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      Overweight and obesity in PKU: The results from 8 centres in Europe and Turkey

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          Abstract

          Introduction

          In PKU there is little data comparing the prevalence of overweight and obesity in different countries. The aim of this cross sectional study was to evaluate prevalence data from different PKU treatment centres in Europe and Turkey.

          Subjects and methods

          In children, body mass index (BMI) and z scores and in adults BMI were calculated in 947 patients (783 children aged < 19 years; 164 adults aged ≥ 19 years) with PKU from centres in Europe and Turkey (Ankara, Birmingham, Brussels, Copenhagen, Groningen, Madrid, Munich and Porto).

          Results

          In adults with PKU, 83% of centres (n = 5/6) had less overweight than the general populations but 83% (n = 5/6) had a higher rate of female obesity. In childhood, all centres reported obesity rates within or similar to local population ranges in boys but in 57% (n = 4/7) of centres a higher rate of obesity in girls. The percentage of overweight and obesity increased with age.

          Discussion

          In PKU, it is clear from a number of treatment centres that women and girls with PKU appear particularly vulnerable to excess weight gain and it is important that female weight gain is closely monitored and individual strategies introduced to prevent excess weight gain. Overall, in PKU there is a need to understand better the food patterns and activity levels of patients.

          Highlights

          • This is the first multicentre study in PKU across Europe/Turkey reporting prevalence of overweight and obesity in 947 patients.

          • In adult centres, 83% had less overweight/obesity then the general populations.

          • In adult centres, 83% had a higher rate of female obesity.

          • In children, all centres reported obesity rates within or similar to local population ranges in boys

          • In children, 57% of centres reported a higher rate of obesity in girls.

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          Most cited references7

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          Global, regional, and national prevalence of overweight and obesity in children and adults during 1980-2013: a systematic analysis for the Global Burden of Disease Study 2013.

          In 2010, overweight and obesity were estimated to cause 3·4 million deaths, 3·9% of years of life lost, and 3·8% of disability-adjusted life-years (DALYs) worldwide. The rise in obesity has led to widespread calls for regular monitoring of changes in overweight and obesity prevalence in all populations. Comparable, up-to-date information about levels and trends is essential to quantify population health effects and to prompt decision makers to prioritise action. We estimate the global, regional, and national prevalence of overweight and obesity in children and adults during 1980-2013. We systematically identified surveys, reports, and published studies (n=1769) that included data for height and weight, both through physical measurements and self-reports. We used mixed effects linear regression to correct for bias in self-reports. We obtained data for prevalence of obesity and overweight by age, sex, country, and year (n=19,244) with a spatiotemporal Gaussian process regression model to estimate prevalence with 95% uncertainty intervals (UIs). Worldwide, the proportion of adults with a body-mass index (BMI) of 25 kg/m(2) or greater increased between 1980 and 2013 from 28·8% (95% UI 28·4-29·3) to 36·9% (36·3-37·4) in men, and from 29·8% (29·3-30·2) to 38·0% (37·5-38·5) in women. Prevalence has increased substantially in children and adolescents in developed countries; 23·8% (22·9-24·7) of boys and 22·6% (21·7-23·6) of girls were overweight or obese in 2013. The prevalence of overweight and obesity has also increased in children and adolescents in developing countries, from 8·1% (7·7-8·6) to 12·9% (12·3-13·5) in 2013 for boys and from 8·4% (8·1-8·8) to 13·4% (13·0-13·9) in girls. In adults, estimated prevalence of obesity exceeded 50% in men in Tonga and in women in Kuwait, Kiribati, Federated States of Micronesia, Libya, Qatar, Tonga, and Samoa. Since 2006, the increase in adult obesity in developed countries has slowed down. Because of the established health risks and substantial increases in prevalence, obesity has become a major global health challenge. Not only is obesity increasing, but no national success stories have been reported in the past 33 years. Urgent global action and leadership is needed to help countries to more effectively intervene. Bill & Melinda Gates Foundation. Copyright © 2014 Elsevier Ltd. All rights reserved.
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            Blood phenylalanine control in phenylketonuria: a survey of 10 European centres.

