Advances in Ketogenic Diet Therapies in Pediatric Epilepsy: A Systematic Review.

Objective: To review the effects of the ketogenic diet on epilepsy in children and adolescents.Data Sources: A literature search was conducted in PubMed with no publication date or language restrictions based on the Preferred Reporting Items for Systematic Reviews and Meta Analyses guidelines. Keywords used included children, adolescent, ketogenic diet, epilepsy, and seizure.Study Selection: After excluding articles that did not meet the inclusion criteria, such as missing variables of study, adult population, and nonrandomized clinical trials, a total of 12 studies were included in the final review.Data Extraction: Data on study design, duration, sample size, population, and type of intervention were collected using a standard template.Results: The ketogenic diet and its modified versions were noted to have beneficial effects in reduction of seizure frequency and severity, with manageable adverse effects such as gastrointestinal disturbances, dehydration, dyslipidemia, hyperuricemia, infection, and metabolic acidosis.Conclusions: Depending on patient compliance and comorbidities, all variations of the ketogenic diet were found to be helpful for seizure treatment, whether as an additive or an alternative treatment option, for children and adolescents with epilepsy.Prim Care Companion CNS Disord 2024;26(3):23r03661. Author affiliations are listed at the end of this article.

The role of diet in diabetes gastroparesis treatment: a systematic review and meta-analysis.

Background: Diabetic gastroparesis is a common complication in patient with diabetes. Dietary intervention has been widely used in the treatment of diabetic gastroparesis. The aim of this study is to evaluate the role of diet in the treatment of diabetic gastroparesis. Methods: This systematic review was conducted a comprehensive search of randomized controlled trials using dietary interventions for the treatment of diabetic gastroparesis up to 9 November 2023. The primary outcomes were gastric emptying time and clinical effect, while fasting blood glucose, 2-hour postprandial blood glucose and glycosylated hemoglobin were secondary outcomes. Data analysis was performed using RevMan 5.4 software, and publication bias test was performed using Stata 15.1 software. Results: A total of 15 randomized controlled trials involving 1106 participants were included in this review. The results showed that patients with diabetic gastroparesis benefit from dietary interventions (whether personalized dietary care alone or personalized dietary care+routine dietary care). Compared with routine dietary care, personalized dietary care and personalized dietary care+routine dietary care can shorten the gastric emptying time, improve clinical efficacy, and reduce the level of fasting blood glucose, 2-hour postprandial blood glucose and glycosylated hemoglobin. Conclusions: Limited evidence suggests that dietary intervention can promote gastric emptying and stabilize blood glucose control in patients with diabetic gastroparesis. Dietary intervention has unique potential in the treatment of diabetic gastroparesis, and more high-quality randomized controlled trials are needed to further validate our research results. Systematic review registration: https://www.crd.york.ac.uk/prospero/, identifier CRD42023481621.

Effects of calorie-restricted diet on health state and intestinal flora in Hashimoto's thyroiditis patients: Study protocol for a randomized controlled trial.

BACKGROUND AND OBJECTIVES: Hashimoto's thyroiditis (HT) is an autoimmune disease, characterized by abnormal elevation in thyroid peroxidase antibody and/or thyroglobulin antibody. In recent decades, HT disease has become more and more widespread. Patients always report multiple symptoms, even though their thyroid hormone levels are kept in normal ranges. However, no treatment exists to effectively reduce the levels of thyroid antibodies. Our study aims to determine whether calorie-restricted diet is helpful in improving health of HT patients. METHODS AND STUDY DESIGN: This is a 3-month randomized controlled trial. HT patients will be randomized into a calorie-restricted (CR) group or a calorie-unrestricted control group. All the participants will be instructed to consume a diet that includes a combination of 45-55% calories from carbohydrates, 20-30% from fats, and 15-25% from proteins, according to current Chinese Dietary Guidelines. Participants in CR group need to limit their calories intake equal to their basal energy expenditure, which means that their daily caloric intake will be limited by about 20-30%. RESULTS: The study population is planned to be 66 HT patients aged 18 to 65 years. The primary outcome is change of thyroid antibody levels from baseline. Secondary outcomes include the changes of non-hypothyroid symptoms scores, thyroid function indexes, morphology of thyroid, T lymphocyte subpopulations, inflammatory biomarkers and lipids from baseline to 12 weeks. CONCLUSIONS: This trial will have implications for nutrition treatment policy in regard to thyroid antibodies control, immune dysfunction and related non-hypothyroid symptoms improvement among HT patients.

