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Medientyp:
E-Artikel
Titel:
Factors that increase risk for poor adherence to phenylketonuria treatment in Brazilian patients
Beteiligte:
Teruya, Katia Irie;
Remor, Eduardo;
Schwartz, Ida Vanessa Doederlein
Erschienen:
Wiley, 2021
Erschienen in:
American Journal of Medical Genetics Part A, 185 (2021) 7, Seite 1991-2002
Sprache:
Englisch
DOI:
10.1002/ajmg.a.62195
ISSN:
1552-4825;
1552-4833
Entstehung:
Anmerkungen:
Beschreibung:
AbstractNeurotoxic effects caused by high phenylalanine (Phe) in patients with phenylketonuria (PKU) can be avoided through dietary treatment. However, achieving the recommended Phe levels has been a challenge. This study aimed to investigate factors associated with adherence to PKU treatment among patients followed at a medical genetics public service in southern Brazil. Twenty‐nine patients (early diagnosed, n = 20; late‐diagnosed, n = 9) with classical (n = 16) or mild PKU (n = 13) aged 6–34 years (16.4 ± 7.5) and 16 caregivers were included. Blood Phe levels were recorded, and assessment tools measuring barriers to treatment, IQ, knowledge about disease, treatment, and perceived adherence were collected. Classical PKU patients showed higher current blood Phe levels than mild PKU patients (U = 37.000, p = 0.003). Lifetime and childhood Phe levels were associated with recent metabolic control (τ = 0.76, p = 0.000; τ = 0.70, p = 0.000, respectively). The perception of barriers to treatment was associated with a higher blood Phe level (τ = 0.39, p = 0.003). Tolerance to Phe, metabolic control throughout childhood, and perceived difficulty in living with demands of treatment are important factors of greater vulnerability to poor adherence in PKU patients.