Maureen Evans

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AIM To compare the measurement of total body water (TBW) and fat-free mass (FFM) using the criterion method of deuterium dilution space (2H2O) with bioelectrical impedance analysis (BIA) using a portable QuadScan 4000, Bodystat® in children and adolescents with phenylketonuria (PKU). METHODS Sixteen patients with PKU, median age is 12.5 (range 5-20.6)(More)
AIM Phenylketonuria (PKU) is an inborn error of protein metabolism that results from perturbation in phenylalanine hydroxylase activity leading to elevated blood levels of phenylalanine (phe). We aimed to explore the relationships between dietary patterns (total-protein, natural-protein, amino-acid formula), and the ratio of protein to energy intake with(More)
Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is an inherited metabolic disorder of fatty acid oxidation. Treatment practices of the disorder have changed over the past 10-15years since this disorder was included in newborn screening programs and patients were diagnosed pre-symptomatically. A genotype-phenotype correlation has been suggested but(More)
OBJECTIVES To examine relationships between dietary intake, growth and body composition patterns in patients with inborn errors of intermediary protein metabolism and to determine a safe protein:energy ratio (P:E ratio) associated with optimal growth outcomes. STUDY DESIGN Retrospective longitudinal data of growth and dietary intake in patients (n = 75)(More)
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