Effect of Diet Counseling on Nutritional Status of Chronic Respiratory Diseases Patients Enrolled in the Pulmonary Rehabilitation Program in a Teaching Hospital: A Pre-Post Intervention Study.

We conducted the study to assess the effect of patient-tailored diet counseling on the nutritional status of chronic respiratory disease (CRD) patients under the pulmonary rehabilitation program from June 2021-May 2022. These patients completed 2 months of patient-tailored diet counseling sessions under the pulmonary rehabilitation program, which consisted of 4-5 interactive diet counseling sessions fortnightly. The pre- and postassessment was done using standardized outcomes: Malnutrition Universal Screening Tool (MUST), body mass index (BMI), and ideal body weight. The study enrolled 110 CRD patients. There was a statistically significant improvement in pre- and postassessment in MUST score, appetite, and unintentional weight loss (P < 0.001). Most of the patient's BMI normalized. In prenutritional assessment, most of the patients were malnourished and in postassessment, the number of malnourished and anemic patients was reduced. This study concludes that nutritional counseling effectively improves nutritional status and anemia.

[Formula diets for weight loss - chances and challenges]. / Formula-Diäten für den Gewichtsverlust ­ Chancen und Herausforderungen.

INTRODUCTION: Formula diets, through the use of industrially manufactured meal replacement products, lead to effective and rapid weight reduction and improvement in obesity-associated comorbidities. The specific composition of these meal replacement products simplifies the adherence to calorie goals and ensures the supply of essential nutrients during significant energy restriction. Despite numerous potential applications, evidence from randomized controlled studies, and simplicity in practical implementation, challenges persist. Monotony and social restrictions complicate adherence and acceptance. The use of formula diets for sustainable weight loss requires integration into a multimodal treatment approach with the goal of long-term changes in eating and activity behaviour. This includes accompanying nutritional counselling, promotion of physical activity, evaluation of adjuvant pharmacological or interventional therapies, as well as psychological support. The development of new incretin-based anti-obesity medications has opened another application field for formula products. There is optimization potential in expanding the product range and combining it with digital applications to enhance acceptance and reach a larger patient group.

A framework to classify gestational diabetes diagnosed by routine antenatal 75g glucose tolerance testing.

OBJECTIVE: To create an objective framework to classify gestational diabetes mellitus diagnosed by routine antenatal 75 g diabetes testing results to provide an alternative to current treatment-based classification. METHODS: A framework was created to classify gestational diabetes according to the severity of glycemic abnormalities after routine antenatal 75 g GTT (classes 1 through 4, determined by fasting and post-test glycemic abnormalities). A retrospective cohort chart review was used to correlate clinically how often diet therapy alone maintained glycemic targets throughout pregnancy in each class. Chi-square analysis was used to assess inter-class differences in the success of diet therapy alone maintaining glycemic targets throughout pregnancy. RESULTS: Seventy-four of 228 (33%), 35/228 (15%), 76/228 (33%), and 43/228 (19%) of the study population were classified as Class 1, 2, 3, or 4, respectively. Of eighty-nine patients who maintained glycemic targets throughout pregnancy with diet alone 51/89 (57%) were Class 1, 20/89 (22.5%) were Class 2, 11/89 (12.5%) were Class 3, and 7/89 (8%) were Class 4. Chi-square analysis showed statistically significant inter-class differences in the likelihood of diet therapy alone maintaining glycemic targets throughout pregnancy. CONCLUSION: In this framework classifying gestational diabetes according to the severity of glycemic abnormalities after routine antenatal 75 g GTT (an objective proxy for disease severity), the higher the assigned class, the less likely that diet therapy alone maintained glycemic targets throughout pregnancy (a clinical proxy for disease severity).

Caloric restriction leading to attenuation of experimental Alzheimer's disease results from alterations in gut microbiome.

