How strict is galactose restriction in adults with galactosaemia? International practice.

Dietary management of 418 adult patients with galactosaemia (from 39 centres/12 countries) was compared. All centres advised lactose restriction, 6 restricted galactose from galactosides ± fruits and vegetables and 12 offal. 38% (n=15) relaxed diet by: 1) allowing traces of lactose in manufactured foods (n=13) or 2) giving fruits, vegetables and galactosides (n=2). Only 15% (n=6) calculated dietary galactose. 32% of patients were lost to dietetic follow-up. In adult galactosaemia, there is limited diet relaxation.

Body mass index in adult patients with diet-treated phenylketonuria.

BACKGROUND: There is an increasing number of adults with phenylketonuria (PKU) on a low phenylalanine diet. In the general population, an increasing body mass index (BMI) in the UK is a major problem with associated co-morbidities. The present study aimed to identify whether patients with diet-treated PKU have obesity rates comparable to those in the general population. METHODS: Two hundred and thirty-six PKU subjects (49% male, 51% female), aged >16 years, who were diagnosed by newborn screening and were receiving a low phenylalanine diet, were identified from seven metabolic centres in the UK. Retrospective data were collated on age, sex, BMI and mean phenylalanine concentration over the previous 12 months. RESULTS: Mean (SD) phenylalanine concentration for all 236 subjects was 789 (311) µm; mean (SD) BMI was 26 (5.4) kg m(-2) [males 25 (4.3) kg m(-2) , females 27 (6.2) kg m(-2) ]; mean (SD) age was 26 (7) years; and 55% had a BMI > 25 kg m(-2) . The percentage of subjects with a BMI >25 kg m(-2) and >30 kg m(-2) , as well as increasing obesity with age, was similar to the UK population. A correlation was observed between increasing BMI and a higher phenylalanine concentration (r = 0.243, P = 0.001). CONCLUSIONS: The number of overweight and obese patients with diet-treated PKU in the UK is a concern. This could lead to other obesity-related complications increasing the complexity of diet and the cost of their care. There is a need to educate patients with respect to adopting a healthy, low phenylalanine diet and lifestyle to prevent further rises in BMI.

Early childhood programs in other nations: goals and outcomes.

United States interest in the potential early childhood programs have for improving outcomes for children is shared by policymakers and researchers in many other nations. Throughout the world, enrollments in preschool and child care programs are rising. This article reviews international research documenting how participation in early childhood programs influenced children's later development and success in school. Studies conducted in 13 nations (Australia, Canada, Colombia, France, Germany, India, Ireland, Japan, Singapore, South Korea, Sweden, Turkey, and the United Kingdom) are included, along with key features of each nation's provision of early childhood programs. The article summarizes conclusions that are supported by research in various countries, indicating that participation in preschool promotes cognitive development and school success, although the specific type of program attended matters little. Preschool experience helps low-income children narrow, but not close, the achievement gap separating them from more advantaged children. International evidence also suggests that maternal employment and reliance on child care do not harm children and may yield benefits if the child care is of good quality. The author draws insights from the experience of other nations concerning such issues as defining quality, the effectiveness of early childhood programs in redressing social and economic inequities, and understanding how research can influence policy.