Dietary habits and metabolic control in adolescents and young adults with phenylketonuria: self-imposed protein restriction may be harmful.
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ABSTRACT:
SUBMITTER: Das AM
PROVIDER: S-EPMC4110337 | biostudies-literature | 2014
REPOSITORIES: biostudies-literature
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Project description:BackgroundPatients with phenylketonuria (PKU) have to follow a lifelong phenylalanine restricted diet. This type of diet markedly reduces the intake of saturated and unsaturated fatty acids especially long chain polyunsaturated fatty acids (LC-PUFA). Long-chain saturated fatty acids are substrates of mitochondrial fatty acid oxidation for acetyl-CoA production. LC-PUFA are discussed to affect inflammatory and haemostaseological processes in health and disease. The influence of the long term PKU diet on fatty acid metabolism with a special focus on platelet eicosanoid metabolism has been investigated in the study presented here.Methodology/principal findings12 children with PKU under good metabolic control and 8 healthy controls were included. Activated fatty acids (acylcarnitines C6-C18) in dried blood and the cholesterol metabolism in serum were analyzed by liquid chromatographic tandem mass spectrometry (LC-MS/MS). Fatty acid composition of plasma glycerophospholipids was determined by gas chromatography. LC-PUFA metabolites were analyzed in supernatants by LC-MS/MS before and after platelet activation and aggregation using a standardized protocol. Patients with PKU had significantly lower free carnitine and lower activated fatty acids in dried blood compared to controls. Phytosterols as marker of cholesterol (re-) absorption were not influenced by the dietary fatty acid restriction. Fatty acid composition in glycerophospholipids was comparable to that of healthy controls. However, patients with PKU showed significantly increased concentrations of y-linolenic acid (C18:3n-6) a precursor of arachidonic acid. In the PKU patients significantly higher platelet counts were observed. After activation with collagen platelet aggregation and thromboxane B(2) and thromboxane B(3) release did not differ from that of healthy controls.Conclusion/significanceLong-term dietary fatty acid restriction influenced the intermediates of mitochondrial beta-oxidation. No functional influence on unsaturated fatty acid metabolism and platelet aggregation in patients with PKU was detected.
| S-EPMC3418234 | biostudies-literature
Project description:Several studies suggest that dietary habits are associated with poor academic performance. However, few studies have evaluated these relations after adjusting for numerous confounding factors. This study evaluated the frequency of various diet items (fruit, soft drinks, fast foods, instant noodles, confections, vegetables, and milk) and the regularity of meal times (breakfast, lunch, and dinner) all at once.A total of 359,264 participants aged from 12 to 18 years old were pooled from the Korea Youth Risk Behavior Web-based Survey (KYRBWS) for the 2009 to 2013 period. Dietary habits over the last 7 days were surveyed, including the regularity of consuming breakfast, lunch and dinner and the frequency of eating fruits, soft drinks, fast foods, instant noodles, confections, vegetables, and milk. Physical activity, obesity, region of residence, subjective assessment of health, stress level, economic level, and parental education level were collected from all of the study participants. School performance was classified into 5 levels. The adjusted odds ratios (AORs) of dietary habits for school performance were analyzed using multinomial logistic regression analyses with complex sampling. Structural equation modeling was used to analyze the effects of diet factors on school performance while considering the effects of other variables on both diet factors and school performance.Frequent intakes of breakfast (AOR?=?2.34, 95% confidence interval [CI]?=?2.20-2.48), fruits (AOR?=?1.73, 95% CI?=?1.62-1.86), vegetables (AOR?=?1.48, 95% CI?=?1.37-1.61), and milk (AOR?=?1.35, 95% CI?=?1.28-1.43) were related to high levels of school performance (each with P?<?0.001). In contrast, soft drinks (AOR?=?0.42, 95% CI?=?0.38-0.46), instant noodles (AOR?=?0.62, 95% CI?=?0.55-0.70), fast food (AOR?=?0.83, 95% CI?=?0.72-0.96), and confectionary (AOR?=?0.86, 95% CI?=?0.80-0.93) were negatively associated with school performance (each with P?<?0.001).This study confirms previous studies of school performance and dietary habits that find a positive association with eating breakfast and consuming fruits and milk and a negative relation with soft drinks, instant noodles, fast foods, and confections.
