Short-term effects of Mediterranean diet on nutritional status in adults affected by Osteogenesis Imperfecta: a pilot study.

BACKGROUND: Osteogenesis Imperfecta (OI) is a heterogeneous group of connective tissue disorders, characterized by varying degrees of skeletal fragility. Patients experience a range of comorbidities, such as obesity, cardiovascular, and gastrointestinal complications, especially in adulthood. All aspects that could benefit from dietary intervention. The aim of this study was to evaluate the effects of a 6-months restricted Mediterranean Diet (rMD) on nutritional status in adult patients affected by OI. We carried out a 6-months longitudinal pilot study. 14 adults (median age: 35 years; 7 women; 7 OI type III) where recruited in 2019 among the members of As.It.O.I., the Italian Association of Osteogenesis Imperfecta. As.It.O.I. All the evaluations were performed at the University of Milan, Italy. The rMD provided a reduction of 30% from daily total energy expenditure. 45% of calories derived from carbohydrates, 35% from fat and 0.7-1.0 g/kg of body weight from proteins. Comparisons of continuous variables after 6 months of intervention were performed by the paired t-test. All P-values were two-tailed, and p < 0.05 was considered significant. RESULTS: Patients showed significant improvement in anthropometric measurements (BMI = 30.5 vs 28.1 kg/cm2, p < 0.001; Body Fat % = 32.9 vs 29.9, p = 0.006; Waist circumferences = 83.6 vs 79.6 cm; p < 0.001; Arm Fat Area = 29.8 vs 23.07 cm2; p < 0.011) and energy expenditure (REE/kg = 27.2 vs 29.2 kcal/kg, p < 0.001). Glucose and lipid profiles improved (Δglycemia = - 8.6 ± 7.3 mg/dL, p = 0.003; ΔTC = - 14.6 ± 20.1 mg/dL, p = 0.036; ΔLDL = - 12.0 ± 12.1 mg/dL, p = 0.009). Adherence to the MD significantly increased, moving from a moderate to a strong adherence and reporting an increased consumption of white meat, legumes, fish, nuts, fruits and vegetables. CONCLUSION: A rMD was effective in improving nutritional status and dietary quality in adults with OI. These results underscores the need to raise awareness of nutrition as part of the multidisciplinary treatment of this disease.

Sex differences in cardiometabolic risk factors and in response to lifestyle intervention in prepubertal and pubertal subjects with obesity.

Objectives: Childhood obesity is a growing health problem and requires a tailored treatment. This study explored the sex differences in cardiovascular risk factors in children/adolescents with obesity and in response to a weight loss intervention. Methods: Five hundred and thirty-three children/adolescents with obesity and their parents underwent to a 3-months lifestyle intervention program. Tanner criteria were used to assess the pubertal stage. Before and after 3 months, anthropometric measurements, blood pressure (BP), and biochemical measurements were assessed. Results: Four hundred and forty five participants completed the treatment (age 12.4 ± 2.7 years, males 45.8%, prepubertal 29.2%, BMI z score 2.3 ± 0.2). In comparison to boys, prepubertal girls had higher values of BMI z score (2.4 ± 02 vs. 2.3 ± 0.2, p < 0.05), waist circumference z score (2.2 ± 0.3 vs. 2.0 ± 0.3, p < 0.05), HOMA-IR [2.9 (2.1-4.9) vs. 2.3(1.5-3.6), p < 0.01], prevalence of hypertransaminasemia (41.3% vs. 17.7%, p < 0.05) and lower levels of HDL cholesterol (46.2 ± 9.8 vs. 51.2 ± 10.5 mg/dl, p < 0.05). In the pubertal stage, boys had worse cardiometabolic risk profile than girls, including unfavourable measure of systolic BP (z score: 0.6 ± 1.0 vs. 0.3 ± 1.0, p < 0.01), fasting glucose (87.2 ± 6.1 vs. 84.8 ± 7.7 mg/dl, p < 0.01), ALT (26.9 ± 21.5 vs. 20.2 ± 10.6 U/L, p < 0.001) and uric acid (6.1 ± 1.9 vs. 5.0 ± 1.0 mg/dl, p < 0.001). After the lifestyle intervention, changes in BMI z score (p < 0.05) were higher in pubertal boys than pubertal girls. The systolic blood pressure decrease was greater in pubertal boys than in their female counterpart (Δ systolic BP: -7.2 mmHg in boys vs. -3.6 mmHg in girls, p < 0.05; Δ systolic BP z score: -0.6 in boys vs. -0.3 in girls, p < 0.05). LDL cholesterol showed an improvement only in boys, and ALT in the whole group. Conclusion: Our study showed that a short-term lifestyle intervention is more effective in reducing BMI z score and cardiovascular risk factors in pubertal boys than in their female counterparts. Further investigation is needed to deepen this gender difference, especially to develop a tailor-made intervention.

