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Long-term follow-up of nutritional status in children with GLUT1 Deficiency Syndrome treated with classic ketogenic diet: a 5-year prospective study.
De Amicis, R, Leone, A, Pellizzari, M, Foppiani, A, Battezzati, A, Lessa, C, Tagliabue, A, Ferraris, C, De Giorgis, V, Olivotto, S, et al
Frontiers in nutrition. 2023;:1148960
Abstract
INTRODUCTION The classic ketogenic diet (cKD) is an isocaloric, high fat, low-carbohydrate diet that induces the production of ketone bodies. High consumption of dietary fatty acids, particularly long-chain saturated fatty acids, could impair nutritional status and increase cardiovascular risk. The purpose of this study was to evaluate the long-term effects of a 5-year cKD on body composition, resting energy expenditure, and biochemical parameters in children affected by Glucose Transporter 1 Deficiency Syndrome (GLUT1DS). METHODS This was a prospective, multicenter, 5-year longitudinal study of children with GLUT1DS treated with a cKD. The primary outcome was to assess the change in nutritional status compared with pre-intervention, considering anthropometric measurements, body composition, resting energy expenditure, and biochemical parameters such as glucose and lipid profiles, liver enzymes, uric acid, creatinine, and ketonemia. Assessments were conducted at pre-intervention and every 12 months of cKD interventions. RESULTS Ketone bodies increased significantly in children and adolescents, and remained stable at 5 years, depending on the diet. No significant differences were reported in anthropometric and body composition standards, as well as in resting energy expenditure and biochemical parameters. Bone mineral density increased significantly over time according to increasing age. Body fat percentage significantly and gradually decreased in line with the increase in body weight and the consequent growth in lean mass. As expected, we observed a negative trend in respiratory quotient, while fasting insulin and insulin resistance were found to decrease significantly after cKD initiation. CONCLUSION Long-term adherence to cKD showed a good safety profile on anthropometric measurements, body composition, resting energy expenditure, and biochemical parameters, and we found no evidence of potential adverse effects on the nutritional status of children and adolescents.
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Ultra-Processed Food Consumption and Incidence of Obesity and Cardiometabolic Risk Factors in Adults: A Systematic Review of Prospective Studies.
Mambrini, SP, Menichetti, F, Ravella, S, Pellizzari, M, De Amicis, R, Foppiani, A, Battezzati, A, Bertoli, S, Leone, A
Nutrients. 2023;(11)
Abstract
Ultra-processed foods (UPF) are energy-dense, nutritionally unbalanced products, low in fiber but high in saturated fat, salt, and sugar. Recently, UPF consumption has increased likewise the incidence of obesity and cardiometabolic diseases. To highlight a possible relationship, we conducted a systematic review of prospective studies from PubMed and Web of Science investigating the association between UPF consumption and the incidence of obesity and cardiometabolic risk factors. Seventeen studies were selected. Eight evaluated the incidence of general and abdominal obesity, one the incidence of impaired fasting blood glucose, four the incidence of diabetes, two the incidence of dyslipidemia, and only one the incidence of metabolic syndrome. Studies' quality was assessed according to the Critical Appraisal Checklist for cohort studies proposed by the Joanna Briggs Institute. Substantial agreement emerged among the studies in defining UPF consumption as being associated with the incident risk of general and abdominal obesity. More limited was the evidence on cardiometabolic risk. Nevertheless, most studies reported that UPF consumption as being associated with an increased risk of hypertension, diabetes, and dyslipidemia. In conclusion, evidence supports the existence of a relationship between UPF consumption and the incidence of obesity and cardiometabolic risk. However, further longitudinal studies considering diet quality and changes over time are needed.
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Study protocol of a clinical randomized controlled trial on the efficacy of an innovative Digital thErapy to proMote wEighT loss in patients with obesity by incReasing their Adherence to treatment: the DEMETRA study.
