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Accumulating evidence suggests that hyperphenylalaninemia in phenylketonuria (PKU) can cause neuropsychological and psychosocial problems in diet-off adult patients, and that such symptoms improve after resumption of phenylalanine-restricted diet, indicating the need for lifetime low-phenylalanine diet. While limiting protein intake, dietary therapy should provide adequate daily intake of energy, carbohydrates, fat, vitamins, and microelements. We evaluated nutrient balance in 14 patients with classical PKU aged 4–38 years. Approximately 80–85% of the recommended dietary allowance (RDA) of protein in Japanese was supplied through phenylalanine-free (Phe-free) milk and Phe-free amino acid substitutes. Nutritional evaluation showed that the calorie and protein intakes were equivalent to the RDA. Phenylalanine intake was 9.8 ± 2.2 mg/kg of body weight/day, which maintained normal blood phenylalanine concentration by the 80% Phe-free protein rule. The protein, fat, and carbohydrate ratio was 9.5:23.9:66.6% with relative carbohydrate excess. Phe-free milk and amino acid substitutes provided 33.7% of carbohydrate, 82.1% of protein, and 66.7% of fat intake in all. Selenium and biotin intakes were 25.0% and 18.1% of the RDA and adequate intake (AI) for Japanese, respectively; both were not included in Phe-free milk. PKU patients showed low serum selenium, low urinary biotin, and high urinary 3-hydroxyisovaleric acid in this study. The intakes of magnesium, zinc, and iodine were low (71.5%, 79.5%, and 71.0% of the RDA, respectively) and that of phosphorus was 79.7% of the AI, although they were supplemented in Phe-free milk. PKU patients depend on Phe-free milk and substitutes for daily requirement of microelements and vitamins as well as protein and fat. Development of low-protein food makes it possible to achieve the aimed phenylalanine blood level, but this lowers the intake of microelements and vitamins from natural foods. The dietary habits vary continuously with age and environment in PKU patients. We recommend the addition of selenium and biotin to Phe-free milk in Japan and the need to review the composition of microelements and vitamins in A-1 and MP-11 preparations., Highlights • 80- 85% of the recommended amount of protein should be supplied from Phe-free amino acid substitutes for good control. • Nutritional evaluation in PKU patients is necessary for dietary control and for checking the contents of medical foods. • Selenium, and biotin in Phe-free milk in Japan is insufficient and should be supplied immediately. • Microelement from early children to adults is necessary more than in infants due to low protein diet.

Aims/Introduction: The daily basal insulin doses/body weight and the daily basal insulin doses/total daily insulin doses of Japanese type 1 diabetes mellitus patients are less than those of Western type 1 diabetes mellitus patients. It is known that Western meals are richer in fat than Japanese meals. We speculated that fat intake might be associated with basal insulin dose in type 1 diabetes mellitus patients. Materials and Methods: Forty-one outpatients with type 1 diabetes mellitus (20 males, 21 females, mean age 15.9) were enrolled. Variables investigated included: gender, SDS-BMI, HbA1c, duration of diabetes, therapy (MDI or CSII), insulin doses and meal contents. Meal contents were recorded for 3 days using a digital camera. Correlation and multiple regression analyses were performed for all subjects and each age group. Results: The mean daily basal insulin doses/total daily insulin doses was 0.35. In the multiple regression analysis among all subjects, when daily basal insulin doses/body weight was used as a dependent variable, fat energy ratio of the meal was obtained as an entered variable (P = 0.001). This tendency was particularly strong among the patients aged 14 or above (P