Elucidating the role of Pyroptosis in papillary thyroid cancer: prognostic, immunological, and therapeutic perspectives.

BACKGROUND: Pyroptosis, an inflammatory form of programmed cell death, has been implicated in the pathogenesis and progression of several cancers. However, the significance of pyroptosis-related genes (PRGs) in papillary thyroid cancer (PTC) remains unclear. METHODS: Transcriptome and clinical data of PTC patients were obtained from The Cancer Genome Atlas. The expression patterns of PRGs were identified by consensus clustering. A prognostic model for predicting the thyroid cancer-free interval (TCFi) employed five machine learning methods. Enrichment and immune-related analyses were performed to elucidate the role of pyroptosis. The responses to radioactive iodine (RAI), immune checkpoint inhibitors (ICIs), molecular targeted therapy (MTT), and chemotherapy (CTx) were predicted based on pyroptosis-derived features. Additionally, the expression of prognostic PRGs was validated via six external datasets, 16 cell lines, and 20 pairs of clinical samples. RESULTS: PTC patients were classified into three PyroClusters, C1 exhibited BRFA-like tumors with the highest invasiveness and the worst prognosis, C2 presented RAS-like tumors, and C3 was characterized by gene fusion. Nine PRGs (CXCL8, GJA1, H2BC8, IFI27, PRDM1, PYCARD, SEZ6L2, SIGLEC15, TRAF6) were filtered out to construct a PyroScore prognostic model. A derived nomogram demonstrated superior predictive performance than four clinical staging systems. A strong correlation between pyroptosis and tumor immune microenvironment (TIME) remodeling was observed in mechanistic analyses. Patients with a high PyroScore exhibited "hot" tumor immunophenotypes and had a poorer prognosis but could benefit more from ICIs and CTx (such as paclitaxel). Patients with a low PyroScore were more sensitive to RAI and MTT (such as pazopanib and sorafenib). CONCLUSIONS: PyroScore model can effectively predict TCFi in patients with PTC. Dysregulated expression of PRGs is associated with the TIME modeling. Pyroptosis features have potential significance for developing novel therapeutic strategies for PTC patients.

A novel model for predicting diabetes remission after bariatric surgery based on the measurement of C-peptide and creatinine in serum: A pilot study.

BACKGROUND AND AIMS: Bariatric surgery is effective for treating type 2 diabetes (T2D) in patients with obesity, although a significant proportion of these patients do not achieve diabetes remission after the surgery even after significant weight loss and metabolic improvement. C-peptide is a valuable marker of beta cell function and insulin secretion, but renal function must be considered when interpreting measurements in patients with T2D. The study aims to investigate the association of serum levels of C-peptide adjusted for creatinine with diabetes remission and glycemic target achievement after bariatric surgery in patients with obesity and T2D. METHODS AND RESULTS: Prospective data from a cohort of 84 patients with obesity and T2D submitted to Roux-en-Y gastric bypass (RYGB) were collected at baseline and at least a 6-month follow up. A multivariate binomial regression model showed that Ln(C-peptide/creatinine) and age were significantly associated with 6-month T2D remission. The area under the curve for the receiver operating characteristic analysis (AUROC) to predict remission was 0.87, and more accurate than the AUROC based on C-peptide levels alone (0.75). The same model was also able to predict achieving an HbA1c target of 7 % (53 mmol/mol) (AUROC 0.96). CONCLUSION: In conclusion, Ln(C-peptide/creatinine) ratio could be a useful tool in predicting T2D remission and target achievement after RYGB surgery, providing a more accurate reflection of beta cell function in bariatric patients.