Castelnuovo, G, Capodaglio, P, De Amicis, R, Gilardini, L, Mambrini, SP, Pietrabissa, G, Cavaggioni, L, Piazzolla, G, Galeone, C, Garavaglia, G, et al
Frontiers in digital health. 2023;:1159744
Abstract
Despite the increasing importance of innovative medications and bariatric surgery for the treatment of obesity, lifestyle interventions (diet and physical activity) remain the first-line therapy for this disease. The use of digital devices in healthcare aims to respond to the patient's needs, in order to make obesity treatment more accessible, so our study aims to assess the safety and efficacy of a Digital Therapy for Obesity App (DTxO) for achieving weight loss and its maintenance in patients affected with obesity undergoing an experimental non-pharmacological treatment. Here we present the study protocol of a prospective, multicenter, pragmatic, randomized, double-arm, placebo-controlled, parallel, single-blind study on obese patients who will be treated with a new digital therapy to obtain an improvement in their disease condition through the application of different simultaneous strategies (a dietary regimen and personalized advice program, a tailored physical exercise program, a cognitive-behavioural assessment and program, alerts and reminders, dedicated section on prescribed drugs intake, and chat and online visits with clinical professionals). We believe that DTxO will offer a promising intervention channel and self-regulation tool holding the potentiality to decrease treatment burden and treat more patients thanks to the partial replacement of traditional medical consultation with digital or telephone management, improving self- engagement and reducing the high demands the "obesity pandemic" for both patients and national health services in terms of time, cost, and effort. Clinical trial registration: clinicaltrials.gov, identifier, NCT05394779.
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Acute Insulin Secretory Effects of a Classic Ketogenic Meal in Healthy Subjects: A Randomized Cross-Over Study.
Battezzati, A, Foppiani, A, Leone, A, De Amicis, R, Spadafranca, A, Mari, A, Bertoli, S
Nutrients. 2023;15(5)
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The ketogenic diet is a dietary regimen providing very low carbohydrate, high fat, and modest protein. Low carbohydrate and ketogenic diets have become increasingly popular in the treatment of metabolic syndrome, obesity, and type 2 diabetes. The main aim of this study was to measure the insulin secretory response to a typical ketogenic meal providing ~40% of individual energy needs and to compare it to the response to an isocaloric Mediterranean meal in healthy subjects. This study is a randomised cross-over study which enrolled twelve healthy subjects (50/50 female/male), adults with an age range of 19–31 years, and with a normal weight. The participants received mixed standardised meals of different compositions on two different days spaced apart by a washout period of 7 days. Each subject consumed two meals of identical energy content but differing in macronutrient composition. Results show that a Mediterranean meal accounting for 40% of daily dietary intake, requires, for its metabolism, the production of 7.8 ± 0.8 times the amount of insulin compared to fasting values, temporarily spiking the insulin secretory rate to 8.9 ± 1.2-fold the basal values. Authors conclude that a ketogenic meal is disposed of with only a minimal insulin secretory response compared to a Mediterranean meal.
Abstract
The classic ketogenic diet (KD) is a high-fat, low-carbohydrate diet that mimics a starvation state with sufficient caloric intake to sustain growth and development. KD is an established treatment for several diseases, and it is currently evaluated in the management of insulin-resistant states, although insulin secretion after a classic ketogenic meal has never been investigated. We measured the insulin secretion to a ketogenic meal in 12 healthy subjects (50% females, age range 19-31 years, BMI range 19.7-24.7 kg/m2) after cross-over administrations of a Mediterranean meal and a ketogenic meal both satisfying ~40% of an individual's total energy requirement, in random order and separated by a 7-day washout period. Venous blood was sampled at baseline and at 10, 20, 30, 45, 60, 90, 120, and 180 min to measure glucose, insulin, and C-peptide concentrations. Insulin secretion was calculated from C-peptide deconvolution and normalized to the estimated body surface area. Glucose, insulin concentrations, and insulin secretory rate were markedly reduced after the ketogenic meal with respect to the Mediterranean meal: glucose AUC in the first OGTT hour -643 mg × dL-1 × min-1, 95% CI -1134, -152, p = 0.015; total insulin concentration -44,943 pmol/L, 95% CI -59,181, -3706, p < 0.001; peak rate of insulin secretion -535 pmol × min-1 × m-2, 95% CI -763, -308, p < 0.001. We have shown that a ketogenic meal is disposed of with only a minimal insulin secretory response compared to a Mediterranean meal. This finding may be of interest to patients with insulin resistance and or insulin secretory defects.
