1 Ocak 2023 Dergi makalesi Açık Erişim

Yilmaz, Ozlem; Cochrane, Barbara; Wildgoose, Jo; Pinto, Alex; Evans, Sharon; Daly, Anne; Ashmore, Catherine; MacDonald, Anita

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    <subfield code="a">Cochrane, Barbara</subfield>
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    <subfield code="a">Wildgoose, Jo</subfield>
    <subfield code="u">Bradford Childrens Hosp, Bradford BD5 0NA, England</subfield>
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    <subfield code="a">Pinto, Alex</subfield>
    <subfield code="u">Birmingham Womens &amp; Childrens Hosp, Birmingham B4 6NH, England</subfield>
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    <subfield code="a">Ashmore, Catherine</subfield>
    <subfield code="u">Birmingham Womens &amp; Childrens Hosp, Birmingham B4 6NH, England</subfield>
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    <subfield code="a">MacDonald, Anita</subfield>
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    <subfield code="a">10.1186/s13023-023-02621-9</subfield>
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    <subfield code="a">Phenylalanine free infant formula in the dietary management of phenylketonuria</subfield>
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    <subfield code="a">Yilmaz, Ozlem</subfield>
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    <subfield code="c">2023-01-01</subfield>
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    <subfield code="a">&lt;p&gt;BackgroundPhenylalanine-free infant formula is an essential source of safe protein in a phenylalanine restricted diet, but its efficacy is rarely studied. We report a multicentre, open, longitudinal, prospective intervention study on a phenylalanine-free infant formula (PKU Start: Vitaflo International Ltd.).ResultsThis was a 2-part study: part I (28 days short term evaluation) and part II (12 months extension). Data was collected on infant blood phenylalanine concentrations, dietary intake, growth, and gastrointestinal tolerance. Ten infants (n = 8 males, 80%), with a median age of 14 weeks (range 4-36 weeks) were recruited from 3 treatment centres in the UK. Nine of ten infants completed the 28-day follow-up (one caregiver preferred the usual phenylalanine-free formula and discontinued the study formula after day 14) and 7/9 participated in study part II. The phenylalanine-free infant formula contributed a median of 57% (IQR 50-62%) energy and 53% (IQR 33-66%) of total protein intake from baseline to the end of the part II extension study. During the 12-month follow-up, infants maintained normal growth and satisfactory blood phenylalanine control. Any early gastrointestinal symptoms (constipation, colic, vomiting and poor feeding) improved with time.ConclusionThe study formula was well tolerated, helped maintain good metabolic control, and normal growth in infants with PKU. The long-term efficacy of phenylalanine-free infant formula should continue to be observed and monitored.&lt;/p&gt;</subfield>
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