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Background: There appears little consensus concerning protein requirements in phenylketonuria (PKU)., Methods: A questionnaire completed by 63 European and Turkish IMD centres from 18 countries collected data on prescribed total protein intake (natural/intact protein and phenylalanine-free protein substitute [PS]) by age, administration frequency and method, monitoring, and type of protein substitute. Data were analysed by European region using descriptive statistics., Results: The amount of total protein (from PS and natural/intact protein) varied according to the European region. Higher median amounts of total protein were prescribed in infants and children in Northern Europe (n=24 centres) (infants <1 year, >2-3g/kg/day; 1-3 years of age, >2-3 g/kg/day; 4-10 years of age, >1.5-2.5 g/kg/day) and Southern Europe (n=10 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, 2 g/kg/day; 4-10 years of age, 1.5-2 g/kg/day), than by Eastern Europe (n=4 centres) (infants <1 year, 2.5 g/kg/day, 1-3 years of age, >2-2.5 g/kg/day; 4-10 years of age, >1.5-2 g/kg/day) and with Western Europe (n=25 centres) giving the least (infants <1 year, >2-2.5 g/kg/day, 1-3 years of age, 1.5-2 g/kg/day; 4-10 years of age, 1-1.5 g/kg/day). Total protein prescription was similar in patients aged >10 years (1-1.5 g/kg/day) and maternal patients (1-1.5 g/kg/day)., Conclusions: The amounts of total protein prescribed varied between European countries and appeared to be influenced by geographical region. In PKU, all gave higher than the recommended 2007 WHO/FAO/UNU safe levels of protein intake for the general population., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background and Aim: Worldwide there is scientific discussion about the dietary management of galactosemia. The dietary management is very different in several countries among Europe, the US and Canada. The main points of discussion are related to the fact that i) despite a strict diet some patients still have poor outcomes; ii) there is lack of scientific knowledge about the role of endogenous production of galactose on disease evolution, with or without diet. The aim of the current work was the creation of a Belgian consensus on dietary guidelines for the management of galactosemia., Methods: A step-wise approach was used to achieve a consensus, including: a workshop, a Delphi round, discussion groups and a round table of different Belgian experts., Results: The consensus is an agreement between strict guidelines (strict limitation of fruits, vegetables and soybean products/French guidelines) and the more liberal guidelines (comparable with a diet free of lactose/guidelines of UK and the Netherlands). The consensus document consists of different modules, including the medical context, the theoretical background of dietary guidelines and the age-specific practical dietary guidelines., Conclusion: A Belgian consensus on the guidelines for the dietary management of classical galactosemia was developed despite the uncertainties of the efficacy and practical application of these guidelines. The final consensus is based on scientific knowledge and practical agreement among experts. In the future, regular revision of the guidelines is recommended and a uniform European guideline is desirable., (Copyright © 2014 European Society for Clinical Nutrition and Metabolism. Published by Elsevier Ltd. All rights reserved.)

Background: There is no published data comparing dietary management of urea cycle disorders (UCD) in different countries., Methods: Cross-sectional data from 41 European Inherited Metabolic Disorder (IMD) centres (17 UK, 6 France, 5 Germany, 4 Belgium, 4 Portugal, 2 Netherlands, 1 Denmark, 1 Italy, 1 Sweden) was collected by questionnaire describing management of patients with UCD on prescribed protein restricted diets., Results: Data for 464 patients: N-acetylglutamate synthase (NAGS) deficiency, n=10; carbamoyl phosphate synthetase (CPS1) deficiency, n=29; ornithine transcarbamoylase (OTC) deficiency, n=214; citrullinaemia, n=108; argininosuccinic aciduria (ASA), n=80; arginase deficiency, n=23 was reported. The majority of patients (70%; n=327) were aged 0-16y and 30% (n=137) >16y. Prescribed median protein intake/kg body weight decreased with age with little variation between disorders. The UK tended to give more total protein than other European countries particularly in infancy. Supplements of essential amino acids (EAA) were prescribed for 38% [n=174] of the patients overall, but were given more commonly in arginase deficiency (74%), CPS (48%) and citrullinaemia (46%). Patients in Germany (64%), Portugal (67%) and Sweden (100%) were the most frequent users of EAA. Only 18% [n=84] of patients were prescribed tube feeds, most commonly for CPS (41%); and 21% [n=97] were prescribed oral energy supplements., Conclusions: Dietary treatment for UCD varies significantly between different conditions, and between and within European IMD centres. Further studies examining the outcome of treatment compared with the type of dietary therapy and nutritional support received are required., (© 2013 Elsevier Inc. All rights reserved.)

Background. Efforts are needed to improve the long-term efficiency of childhood obesity treatment. To adapt strategies, the identification of subgroups of patients with a greater weight loss may be useful. Objective. To analyze the results of a chronic care program for childhood obesity and to determine baseline factors (medical, dietary, and psychosocial) associated with successful weight loss. Subjects and Method. We set up a family-targeted and individually adapted interdisciplinary long-term care program. We reviewed the medical files of 144 children (59 boys and 85 girls; 10.5 ± 3.1 y; mean BMI-z-score: 2.73 ± 0.62) who had ≥2 interdisciplinary visits and ≥1-year treatment. Results. Mean treatment length was 2.2 y (1-6.7 y) with 3 ± 1 visits/year. The duration of treatment did not depend on the initial weight loss, but this was predictive of the weight change over time. Furthermore any additional weight loss was observed with time whatever the initial weight change. High levels of physical activity and daily water intake from baseline conditions were associated with a greater weight loss after 9 months of intervention. In contrast, a high baseline consumption of soft drinks resulted in lower weight loss. Family specific factors such as being a single child or the child's family support were identified as baseline factors which may contribute to better results. Conclusion. Our study suggests that the benefit of a chronic weight control program supports the need for its integration into the current concept of treatment. Better prevention policy and parental support may improve the success of the childhood obesity treatment.