BACKGROUND: Caloric restriction (CR) might be effective for alleviating/preventing Alzheimer's disease (AD), but the biological mechanisms remain unclear. In the current study, we explored whether CR caused an alteration of gut microbiome and resulted in the attenuation of cognitive impairment of AD animal model. METHODS: Thirty-week-old male APP/PS1 transgenic mice were used as AD models (AD mouse). CR was achieved by 30% reduction of daily free feeding (ad libitum, AL) amount. The mice were fed with CR protocol or AL protocol for six consecutive weeks. RESULTS: We found that with CR treatment, AD mice showed improved ability of learning and spatial memory, and lower levels of Aß40, Aß42, IL-1ß, TNF-α, and ROS in the brain. By sequencing 16S rDNA, we found that CR treatment resulted in significant diversity in composition and abundance of gut flora. At the phylum level, Deferribacteres (0.04%), Patescibacteria (0.14%), Tenericutes (0.03%), and Verrucomicrobia (0.5%) were significantly decreased in CR-treated AD mice; at the genus level, Dubosiella (10.04%), Faecalibaculum (0.04%), and Coriobacteriaceae UCG-002 (0.01%) were significantly increased in CR-treated AD mice by comparing with AL diet. CONCLUSIONS: Our results demonstrate that the attenuation of AD following CR treatment in APP/PS1 mice may result from alterations in the gut microbiome. Thus, gut flora could be a new target for AD prevention and therapy.

An open-label, randomized controlled trial to assess a ketogenic diet in critically ill patients with sepsis.

Patients with sepsis experience metabolic and immunologic dysfunction that may be amplified by standard carbohydrate-based nutrition. A ketogenic diet (KD) may offer an immunologically advantageous alternative, although clinical evidence is limited. We conducted a single-center, open-label, randomized controlled trial to assess whether a KD could induce stable ketosis in critically ill patients with sepsis. Secondary outcomes included assessment of feasibility and safety of KD, as well as explorative analysis of clinical and immunological characteristics. Forty critically ill adults were randomized to either a ketogenic or standard high-carbohydrate diet. Stable ketosis was achieved in all KD patients, with significant increases in ß-hydroxybutyrate levels compared with controls [mean difference 1.4 milimoles per liter; 95% confidence interval (CI): 1.0 to 1.8; P < 0.001). No major adverse events or harmful metabolic side effects (acidosis, dysglycemia, or dyslipidemia) were observed. After day 4, none of the patients in the KD group required insulin treatment, whereas in the control group, insulin dependency ranged between 35% and 60% (P = 0.009). There were no differences in 30-day survival, but ventilation-free [incidence rate ratio (IRR) 1.7; 95% CI: 1.5 to 2.1; P < 0.001], vasopressor-free (IRR 1.7; 95% CI: 1.5 to 2.0; P < 0.001), dialysis-free (IRR 1.5; 95% CI: 1.3 to 1.8; P < 0.001), and intensive care unit-free days (IRR 1.7; 95% CI: 1.4 to 2.1; P < 0.001) were higher in the ketogenic group. Next-generation sequencing of CD4+/CD8+ T cells and protein analyses showed reduced immune dysregulation, with decreased gene expression of T-cell activation and signaling markers and lower pro-inflammatory cytokine secretion. This trial demonstrated the safe induction of a stable ketogenic state in sepsis, warranting larger trials to investigate potential benefits in sepsis-related organ dysfunction.

Nutrition guidance within a multimodal intervention improves diet quality in prodromal Alzheimer's disease: Multimodal Preventive Trial for Alzheimer's Disease (MIND-ADmini).