| S-EPMC4998375 | biostudies-literature
Project description:ContextAdolescence is a critical developmental stage in which increasing concerns about body image (BI) coincide with the consolidation of dietary habits (DHs). Multiple studies have sought robust associations between BI and DHs to prevent unhealthy behaviors.ObjectiveThe aim of this systematic review was to examine the available literature on the association between BI perception (BIP) and/or satisfaction (BIS) and DHs in adolescents.Data sourcesA search was carried out of 5 electronic databases (PubMed, SciELO, Cochrane, Embase, and PsycInfo) using a combination of keywords (and synonyms) related to adolescence, BI, and diet.Data extractionData screening, extraction, and quality assessment were performed independently by 2 investigators using the PRISMA and AXIS guidelines.Data analysisOf 2496 articles screened, 30 articles, published in English or Spanish, that evaluated the relationship between BI and DHs in adolescents aged between 10 years and 18 years, were included. A relationship between accurate BI perception in adolescents and healthy DHs was reported in 5 articles (16.2%). A relationship between overestimation of body weight in adolescents and healthy DHs was reported in 4 articles (13.3%). A relationship between underestimation of body weight and unhealthy DHs was reported in 8 articles (26.7%). In addition, 4 articles (13.3%) reported a relationship between BIS and healthy DHs. The desire to gain weight was associated with unhealthy DHs in 3 (10%) of the articles, while the desire to lose weight was related to healthy DHs in 3 (10%) of the articles and to unhealthy DHs in 3 (10%) other articles. There were also gender differences in the relationship between BIP or BIS and DHs.ConclusionAdolescents who underestimate their body weight tend to report less healthy DHs than body weight overestimators. Adolescents unsatisfied with their BI and with a drive for thinness frequently engage in DHs linked to losing weight.Systematic review registrationPROSPERO registration no. CRD42020184625.
| S-EPMC10711440 | biostudies-literature
Project description:The relationship between dietary habits and microbiota composition during adolescence has not been well examined. This is a crucial knowledge gap to fill considering that diet-microbiota interactions influence neurodevelopment, immune system maturation and metabolic regulation. This study examined the associations between diet and the gut microbiota in a school-based sample of 136 adolescents (Mage = 12·1 years; age range 11-13 years; 48 % female; 47 % Black, 38 % non-Hispanic White, 15 % Hispanic or other minorities) from urban, suburban and rural areas in the Southeast USA. Adolescents completed the Rapid Eating Assessment for Participants and provided stool samples for 16S ribosomal RNA gene sequencing. Parents reported their child and family socio-demographic characteristics. The associations between diet and socio-demographics with gut microbiota diversity and abundance were analysed using multivariable regression models. Child race and ethnicity, sex, socio-economic status and geographic locale contributed to variation within microbiota composition (β-diversity). Greater consumption of processed meat was associated with a lower microbial α-diversity after adjusting for socio-demographic variables. Multi-adjusted models showed that frequent consumption of nutrient-poor, energy-dense foods (e.g. sugar-sweetened beverages, fried foods, sweets) was negatively associated with abundances of genera in the family Lachnospiraceae (Anaerostipes, Fusicatenibacter and Roseburia), which are thought to play a beneficial role in host health through their production of short-chain fatty acids (SCFAs). These results provide new insights into the complex relationships among socio-demographic factors, diet and gut microbiota during adolescence. Adolescence may represent a critical window of opportunity to promote healthy eating practices that shape a homoeostatic gut microbiota with life-long benefits.