The usefulness of Integrative Neuromuscular Training to counteract obesity: a narrative review.

BACKGROUND AND OBJECTIVE: The association between physical activity and diet has a valuable impact in weight status management to counteract obesity. In this context, within different training strategies (i.e., endurance, resistance training, concurrent training, agility training) the Integrative Neuromuscular Training (INT) represents a structured training mode focused on global human movement pattern development with the aim to enhance motor control, mobility and stability. In this narrative review we aimed to discuss the feasibility of INT interventions on physical fitness and body composition outcomes in individuals with obesity. SUBJECTS: Medline/PubMed, EMBASE, Web of Science, Google Scholar including were searched before 1st February 2023 without restrictions on publication year. METHODS: Two researchers extracted data from published trials. Randomized controlled trials or clinical trials, Body Mass Index of children and adolescents at the 95% percentile or greater, and for adults to be above 30 kg/m2, detailed intervention description, randomization process and allocation into an experimental or a control group, trials must have been written in English, were included. RESULTS: We included a total of 19 studies complying with the inclusion criteria for the review process. There is evidence that INT promotes positive adaptations in fitness levels in both younger and older participants with concomitant ameliorations during a shorter, medium and longer time period. Moreover, cardiorespiratory fitness, muscular strength, balance, postural control and body composition reached significant remarkable improvements following a specific intervention based on INT principles compared to other training mode. However, Body Mass Index, fat mass percentage and waist circumference showed similar changes overtime. CONCLUSIONS: Taken together, these findings support the effectiveness of INT in ameliorating physical fitness (i.e., health-related and skill related components) without negative changes in body composition. Nevertheless, fitness coaches and therapists may consider this training modality a feasible option when prescribing physical exercise in outpatients with obesity.

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.

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.

Sex Differences in the Relationship between Chronotype and Eating Behaviour: A Focus on Binge Eating and Food Addiction.

BACKGROUND: Men are more likely than women to have subthreshold overeating disorders. Lifestyle plays a role as a determinant, while chronotype is an emerging factor. Chronotype explains the natural preferences of wakefulness and activity throughout the day: evening chronotypes (E-Types), those most productive in the evening, have been linked with unhealthy dietary patterns and a higher propensity to substance addiction than morning types (M-Types). METHODS: We carried out a cross-sectional study on 750 overweight or obese adults (70% females, 48 ± 10 years, BMI 31.7 ± 5.8 kg/m2). The Binge-Eating Scale, the Yale Food Addiction Scale 2.0 (YFAS 2.0), the reduced Morningness-Eveningness Questionnaire (rMEQ), and the MEDAS questionnaire were used to assess binge eating, food addiction, chronotype, and adherence to the Mediterranean diet, respectively. RESULTS: No differences in BES binge-eating and FA food-addiction scores occurred between chronotypes, but we found significant interactions between sex × rMEQ score. While women showed the same prevalence for binge eating and food addiction across all chronotypes, binge eating and food addiction risk increased with reducing rMEQ score in men, indicating that being male and E-Type increases the risk association of binge eating and/or food addiction prevalence. CONCLUSIONS: chronotype is associated with binge eating and food addiction in men, emphasizing the link between chronobiology and sex differences as determinants in appetite and eating behaviour dysregulation and in overweight and obesity.

Use of Branched-Chain Amino Acids as a Potential Treatment for Improving Nutrition-Related Outcomes in Advanced Chronic Liver Disease.