BACKGROUND: Multimodal lifestyle interventions can benefit overall health, including cognition, in populations at-risk for dementia. However, little is known about the effect of lifestyle interventions in patients with prodromal Alzheimer's disease (AD). Even less is known about dietary intake and adherence to dietary recommendations within this population making it difficult to design tailored interventions for them. METHOD: A 6-month MIND-ADmini pilot randomized controlled trial (RCT) was conducted among 93 participants with prodromal AD in Sweden, Finland, Germany, and France. Three arms were included in the RCT: 1) multimodal lifestyle intervention (nutritional guidance, exercise, cognitive training, vascular/metabolic risk management, and social stimulation); 2) multimodal lifestyle intervention + medical food product; and 3) regular health advice (control group). Adherence to dietary advice was assessed with a brief food intake questionnaire by using the Healthy Diet Index (HDI) and Mediterranean Diet Adherence Screener (MEDAS). The intake of macro- and micronutrients were analyzed on a subsample using 3-day food records. RESULTS: The dietary quality in the intervention groups, pooled together, improved compared to that of the control group at the end of the study, as measured with by HDI (p = 0.026) and MEDAS (p = 0.008). The lifestyle-only group improved significantly more in MEDAS (p = 0.046) and almost significantly in HDI (p = 0.052) compared to the control group, while the lifestyle + medical food group improved in both HDI (p = 0.042) and MEDAS (p = 0.007) during the study. There were no changes in macro- or micronutrient intake for the intervention groups at follow-up; however, the intakes in the control group declined in several vitamins and minerals when adjusted for energy intake. CONCLUSION: These results suggest that dietary intervention as part of multimodal lifestyle interventions is feasible and results in improved dietary quality in a population with prodromal AD. Nutrient intakes remained unchanged in the intervention groups while the control group showed a decreasing nutrient density. TRIAL REGISTRATION: ClinicalTrials.gov NCT03249688, 2017-07-08.

Dietary Supplementation with n-3 Polyunsaturated Fatty Acids Delays the Phenotypic Manifestation of Krabbe Disease and Partially Restores Lipid Mediator Production in the Brain-Study in a Mouse Model of the Disease.

Lipid mediators from fatty acid oxidation have been shown to be associated with the severity of Krabbe disease (KD), a disorder linked to mutations in the galactosylceramidase (GALC) gene. This study aims to investigate the effects of n-3 polyunsaturated fatty acid (PUFA) supplementation on KD traits and fatty acid metabolism using Twitcher (Tw) animals as a natural model for KD. Wild-type (Wt), heterozygous (Ht), and affected Tw animals were treated orally with 36 mg n-3 PUFAs/kg body weight/day from 10 to 35 days of life. The end product of PUFA peroxidation (8-isoprostane), the lipid mediator involved in the resolution of inflammatory exudates (resolvin D1), and the total amount of n-3 PUFAs were analyzed in the brains of mice. In Tw mice, supplementation with n-3 PUFAs delayed the manifestation of disease symptoms (p < 0.0001), and in the bran, decreased 8-isoprostane amounts (p < 0.0001), increased resolvin D1 levels (p < 0.005) and increased quantity of total n-3 PUFAs (p < 0.05). Furthermore, total brain n-3 PUFA levels were associated with disease severity (r = -0.562, p = 0.0001), resolvin D1 (r = 0.712, p < 0.0001), and 8-isoprostane brain levels (r = -0.690, p < 0.0001). For the first time in a natural model of KD, brain levels of n-3 PUFAs are shown to determine disease severity and to be involved in the peroxidation of brain PUFAs as well as in the production of pro-resolving lipid mediators. It is also shown that dietary supplementation with n-3 PUFAs leads to a slowing of the phenotypic presentation of the disease and restoration of lipid mediator production.

Greater adherence to the Mediterranean Dietary Approaches to Stop Hypertension Intervention for Neurodegenerative Delay (MIND) diet is associated with lower risk of inflammatory bowel disease: a prospective cohort study.

