Nutrition
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Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine (phe) metabolism caused by a deficiency in the enzyme phenylalanine hydroxylase that converts phe into tyrosine. If left untreated, PKU results in increased phe concentrations in the blood and in the brain, which cause severe intellectual disability, epilepsy, and behavioral problems. These disorders can be prevented if a diet low in phe is introduced. ⋯ However, there are infrequent cases of transient hyperphenylalaninemia secondary to delayed maturation of the hydroxylation enzyme system. They are especially significant in premature infants. Although these forms have not been shown to cause sequelae, in view of high levels of phe in the blood, phe consumption must be restricted.
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One of the main objectives in the treatment of childhood obesity is to reduce the percentage of body fat mass without negatively affecting fat-free mass (FFM) and, consequently, longitudinal growth. The aim of this study was to analyze the changes that take place in body compartments in a group of patients with obesity under combined treatment. ⋯ Maintaining a constant weight in the mid- to long term to improve nutritional status would be adequate in children with infantile-juvenile obesity. However, maintaining a steady weight would not be sufficient for those patients diagnosed with severe obesity.
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Urinary hippuric acid (HA) and citrate can represent useful biomarkers of fruit and vegetable (FAV) intake in nephrolithiasis. However, their clinical significance across the life span has been poorly investigated. The aim of this study was to investigate the association between the two biomarkers with FAV intake across different age groups and sexes in a large group of stone formers (SFs). ⋯ Both urinary citrate and HA were positively associated with FAV intake in SFs. However, unlike HA, citrate excretion was significantly influenced by the female sex and by older age.
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Understanding the body composition (BC) of patients with Huntington's disease (HD) could help to delay disease progression and improve treatment efficacy. The aim of this study was to assess BC parameters, including bone mineral density (BMD), and to find new biomarkers that can be early indicators for weight loss in patients with HD. ⋯ Complete BC assessment can be crucial for preventive interventions and prognosis definition in patients with HD. New biomarkers such as BMD, LBM, and truncal fat can be early indicators of weight loss in patients with HD.
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Most studies on dietary patterns have focused on the total daily intake of foods without differentiating intake at specific eating occasions. The aim of this study was to identify meal-specific (breakfast, lunch, and dinner) dietary patterns and examine their contribution to overall dietary patterns, using data from the 2012 National Health and Nutrition Survey, Japan. ⋯ Major meal-specific dietary patterns were identified in the Japanese context, which differentially contributed to major overall dietary patterns.