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.

Ultra-processed foods and obesity and adiposity parameters among children and adolescents: a systematic review.

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.

Isocaloric Ketogenic Diet in Adults with High-Grade Gliomas: A Prospective Metabolic Study.

The use of the ketogenic diet (KD) as an adjuvant therapy in high-grade gliomas (HGG) is supported by preclinical studies, but clinical data on its effects on metabolism are currently lacking. In this study, we describe the effects of a KD on glucose profile, ketonemia, energy metabolism, and nutritional status, in adults affected by HGG. This was a single-arm prospective study. An isocaloric 3:1 KD was administered for 1 mo. Glucose profile was assessed by using fasting glycemia, insulin, and glycated hemoglobin. To evaluate ketonemia changes, a hand-held ketone meter was used from home. Energy metabolism was assessed by indirect calorimetry. Nutritional status was evaluated through changes in body composition and in lipid and hepatic profile. No changes in fasting glycemia were observed; however, insulinemia dropped to half of baseline levels. The KD shifted the metabolism, rising ketonemia and decreasing glucose oxidation rate to a quarter of the initial values. Moreover, the KD was generally safe. One-month intervention with the KD was able to act upon key metabolic substrates potentially involved in HGG metabolism. The lack of a significant reduction in fasting glycemia should be investigated in future studies.

Evaluation of body composition as a potential biomarker in spinal muscular atrophy.

INTRODUCTION: We aimed to investigate the correlation between body composition (BC) and spinal muscular atrophy (SMA)-specific motor function assessments. METHODS: Patients with SMA types I or II, aged 1 to 10 years, were recruited in this cross-sectional study. The protocol included anthropometric measurements, and dual-energy X-ray absoprtiometry to assess fat mass (FM), lean mass (LM), fat-free mass (FFM), FM and FFM indexes (FMI, FFMI), and motor function assessments (Children's Hospital of Philadelphia Infant Test of Neuromuscular Disorders scale for SMAI, and Hammersmith Functional Motor Scale-Expanded for SMAII). RESULTS: Eighty-eight children were included. All had a higher FM percentage than reference values. Motor function was moderately correlated with body mass index (BMI), FFMI, and LMI in SMAI, and weakly correlated with FFMI, LMI, and LM:FM ratio in SMAII. DISCUSSION: BC shows promise as a potential biomarker for SMA, but further studies are needed.

Triheptanoin Supplementation Does not Affect Nutritional Status: A Case Report of Two Siblings With Adult Polyglucosan Body Disease.

Objective: An anaplerotic diet with the odd-chain triglyceride (triheptanoin-C7TG) supplementation was tested as a therapy for Adult Polyglucosan Body Disease (APBD) and is currently being assessed for various metabolic disorders. The aim of this study was to determine any unknown long-term effect of C7TG supplementation on the nutritional status, body composition, resting energy expenditure and biochemical parameters of two siblings with APBD.Methods: Two adult siblings with APBD were treated over a 2-year period with a high fat, low carbohydrate diet, with C7TG oil representing about 30% of the daily caloric intake. We carried out a long-term longitudinal study to determine weight, height, waist circumference; total, intra and extra cellular water by bioimpedance; body fat, lean mass, and bone mineral density by DEXA; resting energy expenditure by indirect calorimeter; glucose and lipid profiles.Results: C7TG supplementation failed to prevent APBD progression, corroborating recent literature. However, long-term C7TG supplementation did not produce any appreciable changes in nutritional status, body composition, resting energy expenditure or biochemical parameters, and no evidence was found of potential adverse effects.Conclusions: Our data suggest that maintenance of C7TG over a 2-year period still leaves a good safety profile in terms of nutritional status, body composition, resting energy expenditure, and biochemical parameters. However further studies involving larger sample sizes, also other diseases, are needed for a deeper understanding of its long-term effects.

Evaluation of Different Adiposity Indices and Association with Metabolic Syndrome Risk in Obese Children: Is there a Winner?

