Dietary patterns, cost and compliance with low-protein diet of phenylketonuria and other inherited metabolic diseases
Jazyk angličtina Země Anglie, Velká Británie Médium print-electronic
Typ dokumentu časopisecké články, pozorovací studie, práce podpořená grantem
PubMed
28656971
DOI
10.1038/ejcn.2017.102
PII: ejcn2017102
Knihovny.cz E-zdroje
- MeSH
- adherence pacienta * etnologie MeSH
- dítě MeSH
- dospělí MeSH
- fenylketonurie dietoterapie ekonomika etnologie patofyziologie MeSH
- fyziologie výživy dětí * etnologie MeSH
- fyziologie výživy mladistvých * etnologie MeSH
- lidé MeSH
- mentální retardace ekonomika etnologie etiologie prevence a kontrola MeSH
- mladiství MeSH
- mladý dospělý MeSH
- náklady a analýza nákladů MeSH
- nízkoproteinová dieta * ekonomika etnologie MeSH
- osobní újma zaviněná nemocí * MeSH
- osoby pečující o pacienty MeSH
- předškolní dítě MeSH
- vrozené poruchy metabolismu dietoterapie ekonomika etnologie patofyziologie MeSH
- vzácné nemoci dietoterapie ekonomika etnologie patofyziologie MeSH
- zásobování potravinami ekonomika MeSH
- zpráva o sobě MeSH
- Check Tag
- dítě MeSH
- dospělí MeSH
- lidé MeSH
- mladiství MeSH
- mladý dospělý MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- pozorovací studie MeSH
- práce podpořená grantem MeSH
- Geografické názvy
- Česká republika MeSH
BACKGROUND/OBJECTIVES: Phenylketonuria (PKU) and several other inherited metabolic diseases (IMD) require a lifelong low-protein diet (LPD), otherwise they lead to many health complications. LPDs, however, carry a significant economic burden for patients and their families. The objective of this study was to explore the costs of low-protein foods (LPFs) necessary for LPD as well as dietary patterns and compliance towards an LPD. SUBJECTS/METHODS: A detailed questionnaire was created in cooperation with National Association of PKU and other IMD (NSPKU), and consequently sent to all NSPKU members treated with an LPD (n=303). A total of 184 respondents from the Czech Republic were included in the study (174 had PKU, 10 had other IMD). RESULTS: The average daily consumption of LPF was equal to 411.7 g (PKU) and 345.6 g (other IMD), which corresponds to energy value of 5558 kJ and 4438 kJ, respectively, per patient per day. Patients mostly consumed low-protein flour (≈30% of energy intake), pasta (≈18%), basic pastry (≈15%) and sweets (≈10%). The average monthly costs of LPDs were equal to [euro ]130 (PKU) and [euro ]129 (other IMD) per patient per month. The compliance with LPD was decreasing with increasing age (P<0.0001). CONCLUSIONS: This is the largest study examining costs and dietary patterns of LPDs in patients with PKU and the first study of this kind in other IMD patients requiring an LPD. The study clearly showed that an LPD carries a very high economic burden for families, which may lead to less LPD compliance and potential severe health consequences.
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