The nutritional and metabolic characteristics of adult phenylketonuria (PKU) patients in the UK with varying dietary adherence is unknown. In other countries, nutritional and metabolic abnormalities have been reported in nonadherent patients compared to adherent counterparts. A pooled analysis of primary baseline data from two UK multi-centre studies was therefore performed to establish whether this is true from a UK perspective. Adult PKU patients who had provided 3-day food records and amino acid blood samples were included and grouped according to dietary adherence (adherent; n = 16 vs. nonadherent; n = 14). Nonadherent patients consumed greater amounts of natural protein compared to adherent patients (61.6 ± 30.7 vs. 18.3 ± 7.7 g/day; q < 0.001). In contrast, the contribution of protein substitutes to total protein intake was lower in nonadherent compared to adherent patients (3.9 ± 9.2 g/day vs. 58.6 ± 10.2 g/day; q < 0.001). Intakes of iron, zinc, vitamin D₃, magnesium, calcium, selenium, iodine, vitamin C, vitamin A and copper were significantly lower in nonadherent compared to adherent patients and were below UK Reference Nutrient Intakes. Similarly, intakes of thiamin, riboflavin, niacin, vitamin B₆ and phosphorus were significantly lower in nonadherent compared to adherent patients but met the UK Reference Nutrient Intakes. Phenylalanine concentrations in nonadherent patients were significantly higher than adherent patients (861 ± 348 vs. 464 ± 196 µmol/L; q = 0.040) and fell outside of European treatment target ranges. This study shows the nutritional and metabolic consequences of deviation from phenylalanine restriction and intake of PKU protein substitutes in nonadherent adult PKU patients. Collectively, these data further underlie the importance of life-long adherence to the PKU diet.

Background: Prior evidence suggests that high-calcium intake influences postprandial appetite and insulinemia, possibly due to elevated incretins. In

According to national survey data, dairy food consumption has fallen in recent years and declines further with age, especially from childhood to adolescence. Dietary surveys typically rely on retrospective dietary assessment methods and use broad age groupings (4-10 yr; 11-18 yr), making it challenging to differentiate between middle-childhood and adolescence. Consequently, there is a need to assess dairy food consumption during middle-childhood and adolescence using more robust dietary assessment tools. Therefore, the present study aimed to describe and compare patterns of dairy consumption throughout middle-childhood and adolescence. Dairy food consumption was assessed during school term-time over 4 consecutive days, including 2 weekdays and 2 weekend days, in a sample of free-living children (9-11 yr, n=40) and adolescents (15-18 yr, n=35). For children, free-living dairy intake was evaluated through parental-weighed food records, and for adolescents, a combined weighed self-reported food record and 24-h dietary recall technique was utilized. Food records were explored to determine types, amounts, and frequency of dairy food consumption, and were analyzed for differences between middle-childhood and adolescence using a between group 2×2 (age×sex) ANOVA. Descriptive data suggested that milk was the most popular dairy product consumed by both children and adolescents. Statistical analysis revealed a main effect for sex on total milk consumption (mL) and number of daily milk portions consumed. No interaction or main effect was present for any other variable. The present study indicates that independent of age, boys consumed greater amounts of milk compared with girls. Contrary to existing literature, findings suggest no difference in milk-based dairy consumption between middle-childhood and adolescence.