Body shape index (ABSI) and triponderal mass index (TMI) have been recently associated with cardiovascular risk in adults. A cross-sectional study was conducted to evaluate the relationship between different anthropometric adiposity indexes and metabolic syndrome (MetS) in Caucasian obese children and adolescents. Consecutive obese children aged ≥7 years have been enrolled. Anthropometric parameters, body composition (by bioelectrical impedance), and systolic and diastolic blood pressure have been measured. Fasting blood samples have been analyzed for lipids, insulin, glucose. A multivariate logistic regression analyses, with body mass index z-score, waist to height ratio, ABSI z-score, TMI, conicity index as predictors for MetS (IDEFICS and IDF criteria according to age) has been performed. Four hundred and three (179 boys and 224 girls) obese children, aged 7-20 years, have been evaluated. When we explored the joint contribution of each anthropometric and adiposity index of interest and BMIz on the risk of MetS, we found that the inclusion of ABSIz improved the prediction of MetS compared to BMIz alone. ABSI-BMI can be a useful index for evaluating the relative contribution of central obesity to cardiometabolic risk in clinical management of obese children and adolescents.

3D Facial morphology in children affected by spinal muscular atrophy type 2 (SMAII).

OBJECTIVES: The main objective of this study was to assess the three-dimensional facial characteristics of children affected by spinal muscular atrophy (SMA), a severe muscular disorder characterized by hypotonia, areflexia, weakness, and respiratory impairment. MATERIALS/METHODS: Stereophotogrammetric facial scans from 22 SMA type II patients aged 2-7 years were obtained. Data were analysed using both inter-landmark distances and principal component analysis and compared with data collected from matched control subjects. RESULTS: Patients had wider transverse facial diameters, but smaller biocular width. Middle and lower anterior face heights were increased, whereas the mandibular ramus was shorter, with a reduced posterior-to-anterior face height ratio. Facial width-to-length ratio was reduced. In the sagittal plane, mandibular body length, and facial divergence were increased, whereas the gonial angles were decreased. In the horizontal plane, lower facial convexity was greater in patients, whereas mandibular convexity was smaller. Patients had smaller and down-slanted eye fissures, with a larger and more vertically developed nose. LIMITATIONS: This study assessed a relatively small number of patients, due to the rare frequency of SMA type II. CONCLUSIONS/IMPLICATIONS: SMA type II children possess peculiar facial alterations that may be due to the altered muscular activity. As feeding problems may derive also by malocclusion and masticatory muscular alterations, a detailed assessment of the craniofacial individual alterations should be considered in the standards of care of these patients.

Anthropometrics and Body Composition in Adults with High-Grade Gliomas: Effects of Disease-Related Variables.

Nutritional status in adults with high-grade gliomas (HGGs) has been poorly investigated. We studied anthropometrics and fat mass (FM), fat free mass (FFM), and body water in HGGs patients also in relation to disease-related variables. Fifty-one patients (17 III and 34 IV-grade) and fifty-one control group (CG) matched for sex, age, and BMI were enrolled. Neurological scales, anthropometry, bioimpedance, and blood sampling were performed and analyzed according to grade, lesion location, extent of resection, phase of treatment, current steroids and antiepileptic therapy, and age of patient. 41.2% were overweight and 15.7% obese. Compared to the CG, HGGs had similar anthropometrics and body composition. IV-grade, compared to the III, had higher BMI, waist circumference, FM and lower FFM. Only patients during concomitant radio and chemotherapy showed a negative BMI variation from baseline. In conclusion, overnutrition occurs in almost 6 out of 10 HGGs patients and is more relevant in IV-grade. Throughout all the treatment phases, there is a higher risk of weight loss occurred only during concomitant radio and chemotherapy. Body composition showed no specific features compared to controls. These results may assist intervention studies directed towards neurometabolic dietary treatment. Nutritional screening is warranted during the time course of disease.

Association of Binge Eating Behavior with Total and Abdominal Adipose Tissue in a Large Sample of Participants Starting a Weight Loss or Maintenance Program.

Objective: It is not clear whether binge eating (BE) behavior is associated with body composition independently of body mass index (BMI). Our aim has been to evaluate the BMI-independent contribution of BE severity and BE status on the total amount of fat mass and abdominal fat distribution in a large sample of participants.Method: We performed a cross-sectional study among 8524 participants followed at a nutritional center. BMI and waist circumference (WC) were measured, body fat (BF) was estimated by skinfold measurement, and abdominal visceral (VAT) and subcutaneous (SAT) adipose tissues were measured by ultrasonography. BE was assessed using the Binge Eating Scale (BES). The association between the continuous BES score (BE severity) and adiposity was assessed in the whole sample after adjustment for BMI and other confounders. The effect of BE status on adiposity was also assessed by matching binge eaters (BES ≥ 18), for sex, age, and BMI, with non-binge eaters (BES < 18).Results: We found that 17.7% of the participants were binge eaters. Continuous BES score was associated with increasing WC (0.03 cm, 95% confidence interval [CI], 0.02 to 0.05 every 1 BES unit, p < 0.001) and decreasing BF (0.01%, 95% CI, -0.02 to -0.00 every 1 BES unit, p = 0.003). No association was found between BE severity and VAT and SAT. After matching, the BF of binge eaters was 0.29% (95% CI, -0.50 to -0.07, p = 0.01) lower than that of non-binge eaters.Conclusions: Given the very small effect size, BE severity and status are not associated in a biologically meaningful manner with BF content and distribution.