Advanced chronic liver disease (ACLD) represents a complex and multifactorial clinical entity characterized by liver dysfunction and associated complications. In recent years, the significance of nutritional status in ACLD prognosis has gained considerable attention. This review article delves into the multifactorial pathogenesis of malnutrition in ACLD and its profound consequences for health outcomes. We explore the clinical implications of secondary sarcopenia in ACLD and highlight the critical relevance of frailty in both decompensated and compensated ACLD. A specific focus of this review revolves around branched-chain amino acids (BCAAs) and their pivotal role in managing liver disease. We dissect the intricate relationship between low Fischer's ratio and BCAA metabolism in ACLD, shedding light on the molecular mechanisms involved. Furthermore, we critically evaluate the existing evidence regarding the effects of BCAA supplementation on outcomes in ACLD patients, examining their potential to ameliorate the nutritional deficiencies and associated complications in this population.

Impact of 4-week of a restricted Mediterranean diet on taste perception, anthropometric, and blood parameters in subjects with severe obesity.

Introduction: The study of taste functionality and its relation to human health is receiving growing attention. Obesity has been reported to cause alterations in sensory perception regarding system functionality and preferences. However, a small body of research addresses tastes perception and its modification with the achievement of body mass reduction through surgical intervention. Much fewer efforts have been made to evaluate the impact of mild restrictive nutritional intervention on gustatory functions. Thus, the objectives of this study were to determine if a dietary intervention of 4 weeks following a restricted balanced Mediterranean diet would affect the sweet and salty taste thresholds of subjects with severe obesity and could influence their anthropometric and blood parameters. Methods: Fifty-one patients with severe obesity (F: 31; age: 43.7 ± 12.5; BMI = 47.6 ± 1.0) were enrolled in the study. The recognition threshold for sweet and salty taste and anthropometric and blood parameters were assessed before and after the 4-week weight loss program. Results and Discussion: The Mediterranean diet has proven to be an effective treatment, significantly improving all anthropometric and blood parameters (p < 0.05) after 4 weeks of intervention. Moreover, the hypo-sodium treatment associated with the diet significantly improved the salty threshold (p < 0.001). No changes were detected for the sweet threshold. Collectively, these data highlight that dietary treatment might impact taste perception differently. Therefore, a taste-oriented nutritional intervention could represent a novel approach to developing more individualized, taste-oriented follow-up interventions to maintain sustainable and long-term weight loss.

Development of a questionnaire on nutritional knowledge for the obese hospitalized patient: the NUTRIKOB questionnaire.

Introduction: Different approaches, involving different areas and figures, are useful for the rehabilitation of obese subjects through a multidisciplinary hospital path. A focal point of rehabilitation is represented by education on healthy eating by increasing the dietary knowledge patients. Few tools investigating food knowledge are available in Italy: therefore, the need has emerged to develop easy-to-use tools for clinical practice that allow to detect food knowledge to set up a more targeted food re-education. The following work aimed at building and validating a questionnaire capable of investigating the dietary knowledge of the population affected by obesity. Methods: A pool of experts carried out a review of the literature, gathering all the information necessary to select and construct the best set of questions and the format of the final project of the questionnaire. During statistical analysis the validity, reproducibility and stability of the questionnaire were investigate in a sample of 450 subjects with obesity. Results: Early analysis disclosed that 5 questions of the original questionnaire had no discriminating power. The successive validation phases were successful, confirming good content validity, stability and reproducibility over time. Discussion: The questionnaire has all the characteristics to be considered a valid tool for investigating dietary knowledge in the obese population. The psychometric tests confirmed a good internal consistency of the structure, a validity of the content, a good reproducibility and stability over time.

Pharmacotherapy for obstructive sleep apnea: targeting specific pathophysiological traits.

INTRODUCTION: The pathophysiology of obstructive sleep apnea (OSA) is multi-factorial and complex. Varying OSA's pathophysiological traits have been identified, including pharyngeal collapsibility, upper airway muscle reactivity, arousal threshold, and regulation of the ventilatory drive. Being CPAP of difficult tolerance and other interventions reserved to specific subpopulations new pharmacological treatments for OSA might be resolutive. AREAS COVERED: Several existing and newly developed pharmacological drugs can impact one or more endotypes and could therefore be proposed as treatment options for sleep disordered breathing. With this review we will explore different pathophysiological traits as new targets for OSA therapy. This review will summarize the most promising pharmacological treatment for OSA accordingly with their mechanisms of action on upper airway collapsibility, muscle responsiveness, arousal threshold, and loop gain. EXPERT OPINION: Only understanding the pathophysiological traits causing OSA in each patient and placing the disease in the framework of patient comorbidities, we will be able to evolve interventions toward OSA. The development of new drug's combinations will permit different approaches and different choices beside conventional treatments. In the next future, we hope that sleep specialists will select the treatment for a specific patient on the base of its pathophysiology, defining a precision medicine for OSA.