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Growth pattern trajectories in boys with Duchenne muscular dystrophy.
Stimpson, G, Raquq, S, Chesshyre, M, Fewtrell, M, Ridout, D, Sarkozy, A, Manzur, A, Ayyar Gupta, V, De Amicis, R, Muntoni, F, et al
Orphanet journal of rare diseases. 2022;(1):20
Abstract
OBJECTIVES The objective of this study is to analyse retrospective, observational, longitudinal growth (weight, height and BMI) data in ambulatory boys aged 5-12 years with Duchenne muscular dystrophy (DMD). BACKGROUND We considered glucocorticoids (GC) use, dystrophin isoforms and amenability to exon 8, 44, 45, 51 and 53 skipping drug subgroups, and the impact of growth on loss of ambulation. We analysed 598 boys, with 2604 observations. This analysis considered patients from the UK NorthStar database (2003-2020) on one of five regimes: "GC naïve", "deflazacort daily" (DD), "deflazacort intermittent" (DI), "prednisolone daily" (PD) and "prednisolone intermittent" (PI). A random slope model was used to model the weight, height and BMI SD scores (using the UK90). RESULTS The daily regime subgroups had significant yearly height stunting compared to the GC naïve subgroup. Notably, the average height change for the DD subgroup was 0.25 SD (95% CI - 0.30, - 0.21) less than reference values. Those with affected expression of Dp427, Dp140 and Dp71 isoforms were 0.77 (95% CI 0.3, 1.24) and 0.82 (95% CI 1.28, 0.36) SD shorter than those with Dp427 and/or Dp140 expression affected respectively. Increased weight was not associated with earlier loss of ambulation, but taller boys still ambulant between the age of 10 and 11 years were more at risk of losing ambulation. CONCLUSION These findings may provide further guidance to clinicians when counselling and discussing GCs commencement with patients and their carers and may represent a benchmark set of data to evaluate the effects of new generations of GC.
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Ultra-processed foods and obesity and adiposity parameters among children and adolescents: a systematic review.
De Amicis, R, Mambrini, SP, Pellizzari, M, Foppiani, A, Bertoli, S, Battezzati, A, Leone, A
European journal of nutrition. 2022
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Ultra-processed foods (UPFs) are mostly or entirely lacking whole foods and fibre and are often high in fat sugar and salt. The consumption of UPFs may be linked to obesity in adolescents and this systematic review and meta-analysis aimed to synthesis the current research investigating this link. The results showed that over the long-term, the consumption of UPFs was associated with obesity, abdominal obesity, and increased body mass index in children. It was concluded that the long-term consumption of UPFs negatively impacts body composition in children and adolescents. This study could be used by healthcare professionals to understand the importance of dietary advice recommending whole foods with limited or no processed foods for the healthy body development of children.
Abstract
PURPOSE According to the NOVA classification, ultra-processed foods are products made through physical, biological and chemical processes and typically with multiple ingredients and additives, in which whole foods are mostly or entirely absent. From a nutritional point of view, they are typically energy-dense foods high in fat, sugar, and salt and low in fiber. The association between the consumption of ultra-processed food and obesity and adiposity measurements has been established in adults. However, the situation remains unclear in children and adolescents. METHODS We carried out a systematic review, in which we summarize observational studies investigating the association between the consumption of ultra-processed food, as defined by NOVA classification, and obesity and adiposity parameters among children and adolescents. A literature search was performed using PUBMED and Web of Science databases for relevant articles published prior to May 2021. RESULTS Ten studies, five longitudinal and five cross-sectional, mainly conducted in Brazil, were included in this review. Four longitudinal studies in children with a follow-up longer than 4 years found a positive association between the consumption of ultra-processed food and obesity and adiposity parameters, whereas cross-sectional studies failed to find an association. CONCLUSION These data suggest that a consistent intake of ultra-processed foods over time is needed to impact nutritional status and body composition of children and adolescents. Further well-designed prospective studies worldwide are needed to confirm these findings considering country-related differences in dietary habits and food production technologies.
