Everyday Life, Dietary Practices, and Health Conditions of Adult PKU Patients: A Multicenter, Cross-Sectional Study.


Journal

Annals of nutrition & metabolism
ISSN: 1421-9697
Titre abrégé: Ann Nutr Metab
Pays: Switzerland
ID NLM: 8105511

Informations de publication

Date de publication:
2020
Historique:
received: 02 04 2020
accepted: 16 07 2020
pubmed: 1 10 2020
medline: 17 8 2021
entrez: 30 9 2020
Statut: ppublish

Résumé

Only few data on dietary management of adult phenylketonuria (PKU) patients are published. This study aimed to assess living situation, dietary practices, and health conditions of early-treated adult PKU patients. A total of 183 early-treated PKU patients ≥18 years from 8 German metabolic centers received access to an online survey, containing 91 questions on sociodemographic data, dietary habits, and health conditions. 144/183 patients (66% females) completed the questionnaire. Compared with German population, the proportion of single-person households was higher (22 vs. 47%), the rate of childbirth was lower (1.34 vs. 0.4%), but educational and professional status did not differ. 82% of the patients adhered to a low-protein diet, 45% consumed modified low-protein food almost daily, and 84% took amino acid mixtures regularly. 48% of the patients never interrupted diet, and 14% stopped diet permanently. 69% of the patients reported to feel better with diet, and 91% considered their quality of life at least as good. The prevalence of depressive symptoms was high (29%) and correlated significantly to phenylalanine blood concentrations (p = 0.046). However, depressive symptoms were only mild in the majority of patients. This group of early-treated adult German PKU patients is socially well integrated, reveals a surprisingly high adherence to diet and amino acid intake, and considers the restrictions of diet to their daily life as low.

Sections du résumé

BACKGROUND
Only few data on dietary management of adult phenylketonuria (PKU) patients are published.
OBJECTIVES
This study aimed to assess living situation, dietary practices, and health conditions of early-treated adult PKU patients.
METHODS
A total of 183 early-treated PKU patients ≥18 years from 8 German metabolic centers received access to an online survey, containing 91 questions on sociodemographic data, dietary habits, and health conditions.
RESULTS
144/183 patients (66% females) completed the questionnaire. Compared with German population, the proportion of single-person households was higher (22 vs. 47%), the rate of childbirth was lower (1.34 vs. 0.4%), but educational and professional status did not differ. 82% of the patients adhered to a low-protein diet, 45% consumed modified low-protein food almost daily, and 84% took amino acid mixtures regularly. 48% of the patients never interrupted diet, and 14% stopped diet permanently. 69% of the patients reported to feel better with diet, and 91% considered their quality of life at least as good. The prevalence of depressive symptoms was high (29%) and correlated significantly to phenylalanine blood concentrations (p = 0.046). However, depressive symptoms were only mild in the majority of patients.
CONCLUSION
This group of early-treated adult German PKU patients is socially well integrated, reveals a surprisingly high adherence to diet and amino acid intake, and considers the restrictions of diet to their daily life as low.

Identifiants

pubmed: 32998147
pii: 000510260
doi: 10.1159/000510260
doi:

Substances chimiques

Amino Acids 0
Phenylalanine 47E5O17Y3R

Types de publication

Journal Article Multicenter Study Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

251-258

Informations de copyright

© 2020 S. Karger AG, Basel.

Auteurs

Annemarie Klimek (A)

Villa Metabolica, Department of Pediatric and Adolescent Medicine, University Medical Center Mainz, Mainz, Germany.

Christoph Baerwald (C)

Rheumatology Unit, Department of Internal Medicine, Neurology and Dermatology, University Medical Center Leipzig, Leipzig, Germany.

Martin Schwarz (M)

Practice Cooperation and Training Practice Kaarst, Kaarst, Germany.

Frank Rutsch (F)

Department of Pediatrics, University Medical Center Münster, Münster, Germany.

Klaus G Parhofer (KG)

Medical Department IV - Grosshadern, University Munich, Munich, Germany.

Ursula Plöckinger (U)

Interdisciplinary Center of Metabolism: Endocrinology, Diabetes and Metabolism, Charité University Medicine Berlin, Berlin, Germany.

Margret Heddrich-Ellerbrok (M)

Endokrinologikum Hamburg, Hamburg, Germany.

Stephan Vom Dahl (S)

Department for Gastroenterology, Hepatology and Infectious Diseases, University Medical Center Düsseldorf, Düsseldorf, Germany.

Klaus Schöne (K)

Institute for Teachers' Health, University Medical Center Mainz, Mainz, Germany.

Markus Ott (M)

Nutricia GmbH, Metabolics Expert Centre DACH, Frankfurt, Germany.

Frauke Lang (F)

Villa Metabolica, Department of Pediatric and Adolescent Medicine, University Medical Center Mainz, Mainz, Germany.

Julia B Hennermann (JB)

Villa Metabolica, Department of Pediatric and Adolescent Medicine, University Medical Center Mainz, Mainz, Germany, julia.hennermann@unimedizin-mainz.de.

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