Dietary patterns, cost and compliance with low-protein diet of phenylketonuria and other inherited metabolic diseases

Result code in IS VaVaI

<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F18%3A10376311" target="_blank" >RIV/00216208:11110/18:10376311 - isvavai.cz</a>

Alternative codes found

RIV/61384399:31120/18:00050753 RIV/00064165:_____/18:10376311

Result on the web

<a href="https://doi.org/10.1038/ejcn.2017.102" target="_blank" >https://doi.org/10.1038/ejcn.2017.102</a>

DOI - Digital Object Identifier

<a href="http://dx.doi.org/10.1038/ejcn.2017.102" target="_blank" >10.1038/ejcn.2017.102</a>

Result language

angličtina

Original language name

Dietary patterns, cost and compliance with low-protein diet of phenylketonuria and other inherited metabolic diseases

Original language description

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 (approximate to 30% of energy intake), pasta (approximate to 18%), basic pastry (approximate to 15%) and sweets (approximate to 10%). The average monthly costs of LPDs were equal to EUR130 (PKU) and EUR129 (other IMD) per patient per month. The compliance with LPD was decreasing with increasing age (P &lt; 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.

Czech name

—

Czech description

—

Type

J_imp - Article in a specialist periodical, which is included in the Web of Science database

CEP classification

—

OECD FORD branch

30308 - Nutrition, Dietetics

Project

—

Continuities

I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace

Publication year

2018

Confidentiality

S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů

Name of the periodical

European Journal of Clinical Nutrition

ISSN

0954-3007

e-ISSN

—

Volume of the periodical

72

Issue of the periodical within the volume

1

Country of publishing house

GB - UNITED KINGDOM

Number of pages

6

Pages from-to

87-92

UT code for WoS article

000419795000011

EID of the result in the Scopus database

2-s2.0-85040321157