| S-EPMC10864997 | biostudies-literature
Project description:ObjectiveTo compare the dietary habits of children living in northern villages and in the capital of Greenland, given the reported transition from traditional to westernised diet in adults over recent decades, and to explore the association between consumption of marine mammals and fish (MMF) and the children's metabolic profile and vitamin D status.DesignChildren answered an FFQ encompassing sixty-four individual food types pooled into six food categories. Their pubertal stage, body fat, fitness level, metabolic profile (non-HDL-cholesterol, glycated Hb, insulin, glucose, high-sensitivity C-reactive protein) as well as serum 25-hydroxyvitamin D (25(OH)D) concentration were evaluated.SettingSiorapaluk and Qaanaaq (north of Greenland) and Nuuk (west).ParticipantsChildren aged 6-18 years (n 177).ResultsMMF were most frequently eaten by children from Siorapaluk (mean (sd): 73·4 (14·1) times/month), followed by children from Qaanaaq (37·0 (25·0) times/month), and least often eaten by children from Nuuk (23·7 (24·6) times/month; P < 0·001). Children from Qaanaaq consumed 'junk food' more frequently (P < 0·001) and fruits and vegetables less frequently (P < 0·01) than children from Nuuk. MMF consumption was positively associated with serum 25(OH)D concentration (P < 0·05), but the overall prevalence of vitamin D deficiency was high (18 %). No association was found between MMF consumption and metabolic parameters.ConclusionsThe dietary transition and influence of western diets have spread to the north of Greenland and only the most remote place consumed a traditional diet highly based on MMF. We found no strong associations of MMF consumption with metabolic health, but a positive association with vitamin D status.
| S-EPMC10200594 | biostudies-literature
Project description:OBJECTIVE:To compare a gram protein exchange system (1g=50-mg Phenylalanine) with a unit exchange system (1unit=15-mg Phenylalanine) and its effect on the blood Phenylalanine (Phe) levels and acceptance in the dietary management for children and adolescents with Phenylketonuria. METHODS:In Phase One, participants were randomised to continue counting Phe unit exchanges (n=8) or changed to counting gram protein exchanges (n=10), using a new diet chart developed in-house. Foods containing less than 20mg Phe per serve were now considered "free." Interim data analysis confirmed no significant deterioration in Phe levels of the study group and the control group was changed to protein counting.In Phase Two, 18 participants were educated to use an updated version of the in-house diet chart - in this version foods containing less than 50mg Phe per serve were considered "free."In both phases, attitudes to PKU and its management were evaluated at baseline and 6months. Phenylalanine and tyrosine levels were measured from filter paper blood spots by tandem mass spectrometry. RESULTS:Phase One: Phe levels over 6months were comparable to pre-study levels (mean Phe pre 366?mol/L+/- 169, mean Phe post change=388?mol/L+/- 160).Phase Two: Four participants had a significant improvement in blood Phe levels, nine showed no significant change and one participant's levels were significantly higher. There was incomplete data on four participants. All participants preferred the freer diet chart. CONCLUSION:Protein exchanges (foods containing less than 50mg Phe/serve uncounted) are an alternative method of measuring Phe intake in the dietary management of Phenylketonuria.
| S-EPMC3509851 | biostudies-literature
Project description: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.
