Hulya Gokmen-Ozel

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The usual treatment for phenylketonuria (PKU) is a phenylalanine-restricted diet. Following this diet is challenging, and long-term adherence (and hence metabolic control) is commonly poor. Patients with PKU (usually, but not exclusively, with a relatively mild form of the disorder) who are responsive to treatment with pharmacological doses of(More)
OBJECTIVE Glucose polymer-based emergency feeds (EF), used during illness to prevent metabolic decompensation and encephalopathy in inherited metabolic disorders, should be produced accurately and safely. DESIGN In a randomised, prospective, controlled study, the aim was to investigate if when preparing age-appropriate EF, a pre-measured sachet of glucose(More)
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