Mediterranean Diet and Cognitive Status in Free-Living Elderly: A Cross-Sectional Study in Northern Italy.

OBJECTIVE: Few data are available on the Italian elderly population with regard to adherence to the Mediterranean diet (MD) and cognitive impairment. Our aim was to investigate adherence to the MD and its association with cognitive function in an Italian urban sample. METHODS: A cross-sectional study of 279 participants aged ≥ 65 years (80 men, 199 women) was carried out at a nutritional center. Adherence to the MD was evaluated using a 14-item questionnaire. Cognitive function was assessed with the Mini-Mental State Examination (MMSE). RESULTS: The clinical and nutritional assessments performed revealed 30.1% to have a dietary pattern in accordance with the MD; 13.6% had suspected or mild cognitive impairment (MMSE score ≤ 23). The MD pattern was associated with a lower risk of cognitive impairment (odds ratio [OR] = 0.39; 95% confidence interval [CI], 0.15-0.99; p = 0.045), as was the consumption of wine (OR = 0.37; 95% CI, 0.16-0.84; p = 0.018) and nuts (OR = 0.30; 95% CI, 0.13-0.69, p = 0.005). No association was found with other food groups. CONCLUSION: A closer adherence to the MD was associated with a better cognitive status. Further cohort studies and randomized controlled trials are warranted.

The face of Glut1-DS patients: A 3D Craniofacial Morphometric Analysis.

Glut1 deficiency syndrome (Glut1-DS) is a neurological and metabolic disorder caused by impaired transport of glucose across the blood brain barrier (BBB). Mutations on the SCL2A1 gene encoding the glucose transporter protein in the BBB cause the syndrome, which encompasses epilepsy, movement disorders, and mental delay. Such variability of symptoms presents an obstacle to early diagnosis. The patients seem to share some craniofacial features, and identification and quantification of these could help in prompt diagnosis and clinical management. We performed a three-dimensional morphometric analysis of the faces of 11 female Glut1-DS patients using a stereophotogrammetric system. Data were analyzed using both inter-landmark distances and Principal Component Analysis. Compared with data collected from age-, sex-, and ethnicity-matched control subjects, common and homogenous facial features were identified among patients, which were mainly located in the mandible and the eyes. Glut1-DS patients had a more anterior chin; their mandibular body was longer but the rami were shorter, with a reduced gonial angle; they had smaller and down-slanted eyes with a reduced intercanthal distance. This study highlights the importance of morphometric analysis for defining the facial anatomical characteristics of the syndrome better, potentially helping clinicians to diagnose Glut1-DS. Improved knowledge of the facial anatomy of these patients can provide insights into their facial and cerebral embryological development, perhaps further clarifying the molecular basis of the syndrome. Clin. Anat. 30:644-652, 2017. © 2017 Wiley Periodicals, Inc.

Stroke and Stroke-Like Episodes: Recurrent Manifestations in GLUT1 Deficiency Syndrome.

BACKGROUND: Since the initial description of glucose transporter-1 deficiency syndrome (Glut1-DS) the phenotype of the condition has expanded, even leading to the recognition of atypical manifestations. We report on eight patients with Glut1-DS who experienced at least one episode of acute focal neurological deficits. METHODS: We conducted a retrospective analysis, collecting clinical, electrophysiological, neuroradiological, and genetic information. We focused in particular on three well-documented cases. RESULTS: Among 42 patients with Glut1-DS, eight individuals aged between six and 38 years presented with an acute onset of neurological disturbances: dysarthria/aphasia, oral dyskinesia, swallowing difficulties, paresthesia, facial palsy, hemi/monoplegia, vomiting, headache, and behavioral disturbances. When performed, magnetic resonance imaging (MRI) revealed signs of venous congestion and hypoperfusion and electroencephalography showed focal contralateral slowing. Deficits were transient in all patients but one. Four patients (50%) were on a ketogenic diet (KD), and two of these patients had lower than usual ketonemia levels during the episode. In two patients, MRI demonstrated the presence of an ischemic brain lesion. CONCLUSIONS: In Glut1-DS, stroke-like episodes are a recurrent manifestation, particularly during early adulthood, and they were reported in 19% of the patients in our cohort. Stroke mimics should be considered a key feature of Glut1-DS, as other paroxysmal disorders. It remains to be established whether a KD can prevent the recurrence of episodes and, if so, at what level of ketosis. Further observations are needed to confirm the correlation between Glut1-DS and ischemic stroke.

