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2018 | 65 | 2 | 303-308
Article title

Deficiency of long-chain polyunsaturated fatty acids in phenylketonuria: a cross-sectional study

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EN
Abstracts
EN
The etiology of altered blood fatty acid (FA) profile in phenylketonuria (PKU) is understood only partially. We aimed to determine whether FAs deficiency is dependent on the diet or metabolic disturbances. The study comprised 40 PKU patients (20 female, 20 male; aged 11 to 35 years; 12 children and 28 adults) and 40 healthy subjects (HS; 20 female, 20 male, aged 18 to 33 years). We assessed the profile of FAs (gas chromatography/mass spectrometry) and analyzed the 72-hour dietary recalls. The amount of C14:0, C16:0 and C16:1n-7, C18:1n-9 did not differ between the analyzed groups. The percentage of C18:0 was higher, while C20:3n-9, C18:2n-6, C20:2n-6, C20:4n-6, C22:4n-6, C22:5n-6 and C22:6n-3 was lower in PKU than in HS. However, C18:3n-6, C18:3n-3 and n-6/n-3 ratio were higher in PKU patients. The C20:4n-6/C20:3n-6 ratio (reaction catalyzed by Δ5-desaturase), the C22:5n-6/C22:4n-6 and the C22:6n-3/C22:5n-3 ratio (both reactions catalyzed by Δ6 desaturase) were significantly lower in PKU patients. Therefore, the deficiency of long-chain polyunsaturated fatty acids in PKU patients may result not only from inadequate supply but also from metabolic disturbances.
Publisher

Year
Volume
65
Issue
2
Pages
303-308
Physical description
Dates
published
2018
received
2018-02-02
revised
2018-05-04
accepted
2018-05-07
(unknown)
2018-06-18
Contributors
  • Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Poznań, Poland
  • Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Poznań, Poland
  • Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Poznań, Poland
  • Department of Organization and Management in Health Care, Poznan University of Medical Sciences, Poznań, Poland
  • Voivodeship Hospital, Wrocław, Poland
  • Department of Pediatric Gastroenterology and Metabolic Diseases, Poznan University of Medical Sciences, Poznań, Poland
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Document Type
Publication order reference
Identifiers
YADDA identifier
bwmeta1.element.bwnjournal-article-abpv65p303kz
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