Long-term follow-up of nutritional status in children with GLUT1 Deficiency Syndrome treated with classic ketogenic diet: a 5-year prospective study.

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.

Ultra-Processed Food Consumption and Incidence of Obesity and Cardiometabolic Risk Factors in Adults: A Systematic Review of Prospective Studies.

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.

Antioxidant potential of the diet in Italian children with food allergies.

A reduced fruit and vegetable consumption, which implies a decreased intake of antioxidant compounds, seems to play a role in allergic diseases onset. Data on the antioxidant capacity of diet in children with food allergies, who are on an avoidance diet, are still lacking. This pilot study aims to assess the antioxidant potential of diet in Italian children with food allergies, compared to healthy children, using the oxygen radical absorbance capacity (ORAC) method. 95 children (54 with confirmed food allergies and 41 controls), with a median age of 7.8 years, were enrolled and underwent a nutritional assessment. Mean nutrient intakes were compared using the Mann-Whitney test. ORAC resulted significantly lower in allergic children (median 2,908, IQR: 1450;4,716) compared to control children (median 4,392, IQR: 2523;5,836; p = 0.049). Among micronutrients with antioxidant properties, vitamin A intakes were significantly higher in controls than in allergic children. Using Spearman's correlation, a moderate-to-strong correlation between ORAC and vitamin C, potassium and magnesium was observed (ρ = 0.648, p < 0.001; ρ = 0.645, p < 0.001; ρ = 0.500, p < 0.001, respectively). Iron, phosphorus, vitamin E and vitamin A intakes were also moderately-to-low correlated with ORAC values (ρ = 0.351, p < 0.001; ρ = 0.367, p < 0.001; ρ = 0.346, p < 0.001; and ρ = 0.295, p = 0.004, respectively). We hypothesize that the reduced antioxidant potential of the diet might be related to a reduced variety of the diet in children with food allergies. Our study suggests that the diet of children with food allergies has a lower antioxidant potential (expressed as ORAC value) compared to the diet of healthy children, regardless of the allergenic food excluded from the diet. This issue should be further investigated in prospective, powered studies.

Acute Insulin Secretory Effects of a Classic Ketogenic Meal in Healthy Subjects: A Randomized Cross-Over Study.

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.

Reply to Alvarez Stehle, E. Comment on "Leone et al. Association between Mediterranean Diet and Fatty Liver in Women with Overweight and Obesity. Nutrients 2022, 14, 3771".

We thank Dr. Elvira Alvarez Stehle [...].

Nutritional status of children affected by X-linked adrenoleukodystrophy.

BACKGROUND: Adrenoleukodystrophy (ALD) is a rare X-linked metabolic disorder that causes the accumulation of very-long-chain fatty acids (VLCFAs) (C26:0) and the subsequent variety of clinical and neurological symptoms. Little is known about nutritional status and dietary habits of children affected by ALD, and so the present study aimed to assess nutritional status and food intake in children with ALD, also exploring the relationship between food intake and the consumption of disease-specific dietary supplements to reduce blood C26:0 concentrations and increase monounsaturated fatty acids (C26:1). METHODS: All patients underwent a clinical and neurological evaluation and a comprehensive nutritional assessment. The association of VLCFA concentrations with dietary lipids was assessed. RESULTS: Nine boys (11.49 ± 3.61 years) were enrolled in a cross-sectional study. All patients were normal weight, with normal resting energy expenditure. Only six of nine patients followed the low-fat diet and dietary supplements. An inverse association was found between the food intake of polyunsaturated lipids and C26:0; conversely, the C26:0 was positively associated with the dietary saturated lipids. When consumed, dietary supplement consumption correlated positively with C26:1 (ρ = 0.917, p = 0.029) and no correlation was found with C26:0 (ρ = 0.410, p = 0,493). CONCLUSIONS: No children were found to be malnourished or overweight or obese; however, half of the children reported excessive body fat, probably as a result of the pharmacotherapies. A low-fat diet could be adjuvant in the management of the accumulation of VLCFAs, but poor dietary compliance to disease-specific nutritional guidelines appears to be a major problem of this condition and underlines the need for a structured and personalised nutritional management in ALD disease.

