living with phenylketonuria in adulthood: the pku attitude study

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ID: 140088
2018
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Abstract
Dietary treatment is the cornerstone of therapy for phenylketonuria (PKU), but adherence to low- phenylalanine diet progressively decreases after adolescence. We designed a survey to characterize the dietary habits of Italian adult PKU patients and to identify psychological factors influencing disease perception and adherence to diet. Participants to the survey (n = 111; response rate 94%) were asked to complete a structured questionnaire. Patients appeared to have an altered perception and awareness of the disease. About 40% of them did not consider PKU a disease and, despite declaring regular monitoring of phenylalanine levels (85%), nearly half of them reported a high plasma value over the last 6 months (>600 μmol/L, 48%) or were unable to specify it (31%). Adherence to PKU diet was unsatisfactory, with increased consumption of natural protein sources and reduced daily use of amino-acid supplements (<4–5 times/day in 82% patients). In addition to the intrinsic characteristics of AA formula (palatability, ease of use), the most important factor influencing their consumption was the increased social pressure associated with their use (55%). Plasma phenylalanine periodical measurements (61%) and examinations at metabolic centers (49%) were considered relevant for compliance to diet. In Italian adult PKU patients dietary management was found to be inadequate, likely due to inappropriate perception and knowledge of the disease, and lack of awareness of the negative impact of poor metabolic control in adult life. Clinicians should consider implementing more intense and tailored educational measures, as well as structured transitional care processes. Keywords: Phenylketonuria (PKU), Diet, Adulthood, Compliance, Amino acid
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cazzorla2018molecularliving Use this key to autocite in the manuscript while using SciMatic Manuscript Manager or Thesis Manager
Authors ;Chiara Cazzorla;Giulia Bensi;Giacomo Biasucci;Vincenzo Leuzzi;Filippo Manti;Antonella Musumeci;Francesco Papadia;Vera Stoppioni;Albina Tummolo;Marcella Vendemiale;Giulia Polo;Alberto Burlina
Journal advances in skin & wound care
Year 2018
DOI
DOI not found
URL
http://www.sciencedirect.com/science/article/pii/S221442691830065X
Keywords
medicine (general)biology (general)

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