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Systematic Review on the Potential Effect of Berry Intake in the Cognitive Functions of Healthy People.
De Amicis, R, Mambrini, SP, Pellizzari, M, Foppiani, A, Bertoli, S, Battezzati, A, Leone, A
Nutrients. 2022;(14)
Abstract
The increase in life expectancy poses health challenges, such as increasing the impairment of cognitive functions. Berries show a neuroprotective effect thanks to flavonoids, able to reduce neuroinflammatory and to increase neuronal connections. The aim of this systematic review is to explore the impact of berries supplementation on cognitive function in healthy adults and the elderly. Twelve studies were included for a total of 399 participants, aged 18-81 years (mean age: 41.8 ± 4.7 years). Six studies involved young adults (23.9 ± 3.7 years), and four studies involved the elderly (60.6 ± 6.4 years). Most studies investigated effects of a single berry product, but one used a mixture of 4 berries. Non-significant differences were detected across cognition domains and methodologies, but significant and positive effects were found for all cognitive domains (attention and concentration, executive functioning, memory, motor skills and construction, and processing speed), and in most cases they were present in more than one study and detected using different methodologies. Although some limitations should be taken into account to explain these results, the positive findings across studies and methodologies elicit further studies on this topic, to endorse the consumption of berries in healthy populations to prevent cognitive decline.
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The PURPLE N study: objective and perceived nutritional status in children and adolescents with cerebral palsy.
Fogarasi, A, Fazzi, E, Smorenburg, ARP, Mazurkiewicz-Beldzinska, M, Dinopoulos, A, Pobiecka, A, Schröder-van den Nieuwendijk, D, Kraus, J, Tekgül, H, , , et al
Disability and rehabilitation. 2022;(22):6668-6675
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PURPOSE To obtain information on characteristics, management, current objective nutritional status and perception of nutritional status of children with cerebral palsy (CP) from healthcare professionals (HCPs) and caregivers. MATERIALS AND METHODS A detailed survey of several items on eight main topics (general characteristics, motor function, comorbidities, therapies, anthropometry, feeding mode and problems and perceived nutritional status) was developed and tested for the study. Correlation between nutritional status and Gross Motor Function Classification System (GMFCS) levels was assessed using continuous variables (Z-scores for weight-for-age, height-for-age, weight-for-height, and body mass index-for-age), and categorical variables (being malnourished, stunted, or wasted). HCP and caregiver perceptions of the child's nutritional status as well as agreement between perceived and objective nutritional status and agreement between perceived nutritional status and concerns about the nutritional status were analyzed. RESULTS Data were available for 497 participants from eight European countries. Poorer nutritional status was associated with higher (more severe) GMFCS levels. There was minimal agreement between perceived and objective nutritional status, both for HCPs and caregivers. Agreement between HCP and caregiver perceptions of the child's nutritional status was weak (weighted kappa 0.56). However, the concerns about the nutritional status of the child were in line with the perceived nutritional status. CONCLUSIONS The risk of poor nutritional status is associated with more severe disability in children and adolescents with CP. There is a mismatch between HCP and caregiver perceptions of participants' nutritional status as well as between subjective and objective nutritional status. Our data warrant the use of a simple and objective screening tool in daily practice to determine nutritional status in children and adolescents with CP. Clinical trial registration: ClinicalTrials.gov Identifier: NCT03499288 (https://clinicaltrials.gov/ct2/show/NCT03499288). IMPLICATIONS FOR REHABILITATIONUse of the ESPGHAN recommendations and simple screening tools in daily practice is needed to improve nutritional care for individuals with CP.Attention should be paid to the differences in the perception of nutritional status of individuals with CP between professionals and caregivers to improve appropriate referral for nutritional support.Objective measures rather than the professional's perception need to be used to define the nutritional status of individuals with CP.
