页 1 从 46 结果
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,
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
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 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
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
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
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
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
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
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
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
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 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
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