Branov, Caroline Selvage, Masoud Yousefi, and Sandra Sirrs declare that they have no conflict of interest.

Contributions of Individual Authors

Taryn Bosdet and Jennifer Branov were involved in the conception, design, conduct of the study, and the interpretation of data and drafting of article including revision for content of this manuscript.

Caroline Selvage’s primary contribution was in the conduct of the study and data collection as well as the drafting of the manuscript.

Sandra Sirrs was involved in the analysis and interpretation of data as well as in the drafting and revision of the manuscript.

Masoud Yousefi performed analysis and interpretation of data and contributed to the drafting of the manuscript.

Informed Consent

All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1975, as revised in 2000(5). Informed consent was obtained from all patients being included in the study.

Appendix

Questions useful in assessing DHA intake and predicting DHA status (Questions 1–4 selected from Sublette et al. 2011):

  1. 1.

    How many times have you eaten fish or shellfish in any form?

    1. (1)

      Never

    2. (2)

      Less than 1 time each month

    3. (3)

      1 time each month

    4. (4)

      2–3 times each month

    5. (5)

      1 time each week

    6. (6)

      2 times each week

    7. (7)

      3–4 times each week

    8. (8)

      5–6 times each week

    9. (9)

      1 time each day

    10. (10)

      2 or more times each day

  1. 2.

    Each time you ate fish or shellfish, how much did you eat?

    1. (1)

      Less than 2 ounces or less than one fillet or less than 4 pieces of sushi

    2. (2)

      2–7 ounces or about 1 fillet or 4–14 pieces of sushi

    3. (3)

      More than 7 ounces or more than 1 fillet or more than 14 pieces of sushi

  1. 3.

    In the past 6 months, about how often did you use cod liver oil?

    1. (1)

      Never

    2. (2)

      Less than 1 time each month

    3. (3)

      1 time each month

    4. (4)

      2–3 times each month

    5. (5)

      1 time each week

    6. (6)

      2 times each week

    7. (7)

      3–4 times each week

    8. (8)

      5–6 times each week

    9. (9)

      1 time each day

    10. (10)

      2 or more times each day

  1. 4.

    In the past 6 months, have you used an omega-3 fatty acid or fish oil supplement at least once each week?

    1. (1)

      No

    2. (2)

      Yes – What type of an omega-3 fatty acid or fish oil supplement did you take?

      Please write the name of the supplement below:

    3. (3)

      Is the omega-3 fatty acid or fish oil supplement in pill or capsule form?

      1. (1)

        No

      2. (2)

        Yes – How much did you take?

        1. (1)

          1 pill or capsule each week

        2. (2)

          2 pills or capsules each week

        3. (3)

          3–4 pills or capsules each week

        4. (4)

          5–6 pills or capsules each week

        5. (5)

          1 pill or capsule each day

        6. (6)

          2 pills or capsules each day

        7. (7)

          3–4 pills or capsules each day

        8. (8)

          5 or more pills or capsules each

    4. (4)

      Is the omega-3 fatty acid or fish oil supplement (besides cod liver oil) in liquid form?

      1. (1)

        No

      2. (2)

        Yes – How much did you take?

        1. (1)

          Less than 1 tablespoon each week

        2. (2)

          1 tablespoon each week

        3. (3)

          2 tablespoons each week

        4. (4)

          3–4 tablespoons each week

        5. (5)

          5–6 tablespoons each week

        6. (6)

          1 tablespoon each day

        7. (7)

          2 tablespoons each day

        8. (8)

          3–4 tablespoons each day

        9. (9)

          5 or more tablespoons each day

    5. (5)

      Please write down the dosage of omega-3 fatty acids or fish oil supplement if you know it:

      • Dosage:

      • Pills or Capsules: _____mg DHA and ______mg EPA per pill/capsule

      • Liquid: _____mg DHA and ______mg EPA per tablespoon

      • Do not know dosage

  1. 5.

    Do you take a PKU Medical Food Product?

    • No

    • Yes

      Please write down the name of your PKU Medical Food:

  1. 6.

    On average, how much of this PKU Medical Food do you take each day (please write down what you are actually taking, not what would be ideal):

    • Scoops/packages: I take ______scoops/packages of formula each day

    • Grams of powder: I take _______grams of powder per day

    • Tetra’s/pouches: I take _______pouches of formula per day

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Bosdet, T., Branov, J., Selvage, C., Yousefi, M., Sirrs, S. (2014). Diet History Is a Reliable Predictor of Suboptimal Docosahexaenoic Acid Levels in Adult Patients with Phenylketonuria. In: Zschocke, J., Baumgartner, M., Morava, E., Patterson, M., Rahman, S., Peters, V. (eds) JIMD Reports, Volume 21. JIMD Reports, vol 21. Springer, Berlin, Heidelberg. https://doi.org/10.1007/8904_2014_399

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  • DOI: https://doi.org/10.1007/8904_2014_399

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-47171-5

  • Online ISBN: 978-3-662-47172-2

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