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The Adolescent Adjustment Profile (AAP) in comparisons of patients with obesity, phenylketonuria or neurobehavioural disorders.

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Psychosocial development in children with chronic disease is a key issue in paediatrics. This study investigated whether psychosocial adjustment could be reliably assessed with the 42-item Adolescent Adjustment Profile (AAP) instrument. The study mainly focused on adjustment-to-obesity measurement,

Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome.

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BACKGROUND Little is known about the consequences of the special energy enriched diet used to treat patients with phenylketonuria (PKU) in terms of obesity and metabolic syndrome (MetSyn) development. OBJECTIVE To investigate the prevalence of overweight and obesity, and its consequences in terms of

Overweight in classical phenylketonuria children: A retrospective cohort study.

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This cohort study aimed to determine the frequency of overweight and obesity in classical phenylketonuria children and to identify the possible influence of metabolic control on the BMI of the studied patients.The study group included 63 classical

High prevalence of overweight and obesity in females with phenylketonuria.

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The primary treatment for phenylketonuria (PKU) is a low phenylalanine diet together with an amino acid-based, phenylalanine-free formula. Thus, PKU patients tend to consume a diet enriched in carbohydrates which could predispose to obesity. Studies in the 1980s and 1990s demonstrated that

Carriership of the rs113883650/rs2287120 haplotype of the SLC7A5 ( LAT1) gene increases the risk of obesity in infants with phenylketonuria

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Purpose: Phenylketonuria (PKU) can be effectively treated with the use of a low-phenylalanine diet. However, some patients become overweight despite proper dietary treatment. We hypothesized that this phenomenon could be explained by the

Is overweight an issue in phenylketonuria?

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Dietary treatment may be associated with an increased risk of obesity in phenylketonuria (PKU). The earliest studies describe a tendency for overweight in PKU, but not all recent publications confirm this, although there are an increasing number of studies describing increased obesity in female

Effects of oral contraceptives and obesity on carrier tests for phenylketonuria.

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[Phenylketonuria. Treatment and developmental control].

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We evaluated annually the compliance of dietary restriction, weight, height, head circumference EEG and IQ score on 16 children with phenylketonuria comparing the children diagnosed early with those later. The compliance was good in children treated early and bad in the others. Height and head

[Phenylketonuria diagnosed during the neonatal period and breast feeding].

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BACKGROUND Phenylketonuria (PKU) is due to of a defect in the phenylalanine hydroxylase gene (12q22-24.1) leading to hyperphenylalaninemia. Treatment consists in a low phenylalanine (Phe) diet. OBJECTIVE To evaluate the evolution of early diagnosed PKU children, receiving direct breast feeding, and

Recommendations for protein and energy intakes by patients with phenylketonuria.

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Recent reports describe poor growth in treated children with phenylketonuria (PKU). That poor growth is not a concomitant of the disorder and need not result from therapy is demonstrated by data from the U.S.A. PKU Collaborative Study and from recent data from the U.S.A. In these studies, sufficient

Acute exercise in treated phenylketonuria patients: Physical activity and biochemical response.

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BACKGROUND In phenylketonuria, dietary treatment prevents most of the severe brain disease. However, patients have to follow a diet restricted in several natural components, what may cause decreased bone density and obesity. Exercise is known to improve both mental functioning and bone density also

Metabolic syndrome in children and adolescents with phenylketonuria.

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OBJECTIVE This study aimed to identify markers of metabolic syndrome (MS) in patients with phenylketonuria (PKU). METHODS This was a cross-sectional study consisting of 58 PKU patients (ages of 4-15 years): 29 patients with excess weight, and 29 with normal weight. The biochemical variables assessed

Nutritional and metabolic parameters of children and adolescents with phenylketonuria.

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Considering that phenylalanine-poor diets may be monotonous and compromise the development and nutritional status of children and adolescents with phenylketonuria, the aim of this study was to evaluate the anthropometric and biochemical characteristics of children and adolescents with

Evaluation of a phenylalanine-free product for treatment of phenylketonuria.

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Ten children with classic phenylketonuria (PKU) participated in a controlled study of a phenylalanine-free formula recently released in the United States (PKU-2). Control data were obtained in the clinic while the children were receiving their baseline formula. The children were given the study

Reassessment of phenylalanine tolerance in adults with phenylketonuria is needed as body mass changes.

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Lifelong treatment of phenylketonuria (PKU) includes a phenylalanine (phe) restricted diet that provides sufficient phe for growth and maintenance plus phe-free amino acid formula to meet requirements for protein, energy and micronutrients. Phe tolerance (mg phe/kg body weight/day) is the amount of

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