            Only limited data are available on the blood phenylalanine (Phe) concentrations achieved in European patients with phenylketonuria (PKU) on a low-Phe diet. A survey was conducted to compare blood Phe control achieved in diet-treated patients with PKU of different age groups in 10 European centres. Centres experienced in the management of PKU from Belgium, Denmark, Germany, Italy, The Netherlands, Norway, Poland, Spain, Turkey and the United Kingdom provided retrospective audit data of all patients with PKU treated by diet over a 1-year period. Standard questions were used to collect median data on blood Phe concentrations, percentage of blood Phe concentrations below upper target reference ranges and frequency of blood Phe sampling. Data from 1921 patients on dietary management were included. Blood Phe concentrations were well controlled and comparable across centres in the early years of life. The percentages of blood Phe concentrations meeting each centre's local and national target ranges were 88% in children aged up to 1 year, 74% for 1-10 years, 89% for 11-16 years and 65% for adults (>16 years). The frequency of home blood sampling, compared with local and national recommendations for monitoring Phe concentrations, appeared to decline with age (from approximately 100% in infancy to 83% in teenagers and 55% in adults). Although blood Phe control generally deteriorated with age, some improvement was observed in adolescent years across the 10 European centres. The blood Phe control achieved seemed comparable in many of the European centres irrespective of different dietary treatments or national policies.
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              Dietary treatment in phenylketonuria does not lead to increased risk of obesity or metabolic syndrome.

              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. To investigate the prevalence of overweight and obesity, and its consequences in terms of body composition and MetSyn in early treated patients with PKU compared to controls. A sample of 89 patients with PKU (3-30 y; 14.4±6.6 y) and 79 controls (3-47 y; 16.3±7.9 y) were studied. In the fasted state, anthropometric, body composition, blood pressure and analytical parameters [amino acids, glucose, insulin, total and HDL-cholesterol (HDL-c), triglycerides (TG), high sensitivity c-reactive protein and uric acid] were performed. Data on dietary intake was collected. BMI was classified using WHO criteria, while the definition from International Diabetes Federation (IDF) was used for MetSyn. Prevalence of overweight and obesity (32.6% vs. 24.1%; p=0.293), body fat percentage (22% vs. 23.1%, p=0.581) and central obesity (36.9% vs. 36.4%, p=0.999) were comparable to controls. Patients revealed a higher TG/HDL-c (p<0.001). The prevalence of MetSyn was 1.5% and 6.1% in patients and controls, respectively. Patients and not controls with central obesity revealed a further significant increase in TG/HDL-c compared with those without central obesity (p=0.023). Patients and controls were similar in terms of overweight and obesity, body composition and MetSyn. However, the dyslipidemia in patients with PKU in relation to overweight and obesity may help us trying to understand the course and the etiology of MetSyn not only in PKU but also in the general population. Copyright © 2012 Elsevier Inc. All rights reserved.
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                Author and article information

                Contributors
                Journal
                Mol Genet Metab Rep
                Mol Genet Metab Rep
                Molecular Genetics and Metabolism Reports
                Elsevier
                2214-4269
                16 November 2014
                2014
                16 November 2014
                : 1
                : 483-486
                Affiliations
                [a ]Department of Nutrition and Dietetics, Hacettepe University, Ankara, Turkey
                [b ]Center for PKU, The Kennedy Centre, Clinical Genetic Clinic under University Hospital, Glostrup, Denmark
                [c ]Unidad de Enfermedades Metabólicas, Servicio de Pediatría, Hospital Ramon y Cajal, Madrid, Spain
                [d ]Department of Metabolism and Nutrition, Dr. von Hauner Children's Hospital, University of Munich, Munich, Germany
                [e ]San Paolo Hospital University of Milan, Milan, Italy
                [f ]Nutrition and Metabolism Unit, Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium
                [g ]Centro de Genética Médica Doutor Jacinto de Magalhães, CHP EPE, Porto, Portugal
                [h ]Faculdade de Ciências da Saúde, Universidade Fernando Pessoa, Portugal
                [i ]Center for Health Technology and Services Research (CINTESIS), Portugal
                [j ]Unit for Multidisciplinary Research in Biomedicine, Abel Salazar Institute of Biomedical Sciences, University of Porto-UMIB/ICBAS/UP, Porto, Portugal
                [k ]Section of Metabolic Diseases, University of Groningen, University Medical Center Groningen, Groningen, Netherlands
                [l ]The children's Hospital, Birmingham, United Kingdom
                Author notes
                [* ]Corresponding author at: The Children's Hospital, Birmingham, West Midlands, B4 6NH, United Kingdom. anita.macdonald@ 123456bch.nhs.uk
                Article
                S2214-4269(14)00073-1
                10.1016/j.ymgmr.2014.11.003
                5121346
                27896128
                71b6bafb-6813-43a6-9c2c-1b69f5b05b54
                © 2014 Published by Elsevier Inc.

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).

                History
                : 8 November 2014
                : 8 November 2014
                Categories
                Short Communication

                phenylketonuria,obesity,overweight
                phenylketonuria, obesity, overweight

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