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The classic ketogenic diet (cKD) requires constant nutritional monitoring over time both to ensure its effectiveness and to reduce the likelihood of short- and long-term adverse effects. We retrospectively described the use of remote monitoring by e-mail during the first year of follow-up on cKD in 34 children (47% males, age range: 2&minus, 17 years) diagnosed with drug-resistant epilepsy (DRE, n = 14) or glucose transporter type 1 deficiency syndrome (GLUT1-DS, n = 20). All the e-mails were evaluated analyzing their frequency and content at 3, 6 and 12 months. Three families never sent e-mails. A median of 36.0 (IQR 23.0&ndash, 64.0) e-mails per family were sent during the 12 follow-up months by the 31 patients. GLUT1-DS patients sent a greater number of e-mails than the DRE group (median 39.0 (IQR 25.5&ndash, 56.5) vs. median 26.0 (IQR 19.0&ndash, 65.0)). At the end of the follow-up period, a greater number of e-mails had been exchanged between the nutritional team and the families belonging to the group that increased its linear growth (median 83.5, IQR 48.0&ndash, 102.0), compared to the other ones. Constant remote monitoring by e-mail could be a feasible and effective way for a better cKD management.

The ketogenic diet (KD) is the first line intervention for glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency, and is recommended for refractory epilepsy. It is a normo-caloric, high-fat, adequate-protein, and low-carbohydrate diet aimed at switching the brain metabolism from glucose dependence to the utilization of ketone bodies. Several variants of KD are currently available. Depending on the variant, KDs require the almost total exclusion, or a limited consumption of carbohydrates. Thus, there is total avoidance, or a limited consumption of cereal-based foods, and a reduction in fruit and vegetable intake. KDs, especially the more restrictive variants, are characterized by low variability, palatability, and tolerability, as well as by side-effects, like gastrointestinal disorders, nephrolithiasis, growth retardation, hyperlipidemia, and mineral and vitamin deficiency. In recent years, in an effort to improve the quality of life of patients on KDs, food companies have started to develop, and commercialize, several food products specific for such patients. This review summarizes the foods themselves, including sweeteners, and food products currently available for the ketogenic dietary treatment of neurological diseases. It describes the nutritional characteristics and gives indications for the use of the different products, taking into account their metabolic and health effects.

Objective: To describe families’ experiences in managing epileptic patients undergoing ketogenic dietary therapies (KDTs) in acute medical settings. Methods: We conducted a short online survey addressed to the families of patients undergoing a classic ketogenic diet (cKD) for at least three months. The survey was composed of 18 questions exploring the following issues: demographic characteristics, epilepsy diagnosis, ketogenic-diet treatment history, the reason for emergency-ward admission and patient management, surgery-procedure management, and outcomes. Results: A sample of 50 families agreed to participate. Out of 50 patients, 33 (66%) had been undergoing a cKD for more than two years. Fifteen (30%) patients had been admitted at least once to the Emergency Room (ER), and 8.2% had undergone surgical procedures during cKD treatment. The causes of ER admission were the following: seizures, infection, trauma, and gastrointestinal or respiratory problems. In 75% of cases, blood ketonemia was not monitored during ER admission, and according to 46% of responders, the medical staff intervening did not have a basic knowledge of KDTs. Conclusions: According to both our experience and caregivers’ reports, it might be useful to search for standardized specific approaches to patients undergoing KDTs in the emergency setting.

Ketogenic Diet (KD) is a high-fat diet used to treat refractory epilepsy in patients, also including children. Because of the inherent differences with a balanced diet, patients starting KD face an abrupt switch of dietary habits. Moreover, KD is associated with several side effects that should be closely monitored. In this paper, we propose an mHealth application for training and empowering patients in managing KD. The application also acts as a bridge connecting patients with the health care staff for coaching and monitoring purposes.

