DIFFICULTIES RETURNING TO DIET THERAPY IN PATIENTS WITH PHENYLKETONURIA
نویسندگان
چکیده
منابع مشابه
Efect of a controlled diet program on behavioral-emotional disorders in patients with phenylketonuria
Introduction: Phenylketonuria is a disorder due to deficiency of the phenylalanine hydroxylase enzyme. Delay or untreatement would cause impacts on motor-cognitive and individual-social skills. This study aimed to investigate the effect of a controlled diet program on behavioral-emotional disorders in patients with phenylketonuria. Materials and Methods: In a quasi-experimental study, 60 patien...
متن کاملAdherence to tetrahydrobiopterin therapy in patients with phenylketonuria.
Phenylketonuria (PKU) is an inborn error in phenylalanine metabolism due to deficiency of the enzyme, phenylalanine hydroxylase (PAH). Treatment includes restriction of dietary phenylalanine, and in some individuals, supplementation with the PAH cofactor, tetrahydrobiopterin (sapropterin dihydrochloride). A survey was conducted among patients with PKU who had been prescribed sapropterin to asse...
متن کاملPlasma phenylalanine in patients with phenylketonuria self-managing their diet.
D ietary adherence in phenylketonuria (PKU)—measured by plasma phenylalanine (Phe) concentrations—is a major issue. 2 In the so-called professionally steered situation, it was our practice to take all blood samples for measurement of Phe during patients’ hospital visits. Phe concentrations were interpreted by the paediatrician, and the dietician phoned patients/parents with dietary advice. Self...
متن کاملAnemia in Patients with Phenylketonuria in Yazd
Abstract Background Phenyl ketonuria (PKU) is a congenital metabolic disease. Irreversible brain damage is the result of phenyl alanin accumulation, so its amount should be restricted in patients diet. In their diet, trace elements such as hem Iron, folic acid, vitamin B12 and, etc are also limited, and could represent anemia. In this study, the frequency of anemia in PKU patients in Yazd wa...
متن کاملA long-term study of bone mineral density in patients with phenylketonuria under diet therapy
INTRODUCTION Dietary control of classic phenylketonuria (PKU) needs restriction of natural proteins; adequate protein intake is achieved by adding low phenylalanine (phe) formulae. The adequacy of this diet for normal bone mineralization had not been sufficiently evaluated. Our aim was to evaluate and follow up bone mineral density (BMD) in children and adolescents with PKU within a 2-year time...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1984
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-198404001-00032