What drives patients' acceptance of Digital Therapeutics? Establishing a new framework to measure the interplay between rational and institutional factors.

BACKGROUND: The rising incidence of chronic diseases among the population, further exacerbated by the phenomenon of aging, is a primary concern and a serious challenge for the healthcare systems worldwide. Among the wide realm of health digital technologies, the rise of Digital Therapeutics (DTx), which are medical devices able to deliver evidence-based treatments to manage and treat diseases, opens new opportunities. However, their diffusion and usage are still fragmented among countries. As the diffusion results from the adoption of technology from a social system and individual acceptance, this study aims to design and test a theoretical model that investigates the intention to use DTx, with a particular focus on the treatment of obesity, as a widespread and burdensome chronic condition. METHODS: This research is built on 336 answers coming from a survey to test the proposed model, which consists of a combination of organizational mechanisms, derived from Institutional Theory, and rational factors, derived from the Technology Acceptance Model (TAM). The survey has been delivered to patients and former patients of Istituto Auxologico Italiano, a hospital with several locations in northern Italy, recognized as a center of excellence for the treatment of obesity. RESULTS: The analyses of the answers, performed through the Structural Equation Modelling (SEM) technique, confirmed the influence of the Perceived Usefulness on Intention To Use, and of the Perceived Ease Of Use on the Perceived Usefulness, confirming the validity of the assumptions derived from the TAM. On the other hand, institutional factors were introduced as antecedents of the Perceived Usefulness, and the Perceived Ease Of Use. Results show that the Regulative Pillar influences both the TAM constructs, the Normative Pillar (peer influence) has a positive effect only on the Perceived Usefulness, and finally, the Cultural Pillar impacts the Perceived Ease Of Use. CONCLUSION: This study allows filling the knowledge gap regarding the usage of the Institutional as a means to predict individuals' intentions. Moreover, managerial contributions are available as the results have been operationalized into practical advice to managers and healthcare professionals to foster the adoption, and thus the diffusion, of Digital Therapeutics.

Longitudinal Anthropometry and Body Composition in Children With SARS-CoV-2-Associated Multisystem Inflammatory Syndrome.

OBJECTIVES: Acute coronavirus disease 2019 infection has been shown to negatively affect body composition among adult and malnourished or obesity children. Our aim is to longitudinally evaluate body composition in children affected by the Multisystem Inflammatory Syndrome (MIS-C). METHODS: In this cohort study, we recruited 40 patients affected by MIS-C, aged 2-18 years old, who were admitted in our clinic between December 2020 and February 2021. Physical examination for each participant included weight, height, body mass index (BMI) z score, circumferences, and skinfolds assessment. The same measurements were repeated during outpatient follow-up at 10 (T2), 30 (T3), 90 (T4), and 180 (T5) days after hospital discharge. Fat mass and fat free mass were calculated according to skinfolds predictive equations for children and adolescents. A control group was randomly selected among patients attending a pediatric nutritional outpatient clinic. RESULTS: BMI z score significantly decrease between preadmission and hospital discharge. Similarly, arm circumference z score, arm muscular area z score, and arm fat area z score significantly decreased, during hospital stay. Fat mass index (FMI) significantly increased over time, peaking at T3. Fat free mass index decreased during hospitalization. CONCLUSIONS: To the best of our knowledge, this is the first study to assess body composition in a numerically large pediatric MIS-C population from acute infection to 6 months after triggering event. FMI and anthropometric parameters linked to fat deposits were significantly higher 6 months after acute event. Thus, limiting physical activity and having sedentary lifestyle may lead to an accumulation of adipose tissue even in healthy children who experienced MIS-C and long hospitalization.