Background: Nutrition is an important factor for sports performance and the assessment of dietary intakes in athletes can correct unhealthy eating habits. However, dietary assessment may be biased due to misreporting. The aim of our study was to investigate the occurrence of misreporting in a sample of collegiate team sport athletes. Methods: A total of 50 athletes participated. Each athlete filled in food records for seven days. Reported energy intake (EI) was considered in relation to the predicted basal metabolic rate (BMR) and expressed as the ratio EI/ BMR. All participants with EI/BMRestd &le, 1.23 were classified as &ldquo, low energy reporters&rdquo, (LER), and those with an EI/BMRestd ratio &gt, adequate energy reporters&rdquo, (AER). Results: According to cut-off values for under-reporting, 28 out of 50 athletes (56%) were classified as LER. The LER (16 M/12 F) had significantly higher BMI (23.17 &plusmn, 3.46 kg/m2 compared to 21.41 &plusmn, 1.91 kg/m2, p = 0.038) than the AER. The EI/kg fat free mass (FFM) was significantly lower in LER than the AER (33.34 &plusmn, 6.56 kcal/FFM compared to 48.51 &plusmn, 8.59 kcal/FFM, p &lt, 0.0001). Nutrient intake was also significantly different between the two groups. Conclusions: Our results suggest that under-reporting of energy intake by collegiate team sport athletes may occur frequently and needs to be taken into consideration in the interpretation of nutrient intake.

Background&aims The classical ketogenic diet (KD) is a high-fat, very low-carbohydrate normocaloric diet used for drug-resistant epilepsy and Glucose Transporter 1 Deficiency Syndrome (GLUT1 DS). In animal models, high fat diet induces large alterations in microbiota producing deleterious effects on gut health. We carried out a pilot study on patients treated with KD comparing their microbiota composition before and after three months on the diet. Methods Six patients affected by GLUT1 DS were asked to collect fecal samples before and after three months on the diet. RT – PCR analysis was performed in order to quantify Firmicutes, Bacteroidetes, Bifidobacterium spp. , Lactobacillus spp. , Clostridium perfringens, Enterobacteriaceae, Clostridium cluster XIV, Desulfovibrio spp. and Faecalibacterium prausnitzii. Results Compared with baseline, there were no statistically significant differences at 3 months in Firmicutes and Bacteroidetes. However fecal microbial profiles revealed a statistically significant increase in Desulfovibrio spp. (p = 0.025), a bacterial group supposed to be involved in the exacerbation of the inflammatory condition of the gut mucosa associated to the consumption of fats of animal origin. Conclusions A future prospective study on the changes in gut microbiota of all children with epilepsy started on a KD is warranted. In patients with dysbiosis demonstrated by fecal samples, it my be reasonable to consider an empiric trial of pre or probiotics to potentially restore the «ecological balance» of intestinal microbiota.

Ketogenic diet is an established and effective non-pharmacologic treatment for drug-resistant epilepsy. Ketogenic diet represents the treatment of choice for GLUT-1 deficiency syndrome and pyruvate dehydrogenase complex deficiency. Infantile spasms, Dravet syndrome and myoclonic-astatic epilepsy are epilepsy syndromes for which ketogenic diet should be considered early in the therapeutic pathway. Recently, clinical indications for ketogenic diet have been increasing, as there is emerging evidence regarding safety and effectiveness. Specifically, ketogenic diet response has been investigated in refractory status epilepticus and encephalopathy with status epilepticus during sleep. New targets in neuropharmacology, such as mitochondrial permeability transition, are being studied and might lead to using it effectively in other neurological diseases. But, inefficient connectivity and impaired ketogenic diet proposal limit ideal availability of this therapeutic option. Ketogenic diet in Italy is not yet considered as standard of care, not even as a therapeutic option for many child neurologists and epileptologists.The aim of this review is to revisit ketogenic diet effectiveness and safety in order to highlight its importance in drug-resistant epilepsy and other neurological disorders.• Ketogenic diet efficacy is now described in large case series, with adequate diet compliance and side effects control. • Ketogenic diet is far from being attempted as a first line therapy. Its availability varies worldwide. What is New: • New pharmacological targets such as mitochondrial permeability transition and new epileptic syndromes and etiologies responding to the diet such as refractory status epilepticus are being pointed out. • Ketogenic diet can function at its best when used as a tailor-made therapy. Fine tuning is crucial.

