Subject(s)
Micronutrients , Phenylketonurias , Bone Density , Bone and Bones , Fatty Acids, Essential , HumansABSTRACT
INTRODUCTION: In phenylketonuria (PKU), a natural protein-restricted dietary treatment prevents severe cognitive impairment. Nutrient deficiencies may occur due to strict diet. This study is aimed at evaluating the dietary intake and blood concentrations of micronutrients and essential fatty acids (FA), bone mineral density (BMD) and fracture history in patients on long-term dietary treatment. METHODS: Sixty early diagnosed Dutch patients (aged 1-39 years) were included in a multi-center cross-sectional study. Their dietary intake, blood concentrations of micronutrients, FA, fracture history and BMD were assessed. RESULTS: Selenium dietary intake and serum concentrations were low in 14 and 46% of patients, respectively. The serum 25-OH vitamin D2 + D3 concentration was low in 14% of patients while 20% of patients had a low vitamin D intake. Zinc serum concentrations were below normal in 14% of patients, despite adequate intake. Folic acid serum concentrations and intake were elevated. Despite safe total protein and fat intake, arginine plasma concentrations and erythrocyte eicosapentaenoic acid were below reference values in 19 and 6% of patients, respectively. Low BMD (Z-score <-2) was slightly more prevalent in patients, but the lifetime fracture prevalence was comparable to the general population. CONCLUSIONS: Dutch patients with PKU on long-term dietary treatment have a near normal nutrient status. Supplementation of micronutrients of which deficiency may be deleterious (e.g., vitamin D and selenium) should be considered. BMD warrants further investigation.
Subject(s)
Bone and Bones/drug effects , Fatty Acids, Essential/administration & dosage , Micronutrients/blood , Phenylketonurias/blood , Phenylketonurias/drug therapy , Adolescent , Adult , Bone Density/drug effects , Bone and Bones/physiology , Child , Child, Preschool , Cross-Sectional Studies , Dietary Proteins/administration & dosage , Dietary Proteins/blood , Erythrocytes/chemistry , Fatty Acids, Essential/blood , Female , Humans , Infant , Male , Micronutrients/administration & dosage , Nutrition Assessment , Nutritional Status , Phenylalanine/administration & dosage , Phenylalanine/blood , Selenium/administration & dosage , Selenium/blood , Vitamin D/administration & dosage , Vitamin D/blood , Young AdultABSTRACT
Accumulating evidence shows that children are not simply little adults, and adapting adult evidence on medical care to children can result in ineffective or even unsafe medical care. The growing number of trials in children reflects this imperative to create a child-specific evidence base. Motivated by the increased focus on clinical research in children and the stark deficiencies in knowledge about the best ways to deal with methodological and practical challenges of research in children, the STaR Child Health initiative was created. It aims to enhance the design, conduct, and reporting of trials in children. The initiative brings together an international group of leading methodologists, clinicians, regulators, funders, and decision-makers to systematically identify what is known, create a research agenda where gaps exist and translate information into practical guidance for end-users. It is critical that funders, researchers, and editors work together for improved conduct and reporting of trials in the next 5-10 years to ensure that the investment in this area will produce the most reliable and relevant results. The first StaR Child Health summit will take place in Amsterdam on the 26th and 27th of October.