Background: The Mediterranean-DASH Intervention for Neurodegenerative Delay (MIND) diet is emerging as a promising candidate for preventive measures against inflammatory bowel disease (IBD), though there is currently no direct evidence from population-based studies. This study aims to bridge the gap in understanding of the association of the MIND diet with IBD risk. Methods: We utilized data from 187 490 participants in the UK Biobank who provided dietary information and were free of IBD at baseline. Dietary information was obtained using a validated web-based 24-hour dietary recall questionnaire. A MIND diet score was evaluated based on the intake of ten beneficial and five unhealthy food groups and the scores were further grouped into tertiles. The outcome of interest was incident IBD, Crohn's disease (CD), and ulcerative colitis (UC). Multivariable-adjusted hazard ratios (HRs) and 95% confidence intervals (CIs) were estimated using Cox proportional hazard models adjusted for demographic characteristics, lifestyle factors, cancer history, and other dietary factors. Mediation analyses were performed to evaluate the role of systemic inflammation and metabolic disorders represented by the integrated biomarkers in the MIND diet-IBD association. Results: After a mean follow-up of 10.7 years, we documented 825 incident IBD cases (250 CD and 575 UC). The average age of the participants was 56.2 years, of which 55.0% were females. We found that greater adherence to the MIND diet, represented by a higher diet score, was associated with a lower risk of IBD (HRcomparing extreme tertiles 0.74, 95% CI 0.62-0.90, p = 0.002; p for trend = 0.005), CD (HR 0.66, 95% CI 0.47-0.94, p = 0.022; p for trend = 0.023), and UC (HR 0.78, 95% CI 0.62-0.98, p = 0.031; p for trend = 0.022). The associations were partially mediated by metabolic and inflammation status (mediation proportion: 5.5-15.9%). Conclusion: We found higher adherence to the MIND diet was associated with a lower risk of IBD, and that inflammatory and metabolic conditions may play an important role in the underlying mechanistic pathways.

Beneficial effects of a new probiotic formulation on adipocytokines, appetite-regulating hormones, and metabolic parameters in obese women.

Obesity is often accompanied by low-grade chronic inflammation and metabolic syndrome. It has been established that microbiota influences many physiological processes, including the development of obesity, and dysbiosis has been observed in obese individuals. In this study, we aimed to evaluate the impact of a new probiotic formulation, containing two probiotic strains and the bioactive compound octacosanol, on body weight, metabolic parameters, and concentrations of certain adipocytokines and appetite-regulating hormones in obese women. This double blind placebo-controlled supplementary intervention study included twenty-five women in the intervention group and twenty-three in the placebo group, and it lasted 12 weeks. Daily oral supplementation included 7 × 1010 CFU of Lactiplantibacillus plantarum 299v (DSM9843), 5 × 109 CFU of Saccharomyces cerevisiae var. boulardii (DBVPG6763), and 40 mg of octacosanol or placebo. Body weight, metabolic parameters, adipocytokines, and appetite-regulating hormones were assessed before (T0) and after the intervention (T1). After the intervention, significantly lower median concentrations of CRP (p = 0.005) and IL-6 (p = 0.012) were measured in the intervention group than the baseline, while the median concentrations of ghrelin (p = 0.026) and HDL-cholesterol (p = 0.03) were significantly increased. The intervention group had lower CRP levels (p = 0.023) and higher ghrelin levels (p = 0.006) than the placebo group. Significant changes in BMI between groups were not observed. In summary, although the new probiotic formulation showed beneficial effects on IL-6, CRP, HDL, and ghrelin levels, its potential effects on regulating triglyceride, insulin, and glucose levels require further studies before the novel dietary intervention could be considered a useful adjuvant therapy and an effective strategy for the management of obesity and obesity-associated comorbidities.

Dietary management of gestational diabetes: A review.

Gestational diabetes mellitus (GDM) is a common condition in pregnant women that can affect the health of both the mother and the fetus. A healthy diet reduces the risk of GDM, while on the contrary, an unhealthy diet can increase the risk of developing GDM. Dietary interventions remain an important way to control GDM at this time. However, real-life diets are complex and varied, and the effect of these diets on gestational diabetes is unknown. This article summarizes research related to dietary control of GDM. Hopefully, this will help with dietary interventions for people with GDM.

Interactive Effects of Arabinoxylan Oligosaccharides and Green Tea Polyphenols on Obesity Management and Gut Microbiota Modulation in High-Fat Diet-Fed Mice.

Dietary fiber and polyphenols have been shown to possess antiobesity properties. However, their combined effects need further investigation. This study investigated the individual and combined effects of arabinoxylan oligosaccharides (AXOS) from rice bran and green tea polyphenols (GTP) in high-fat diet-induced obese mice. We found that the combination of AXOS and GTP (A + G) significantly reduced overall fat mass and improved lipid profiles, although the effects were not synergistic. AXOS and GTP regulated lipid metabolism in different tissues and exhibited counteractive effects on gut microbiota. AXOS decreased α diversity and promoted Bifidobacterium, with GTP counteracting these effects. In vitro fermentation confirmed that GTP counteracted AXOS-induced microbiota changes in a dose-dependent manner. This study highlights the potential of tailored combinations of dietary fiber and polyphenols to treat obesity while considering their complex microbial interplay.