This study was designed to assess the effect of a dairy-based recovery beverage on post-exercise appetite and energy intake in active females. Thirteen active females completed three trials in a crossover design. Participants completed 60 min of cycling at 65% V̇O_2peak, before a 120 min recovery period. On completion of cycling, participants consumed a commercially available dairy-based beverage (DBB), a commercially available carbohydrate beverage (CHO), or a water control (H₂O). Non-esterified fatty acids, glucose, and appetite-related peptides alongside measures of subjective appetite were sampled at baseline and at 30 min intervals during recovery. At 120 min, energy intake was assessed in the laboratory by ad libitum assessment, and in the free-living environment by weighed food record for the remainder of the study day. Energy intake at the ad libitum lunch was lower after DBB compared to H₂O (4.43 ± 0.20, 5.58 ± 0.41 MJ, respectively; p = 0.046; (95% CI: −2.28, −0.20 MJ)), but was not different to CHO (5.21 ± 0.46 MJ), with no difference between trials thereafter. Insulin and GLP-1_7-36 were higher following DBB compared to H₂O (p = 0.015 and p = 0.001, respectively) but not to CHO (p = 1.00 and p = 0.146, respectively). In addition, glucagon was higher following DBB compared to CHO (p = 0.008) but not to H₂O (p = 0.074). The results demonstrate that where DBB consumption may manifest in accelerated recovery, this may be possible without significantly affecting total energy intake and subsequent appetite-related responses relative to a CHO beverage.

The acute effects of active and seated video gaming on energy intake (EI), blood glucose, plasma glucagon-like peptide-1 (GLP-1_7–36) and subjective appetite (hunger, prospective food consumption and fullness) were examined in 8–11-year-old boys. In a randomised, crossover manner, twenty-two boys completed one 90-min active and one 90-min seated video gaming trial during which food and drinks were provided ad libitum. EI, plasma GLP-1_7–36, blood glucose and subjective appetite were measured during and following both trials. Time-averaged AUC blood glucose was increased (P=0·037); however, EI was lower during active video gaming (1·63 (sem 0·26) MJ) compared with seated video gaming (2·65 (sem 0·32) MJ) (P=0·000). In a post-gaming test meal 1 h later, there were no significant differences in EI between the active and seated gaming trials. Although estimated energy expenditure was significantly higher during active video gaming, there was still no compensation for the lower EI. At cessation of the trials, relative EI (REI) was significantly lower following active video gaming (2·06 (sem 0·30) MJ) v. seated video gaming (3·34 (sem 0·35) MJ) (P=0·000). No significant differences were detected in time-averaged AUC GLP-1_7–36 or subjective appetite. At cessation of the active video gaming trial, EI and REI were significantly less than for seated video gaming. In spite of this, the REI established for active video gaming was a considerable amount when considering the total daily estimated average requirement for 8–11-year-old boys in the UK (7·70 MJ).

Noncompliance is widespread in adults with PKU and is associated with adverse metabolic, nutritional and cognitive abnormalities. Returning to the PKU diet is important for this at-risk population, yet for many this is challenging to achieve. Strategies that ease the return to the PKU diet, while offering nutritional and cognitive advantages, are needed. Twelve PKU adults (33.7 ± 2.6 years), who had been noncompliant for 4.5 years (range: 1 to 11 years), took 33 g of a low-volume, nutrient-enriched, protein substitute daily for 28 days. Outcomes of eating behaviour, nutrient intake and mood were assessed at entry (baseline, days 1–3) and after the intervention period (days 29–31). At baseline, intakes of natural protein and estimated phenylalanine were high (66.4 g and 3318.5 mg, respectively) and intakes of calcium, magnesium, iron, zinc, iodine and vitamin D were below country-specific recommendations. With use of the experimental protein substitute, natural protein and estimated phenylalanine intake declined (p = 0.043 for both). Fat and saturated fat intakes also decreased (p = 0.019 and p = 0.041, respectively), while energy and carbohydrate intake remained unchanged. Micronutrient intake increased (p ≤ 0.05 for all aforementioned) to levels well within reference nutrient intake recommendations. Blood vitamin B₁₂ and vitamin D increased by 19.8% and 10.4%, respectively. Reductions in anxiety and confusion were also observed during the course of the study yet should be handled as preliminary data. This study demonstrates that reintroducing a low-volume, nutrient-enriched protein substitute delivers favourable nutritional and possible mood benefits in noncompliant PKU patients, yet longer-term studies are needed to further confirm this. This preliminary knowledge should be used in the design of new strategies to better facilitate patients’ return to the PKU diet, with the approach described here as a foundation.