Influence of knowledge of the disease on metabolic control in phenylketonuria

article





Bekhof, J.

Spronsen, F.J. van

Crone, M.R.

Rijn, M. van

Oudshoorn, C.G.M.

Verkerk, P.H.
In patients with phenylketonuria (PKU) knowledge of the disease and its treatment is not a major independent predictor for dietary compliance. PKU is an inborn error of amino-acid metabolism caused by a deficiency of phenylalanine hydroxylase (PAH), resulting in high plasma phenylalanine (Phe) concentrations and consequently severe mental retardation. Treatment comprises a life-long diet and PKU patients maintaining good metabolic control develop normally [3]. Chemicals/CAS: phenylalanine, 3617-44-5, 63-91-2
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adolescentchilddietdisease severityfeeding behaviormajor clinical studymetabolic regulationpatient attitudepatient compliancephenylketonuriapriority journalAdultHealth Knowledge, Attitudes, PracticeHumansPatient CompliancePatient EducationPhenylketonurias
TNO Identifier
237131
Repository link
https://resolver.tno.nl/uuid:f54978bd-9821-455d-8c1e-b5fbe22595f6
ISSN
03406199
Source
European Journal of Pediatrics, 162(6), pp. 440-442.
Pages
440-442
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