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Blood phenylalanine (Phe) is used as the primary marker to evaluate metabolic control. Our study aimed to describe the metabolic control of patients with phenylketonuria (PKU) comparing three different treatment recommendations (European guidelines/US guidelines/Portuguese consensus). This was a retrospective, observational, single centre study in patients with PKU collecting data on blood Phe levels from 2017. Nutritional intake data and sapropterin (BH4) prescription were collected at the last appointment of 2017. The final sample studied included 87 patients (48% females) [13 hyperphenylalaninemia, 47 mild PKU, 27 classical PKU] with a median age of 18 y (range: 1–36 y). The median number of blood Phe measurements for patients was 21 (range: 6–89). In patients aged &lt, 12 y, the median blood Phe level was 300 μmol/L (range 168–480) and 474 μmol/L (range 156–1194) for patients ≥ 12 y. Overall, a median of 83% of blood Phe levels were within the European PKU guidelines target range. In patients aged ≥ 12 years, there was a higher median % of blood Phe levels within the European PKU guidelines target range (≥12 y: 84% vs. &lt, 12 y: 56%). In children &lt, 12 y with classical PKU (n = 2), only 34% of blood Phe levels were within target range for all 3 guidelines and 49% with mild PKU (n = 11). Girls had better control than boys (89% vs. 66% median Phe levels within European Guidelines). Although it is clear that 50% or more patients were unable to achieve acceptable metabolic control on current treatment options, a globally agreed upper Phe target associated with optimal outcomes for age groups is necessary. More studies need to examine how clinics with dissimilar resources, different therapeutic Phe targets and frequency of monitoring relate to metabolic control.

Adolescence is a time in life when lifestyle behaviours are acquired. One indicator of poor diet quality is the intake of foods and beverages with a relatively low nutritional value. Using the Australian classification of such foods, termed “discretionary”, we classified the intakes of Swedish adolescents who participated in the Riksmaten Adolescent 2016–17 national dietary survey. From selected schools, 3099 adolescents in age groups 11–12, 14–15 and 17–18 years provided two 24-h recalls. Intakes and healthy dietary scores were calculated. Plasma ferritin, folate and 25(OH)D were available for a third. Almost 40% of total energy came from discretionary foods/beverages. Adolescents with higher intakes were more likely to be female, older, from a low socioeconomic position-household and born in Sweden. Most discretionary foods/beverages were consumed on weekend days and during in-between meals, outside of the home and at school. Percent energy from discretionary intake was associated with healthy dietary scores but not nutritional status. A substantial amount of energy was obtained from discretionary foods/beverages, and we found that consumption is pervasive across sociodemographic factors, time and place. Addressing this pattern will require a comprehensive approach to food environments and behaviours to reach all adolescents in an equitable manner.

Objective: To investigate the levels of a deoxynivalenol (DON) biomarker in the urine of subjects living in two China provinces with different geographic locations and dietary patterns, and estimate their dietary DON exposures and health risks. Methods: First morning urine samples were collected on three consecutive days from 599 healthy subjects&mdash, 301 from Henan province and 298 from Sichuan province&mdash, to analyze the total DON concentrations (tDON) after &beta, glucuronidase hydrolysis using a high-performance liquid chromatography tandem mass spectrometry-based method. The consumption of cereal foods in the previous 24 h before each urine collection was recorded using a duplicate diet method. DON exposure levels were estimated based on the urinary tDON concentrations. Results: Total DON were detected in 100% and 92% of the urine samples from Henan and Sichuan, respectively. Mean urinary tDON concentrations were 52.83 ng/mL in Henan subjects and 12.99 ng/mL in Sichuan subjects, respectively. The tDON levels were significantly higher in the urine of Henan subjects than that of the Sichuan subjects (p &lt, 0.001). Urinary tDON levels were significantly different among age groups in both areas (Henan: p &lt, 0.001, Sichuan: p = 0.026) and were highest in adolescents aged 13&ndash, 17 years, followed by children aged 7&ndash, 12 years. Based on the DON biomarker and exposure conversion reported by the European Food Safety Authority (EFSA), the mean estimated dietary intakes of DON were 1.82 &mu, g/kg bw/day in Henan subjects and 0.45 &mu, g/kg bw/day in Sichuan subjects. A total of 56% of Henan subjects and 12% of Sichuan subjects were estimated to exceed the PMTDI of 1 &mu, g/kg bw/day. Consistent with urinary tDON levels, the highest estimated dietary DON intakes were also in children and adolescents aged 7&ndash, 17 years. For all kinds of wheat-based foods except dumplings, the consumptions were significantly higher in Henan than those in Sichuan. The mean consumption of steamed buns was 8.4-fold higher in Henan (70.67 g/d) than that in Sichuan (8.45 g/d). The mean consumption of noodles in Henan (273.91 g/d) was 3.6-fold higher than that in Sichuan (75.87 g/d). Conclusions: The levels of urinary DON biomarker and the estimated dietary DON intakes in Henan province were high and concerning, especially for children and adolescents. The overall exposure level of Sichuan inhabitants was low.