| S-EPMC6835765 | biostudies-literature
Project description:Phenylalanine (Phe) tolerance is highly variable in phenylketonuria (PKU) and rarely described in patients aged ≥12 years. Patients ≥12 years of age with PKU were systematically challenged with additional natural protein (NP) if blood Phe levels remained below 480 µmol/L (i.e., upper target blood Phe level for patients aged ≥12 years using Portuguese PKU guidelines). In PKU patients, NP tolerance was calculated at baseline and a median of 6 months after systematic challenge with NP whilst patients were maintaining a blood Phe ≤480 μmol/L. Anthropometry was assessed at both times. Routine blood Phe levels were collected. We studied 40 well-controlled PKU patients (10 hyperphenylalaninemia (HPA), 23 mild and 7 classic PKU), on a low-Phe diet with a mean age of 17 years (12-29 years). Median daily NP intake significantly increased between assessments (35 vs. 40 g/day, p = 0.01). Twenty-six patients (65%) were able to increase their median NP intake by a median 12 g/day (2-42 g)/day and still maintain blood Phe within target range. Out of the previous 26 patients, 20 (77%) (8 HPA, 11 mild and 1 classical PKU) increased NP from animal sources (e.g. dairy products, fish and meat) and 6 patients (23%) (3 mild and 3 classical PKU) from plant foods (bread, pasta, potatoes). Median protein equivalent intake from Phe-free/low-Phe protein substitute decreased (0.82 vs. 0.75 g/kg, p = 0.01), while median blood Phe levels remained unchanged (279 vs. 288 μmol/L, p = 0.06). Almost two-thirds of patients with PKU tolerated additional NP when challenged and still maintained blood Phe within the national target range. This suggests that some patients with PKU treated by a low-Phe diet only may over restrict their NP intake. In order to minimise the burden of treatment and optimise NP intake, it is important to challenge with additional NP at periodic intervals.
| S-EPMC6566391 | biostudies-literature
Project description:BACKGROUND AND AIMS:BH4-sensitive phenylketonuria (PKU) patients relax their phenylalanine (Phe) restricted diet due to increased Phe tolerance, while keeping dried blood Phe concentrations with in the therapeutic range. We aimed to investigate metabolic control, eating habits and nutrient supply under long-term BH4-therapy. PATIENTS AND METHODS:Retrospective analysis of mean dried blood Phe concentrations and their variability, food and nutrient intake in BH4-sensitive patients (n = 8, 3f, age 6.0-16.6 y) under classical dietary treatment for one year and during the three years after initiation of BH4. RESULTS:Phe concentrations of BH4-sensitve PKU patients remained within therapeutic range throughout the observation period, independent of therapeutic regime. Under BH4, Phe tolerance increased significantly (493.2 ± 161.8 mg/d under classical diet vs 2021.93 ± 897.4 mg/d two years under BH4; P = 0.004). Variability of Phe concentrations remained unchanged (mean SD; P = 1.000). Patients adjust their food choice and significantly increased their intake of cereals, potatoes, dairy products and meat (P = 0.019, P = 0.016, P = 0.016 and P = 0.016, respectively). Under diet changes after implementation of BH4 a drop in micronutrient intake (vitamin D, folic acid, iron, calcium, iodine) could be revealed (P = 0.005, P < 0.001, P = 0.004, P = 0.001, P = 0.003, respectively). CONCLUSIONS:BH4-sensitive PKU patients can achieve good metabolic control under an adjuvant BH4- or a BH4 monotherapy. The liberalized diet under BH4 seems to jeopardize the quality of patients' nutrition, and these patients require close follow-up and special nutrition education to minimize the risk for imbalanced diet and nutrient deficiencies.
| S-EPMC4750587 | biostudies-literature
Project description:Chronic inflammation predisposes to aging-associated disease, but it is unknown whether immunity regulation might be important for extending healthy lifespan. Here we show that in C. elegans, dietary restriction (DR) extends lifespan by modulating a conserved innate immunity pathway that is regulated by p38 signaling and the transcription factor ATF-7. Longevity from DR depends upon p38-ATF-7 immunity being intact but downregulated to a basal level. p38-ATF-7 immunity accelerates aging when hyperactive, influences lifespan independently of pathogen exposure, and is activated by nutrients independently of mTORC1, a major DR mediator. Longevity from reduced insulin/IGF-1 signaling (rIIS) also involves p38-ATF-7 downregulation, with signals from DAF-16/FOXO reducing food intake. We conclude that p38-ATF-7 is an immunometabolic pathway that senses bacterial and nutrient signals, that immunity modulation is critical for DR, and that DAF-16/FOXO couples appetite to growth regulation. These conserved mechanisms may influence aging in more complex organisms.
| S-EPMC6506407 | biostudies-literature