Celiac Disease, Gluten-Free Diet and Metabolic Dysfunction-Associated Steatotic Liver Disease.

Celiac disease (CD) is a chronic autoimmune disorder triggered by the ingestion of gluten-containing food by genetically predisposed individuals. Hence, treatment of CD consists of permanent avoidance of wheat, rye, barley, and other gluten-containing foods. Lifelong adherence to a gluten-free diet (GFD) improves the symptoms of CD, but recent evidence suggests it is also associated with a higher risk for hepatic steatosis and the coexistence or emergence of other cardiometabolic risk factors. Moreover, a higher risk for liver steatosis is also reported by some authors as a potential extraintestinal complication of the CD itself. Recent nomenclature changes designate the association between hepatic steatosis and at least one of five cardiometabolic risk factors as metabolic dysfunction-associated steatotic liver disease (MASLD). An extended network of potentially causative factors underlying the association between MAFLD and CD, before and after dietary therapy is implemented, was recently described. The individualized treatment of these patients is less supported by evidence, with most of the current recommendations relying on empiric clinical judgment. This review focuses on the causative associations between CD and hepatic injury, either as an extraintestinal manifestation of CD or a side effect of GFD, also referring to potential therapeutic strategies for these individuals.

Digital Biohacking Approach to Dietary Interventions: A Comprehensive Strategy for Healthy and Sustainable Weight Loss.

The rising obesity epidemic requires effective and sustainable weight loss intervention strategies that take into account both of individual preferences and environmental impact. This study aims to develop and evaluate the effectiveness of an innovative digital biohacking approach for dietary modifications in promoting sustainable weight loss and reducing carbon footprint impact. A pilot study was conducted involving four participants who monitored their weight, diet, and activities over the course of a year. Data on food consumption, carbon footprint impact, calorie intake, macronutrient composition, weight, and energy expenditure were collected. A digital replica of the metabolism based on nutritional information, the Personalized Metabolic Avatar (PMA), was used to simulate weight changes, plan, and execute the digital biohacking approach to dietary interventions. The dietary modifications suggested by the digital biohacking approach resulted in an average daily calorie reduction of 236.78 kcal (14.24%) and a 15.12% reduction in carbon footprint impact (-736.48 gCO2eq) per participant. Digital biohacking simulations using PMA showed significant differences in weight change compared to actual recorded data, indicating effective weight reduction with the digital biohacking diet. Additionally, linear regression analysis on real data revealed a significant correlation between adherence to the suggested diet and weight loss. In conclusion, the digital biohacking recommendations provide a personalized and sustainable approach to weight loss, simultaneously reducing calorie intake and minimizing the carbon footprint impact. This approach shows promise in combating obesity while considering both individual preferences and environmental sustainability.

A Healthful Plant-Based Diet as an Alternative Dietary Approach in the Management of Metabolic Dysfunction-Associated Steatotic Liver Disease.

Plant-based diets (PBDs) are gaining attention as a sustainable and health-conscious alternative for managing various chronic conditions, including metabolic dysfunction-associated steatotic liver disease (MASLD). In the absence of pharmacological treatments, exploring the potential of lifestyle modifications to improve biochemical and pathological outcomes becomes crucial. The adoption of PBDs has demonstrated beneficial effects such as weight control, increased metabolic health and improved coexisting diseases. Nonetheless, challenges persist, including adherence difficulties, ensuring nutritional adequacy, and addressing potential deficiencies. The aim of this review is to provide a comprehensive overview of the impact of PBDs on MASLD, emphasizing the need for tailored dietary interventions with professional support to optimize their effectiveness in preventing and treating metabolic diseases.

Health-Related Quality of Life during 26-Week Intervention with the New Nordic Renal Diet.