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Growth patterns in children with spinal muscular atrophy.
De Amicis, R, Baranello, G, Foppiani, A, Leone, A, Battezzati, A, Bedogni, G, Ravella, S, Giaquinto, E, Mastella, C, Agosto, C, et al
Orphanet journal of rare diseases. 2021;(1):375
Abstract
BACKGROUND Spinal muscular atrophy (SMA) is a neuromuscular disorder characterized by muscle atrophy and weakness. SMA type 1 (SMA1) is the most severe form: affected infants are unable to sit unaided; SMA type 2 (SMA2) children can sit, but are not able to walk independently. The Standards of Care has improved quality of life and the increasing availability of disease-modifying treatments is progressively changing the natural history; so, the clinical assessment of nutritional status has become even more crucial. Aims of this multicenter study were to present the growth pattern of treatment-naïve SMA1 and SMA2, and to compare it with the general growth standards. RESULTS Body Weight (BW, kg) and Supine Length (SL, cm) were collected using a published standardized procedure. SMA-specific growth percentiles curves were developed and compared to the WHO reference data. We recruited 133 SMA1 and 82 SMA2 (48.8% females). Mean ages were 0.6 (0.4-1.6) and 4.1 (2.1-6.7) years, respectively. We present here a set of disease-specific percentiles curves of BW, SL, and BMI-for-age for girls and boys with SMA1 and SMA2. These curves show that BW is significantly lower in SMA than healthy peers, while SL is more variable. BMI is also typically lower in both sexes and at all ages. CONCLUSIONS These data on treatment-naïve patients point toward a better understanding of growth in SMA and could be useful to improve the clinical management and to assess the efficacy of the available and forthcoming therapies not only on motor function, but also on growth.
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Patients with Severe Obesity during the COVID-19 Pandemic: How to Maintain an Adequate Multidisciplinary Nutritional Rehabilitation Program?
De Amicis, R, Cancello, R, Capodaglio, P, Gobbi, M, Brunani, A, Gilardini, L, Castenuovo, G, Molinari, E, Barbieri, V, Mambrini, SP, et al
Obesity facts. 2021;(2):205-213
Abstract
BACKGROUND The COVID-19 pandemic is spreading all over the world, particularly in developed countries where obesity is also widespread. There is a high frequency of increased BMI in patients admitted to intensive care for SARS-CoV-2 infection with a major severity in patients with an excess of visceral adiposity. Patients at risk of severe SARS-CoV-2 acute respiratory syndrome are characterised by the high prevalence of pre-existing diseases (high blood pressure and cardiovascular disease, diabetes, chronic respiratory disease, or cancer), most of them typically present in severely obese patients. Indeed, the biological role of adipose tissue in sustaining SARS-CoV-2 infection is not completely elucidated. SUMMARY The forced isolation due to pandemic containment measures abruptly interrupted the rehabilitation programs to which many patients with severe obesity were enrolled. People affected by obesity, and especially those with severe obesity, should continue clinical rehabilitation programs, taking extra measures to avoid COVID-19 infection and reinforcing the adoption of preventive procedures. In this review, the available data on obesity and COVID-19 are discussed along with evidence-based strategies for maintaining the necessary continuous rehabilitation programs. Key Messages: Greater attention is needed for obese and severely obese patients in the face of the current COVID-19 pandemic, which represents a huge challenge for both patients and healthcare professionals. The adoption of new strategies to guarantee adequate and continuous multidisciplinary nutritional rehabilitation programs will be crucial to control the severity of SARS-CoV-2 infection in high-risk populations as well as the worsening of obesity-linked complications. Health authorities should be urged to equip hospitals with tools for the diffusion of telemedicine to maintain physician-patient communication, which is fundamental in chronic and complicated obese patients.