Dietary patterns, cost and compliance with low-protein diet of phenylketonuria and other inherited metabolic diseases
Language English Country England, Great Britain Media print-electronic
Document type Journal Article, Observational Study, Research Support, Non-U.S. Gov't
PubMed
28656971
DOI
10.1038/ejcn.2017.102
PII: ejcn2017102
Knihovny.cz E-resources
- MeSH
- Patient Compliance * ethnology MeSH
- Child MeSH
- Adult MeSH
- Phenylketonurias diet therapy economics ethnology physiopathology MeSH
- Child Nutritional Physiological Phenomena * ethnology MeSH
- Adolescent Nutritional Physiological Phenomena * ethnology MeSH
- Humans MeSH
- Intellectual Disability economics ethnology etiology prevention & control MeSH
- Adolescent MeSH
- Young Adult MeSH
- Costs and Cost Analysis MeSH
- Diet, Protein-Restricted * economics ethnology MeSH
- Cost of Illness * MeSH
- Caregivers MeSH
- Child, Preschool MeSH
- Metabolism, Inborn Errors diet therapy economics ethnology physiopathology MeSH
- Rare Diseases diet therapy economics ethnology physiopathology MeSH
- Food Supply economics MeSH
- Self Report MeSH
- Check Tag
- Child MeSH
- Adult MeSH
- Humans MeSH
- Adolescent MeSH
- Young Adult MeSH
- Male MeSH
- Child, Preschool MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Observational Study MeSH
- Research Support, Non-U.S. Gov't MeSH
- Geographicals
- Czech Republic 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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