The New Nordic Renal Diet (NNRD) is a whole-food approach, tailored to meet recommended guidelines in patients with moderate chronic kidney disease (stage 3b-4). The NNRD improved various metabolic and physiological endpoints during a 26-week randomized controlled study. Here, we examined the effect of dietary intervention on health-related quality of life (HRQoL). Sixty participants were recruited (NNRD group n = 30, control group n = 30) and 58 completed the study. During the intervention, the NNRD group received food boxes, and recipes once a week. The control group continued their habitual diet. HRQoL was examined at baseline and at the end of the intervention using the validated EuroQol-5D-5L, including a 5-point scale Likert questionnaire at the end of the intervention. Assessed by the EuroQol-5D-5L questionnaire, the NNRD group experienced a reduction in pain/discomfort during the intervention by 26% [-0.44 points (95% CI; -0.73, -0.16)], compared with no change in the control group [0.25 points (95% CI; -0.02, 0.53)] and a between-group difference of -0.70 points (95% CI; -1.03, -0.37, p < 0.001). A larger decrease of body fat mass was associated with a larger decrease in pain/discomfort (p = 0.014). In addition, the NNRD group reported an overall improvement in conducting usual daily activities by 23% [-0.30-point (95% CI; -0.50, -0.11)], while no change was seen in the control group [-0.02 points (95% CI; -0.21, 0.17)], with a between-group difference -0.28 points (95% CI; -0.51, -0.06, p = 0.014). A larger decrease in 24 h urine phosphorus excretion, used as a marker of compliance, was associated with a larger improvement in conducting usual daily activities (p = 0.036). The NNRD group had a clinically relevant improvement in various HRQoL outcomes.

No Impairment in Bone Turnover or Executive Functions in Well-Treated Preschoolers with Phenylketonuria-A Pilot Study.

Patients with phenylketonuria (PKU) present signs of impaired executive functioning and bone health in adolescence and adulthood, depending in part on the success of therapy in childhood. Therefore, nine children with well-treated PKU (4-7 years old, 22.2% ♀, seven with a full set of data, two included into partial analysis) and 18 age-, gender- and season-matched controls were analyzed for differences in executive functioning and bone parameters in plasma. Plasma was analyzed with commercially available kits. Cognitive performance in tonic alertness, visuo-spatial working memory, inhibitory control and task switching was assessed by a task battery presented on a touch screen. Regarding cognition, only the performance in incongruent conditions in inhibitory control was significantly better in children with PKU than in controls. No further differences in cognitive tests were detected. Furthermore, no significant difference in the bone turnover markers osteocalcin, undercarboxylated osteocalcin and CTX were detected between children with PKU and controls, while children with PKU had a significantly higher vitamin D concentration (69.44 ± 12.83 nmol/L vs. 41.87 ± 15.99 nmol/L, p < 0.001) and trended towards lower parathyroid hormone concentrations than controls (48.27 ± 15.16 pg/mL vs. 70.61 ± 30.53 pg/mL, p = 0.066). In this small group of well-treated preschoolers with PKU, no impairments in cognitive performance and bone turnover were observed, while vitamin D supplementation of amino acid supplements seems to be sufficient to achieve good vitamin D status.

A Multi-Omics Approach to Disclose Metabolic Pathways Impacting Intestinal Permeability in Obese Patients Undergoing Very Low Calorie Ketogenic Diet.

A very low calorie ketogenic diet (VLCKD) impacts host metabolism in people marked by an excess of visceral adiposity, and it affects the microbiota composition in terms of taxa presence and relative abundances. As a matter of fact, there is little available literature dealing with microbiota differences in obese patients marked by altered intestinal permeability. With the aim of inspecting consortium members and their related metabolic pathways, we inspected the microbial community profile, together with the set of volatile organic compounds (VOCs) from untargeted fecal and urine metabolomics, in a cohort made of obese patients, stratified based on both normal and altered intestinal permeability, before and after VLCKD administration. Based on the taxa relative abundances, we predicted microbiota-derived metabolic pathways whose variations were explained in light of our cohort symptom picture. A totally different number of statistically significant pathways marked samples with altered permeability, reflecting an important shift in microbiota taxa. A combined analysis of taxa, metabolic pathways, and metabolomic compounds delineates a set of markers that is useful in describing obesity dysfunctions and comorbidities.