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.

The Effect of the Mediterranean Diet on Metabolic Health.

The Mediterranean Diet (MedDiet) is one of the healthiest and most balanced dietary patterns worldwide [...].

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.

The Impact of Nutrition on Cognitive Performance in a Frail Elderly Population Living in Northern Italy.

Objectives: There is increasing evidence for a protective role of nutritional factors on cognitive decline. Many studies have reported a preventive effect of specific dietary patterns, in particular of the Mediterranean diet (MD), on the risk of cognitive decline, but only limited evidence is available about its effects on neuropsychological performance. The aim of this work is to evaluate the relationship between nutrition and cognitive performance in a frail elderly population living in Northern Italy. Methods: In this study we have investigated the impact of the Mediterranean dietary pattern on cognitive performance in a frail elderly population (n = 140). Structural equation modeling was applied to highlight the interrelationship between three cognitive domains: i) Verbal Fluency; ii) Attention/executive function; iii) Memory, and two MD factors: i) Nutrition; ii) Anthropometric/physical activity. In addition, the Multiple Correspondence Analysis was performed to detect the food and nutrients with the highest association with MD and nutritional status. Results: In our sample, 54% of subjects have a medium-high adherence to MD and only 4% have a risk of malnutrition. The variable Nutrition was significantly associated (p < 0.001) with the cognitive domain Attention/executive function, with an increase in Nutrition directly associated with a better performance in Attention/executive function. Conclusions: This study provides preliminary evidence of an association between good nutritional status, related to a high adherence to the MD, and cognitive performance in a non-clinical elderly population living in Northern Italy.

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.

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.

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.

Adherence to the Mediterranean Diet and Risk of Metabolically Unhealthy Obesity in Women: A Cross-Sectional Study.

Some obese individuals do not present any metabolic alteration and are considered metabolically healthy (MHO). Adherence to high-quality dietary pattern may favor this phenotype. We aimed to evaluate the association between the adherence to the Mediterranean diet and risk of metabolically unhealthy obesity (MUO) in women. We conducted a cross-sectional study on 2,115 obese women. All patients underwent a medical examination, anthropometric evaluation, bioelectrical impedance, ultrasound measurements of abdominal visceral (VAT) and subcutaneous (SAT) fat, blood sampling and evaluation of adherence to the Mediterranean diet through MEDAS questionnaire. The diagnosis of MHO and MUO was made using the harmonized criteria. A multivariable logistic regression adjusted for age, BMI, fat free mass, ultrasound-estimated VAT:SAT ratio, marital status, education, past diet, antidepressant use, family history of diabetes and cardiovascular disease, menopausal status, smoking, and physical activity was used to assess the association between Mediterranean diet and MUO risk. The prevalence of MHO was 21.2% (N = 449). Compared to MUO women, MHO women were younger, had lower BMI and VAT, and had higher fat free mass and SAT. In the multivariable model, the adherence to the Mediterranean diet was not associated with the risk of MUO (OR = 0.91, 95%CI: 0.62; 1.34, P = 0.624). Given the impact of menopause on metabolic health we also carried out the analysis in pre- and post-menopausal women separately. Higher adherence to the Mediterranean diet was associated with a lower risk of MUO in postmenopausal women (OR = 0.55, 95%CI: 0.31; 0.96, P = 0.034). No association was found in premenopausal women (OR = 1.18, 95%CI: 0.70; 1.99, P = 0.532). In conclusion, adherence to the Mediterranean diet was associated with a better metabolic health in postmenopausal women. Further studies are needed to confirm the ability of the Mediterranean diet in promoting maintenance of the healthy phenotype and reversion from MUO.