The aim of this nested case-control study was to compare the effectiveness of cognitive-behavioral treatment (CBT) for treatment-resistant obese (body mass index [BMI] ≥ 30 kg/m²) women compared with standard dietary treatment. The main outcome measures were attrition and weight loss success.We designed a 6-month case-control study, nested within a cohort of adult (age ≥ 18 years) treatment-resistant (history of at least two previous diet attempts) obese women. Cases were 20 women who were offered CBT sessions. Controls (n=39) were randomly selected from the source population and matched to cases in terms of baseline age, BMI, and number of previous diet attempts.Compared with controls, cases were significantly more likely to complete the 6-month program in both age-adjusted (odds ratio [OR]=2.94, 95% confidence interval [CI]=1.05-8.97) and multivariate-adjusted (OR=2.77, 95% CI=1.02-8.34) analyses. In contrast, cases were not more likely to achieve weight loss success in age-adjusted (OR=1.32, 95% CI=0.86-1.67) and multivariate-adjusted (OR=1.21, 95% CI=0.91-1.44) analyses.Compared with a standard dietary treatment, CBT was significantly more effective in reducing attrition in treatment-resistant obese women, without differences in terms of weight loss success.

Objective The aim of this study was to evaluate the effects of a 12-wk ketogenic diet (KD) on inflammatory status, adipose tissue activity biomarkers, and abdominal visceral (VAT) and subcutaneous fat (SAT) in children affected by glucose transporter 1 deficiency syndrome GLUT1 DS. Methods We carried out a short-term longitudinal study on 10 children (mean age: 8.4 y, range 3.3–12 y, 5 girls, 5 boys) to determine fasting serum proinflammatory cytokines (high sensitivity C-reactive protein, tumor necrosis factor-α interleukin-6), adipocyte-derived chemokines (leptin and adiponectin), lipid profile, homeostatic model assessment-insulin resistance (HOMA-IR), quantitative insulin sensitivity index (QUICKI), anthropometric measurements, and VAT and SAT (by ultrasonography). Results Children showed no significant changes in inflammatory and adipose tissue activity biomarkers, blood glucose, lipid profile, anthropometric measurements, VAT, and SAT. Fasting insulin decreased (6 ± 3.2 μU/mL versus 3 ± 2 μU/mL; P = 0.001), and both HOMA-IR and QUICKI indexes were significantly modified (1.2 ± 0.6 versus 0.6 ± 0.4; P = 0.002; 0.38 ± 0.03 versus 0.44 ± 0.05; P = 0.002, respectively). Conclusions Only HOMA-IR and QUICKI indexes changed after 12 wk on a KD, suggesting that over a short period of time KD does not affect inflammatory cytokines production and abdominal fat distribution despite being a high-fat diet. Long-term studies are needed to provide answers concerning adaptive metabolic changes during KD.

Weight preoccupations have been frequently reported in normal-weight subjects. Subthreshold anorexia nervosa (s-AN, all DSM IV TR criteria except amenorrhea or underweight) is a form of eating disorder not otherwise specified that has received scarce scientific attention. Under a case-control design we compared the general characteristics, body composition, and psychopathological features of normal-weight patients with s-AN with those of BMI- and sex-matched controls.Participants in this pilot study included 9 normal-weight women who met the DSM IV TR criteria for s-AN and 18 BMI-matched normal-weight controls. The general characteristics of the study participants were collected by questionnaire. Body composition was measured by bioelectrical impedance. Behavioral and psychological measures included the standardized symptom checklist (SCL-90-R) and the eating disorder inventory (EDI-2).There were no differences in age, education, employment status, marital status, and history of previous slimming treatment in the two study groups. In addition, anthropometric measures and body composition of s-AN patients and BMI-matched normal weight controls were not significantly different. In the s-AN subgroup, we found a significant relationship between waist circumference and the SCL-90-R obsessivity-compulsivity scale (n=9, r=-0.69, p0.05). After multiple regression analysis, the SCL-90-R obsessivity-compulsivity scale (beta = 0.61, t=2.7, p=0.017) was the only independent predictor of the presence s-AN in our study cohort.These pilot results suggest that psychopathological criteria (particularly related to the obsessivity-compulsivity dimension) may be more useful than anthropometric measures for screening of s-AN in normal-weight women.

summary Background & aims: This 6-month prospective, single-arm observational study was designed to assess the effects of the KD on the nutritional status, resting energy expenditure (REE), and substrate oxidation in patients with drug-resistant epilepsy. Methods: Eighteen patients with medically refractory epilepsy underwent assessment of body composition, REE, and substrate oxidation rates before and after 6 months of KD. Results: Compared with baseline, there were no statistically significant differences at 6 months in terms of height, weight, BMI z-scores, and REE. However, the respiratory quotient decreased significantly (from 0.80 � 0.06to0.72 � 0.05,p

Background children affected by refractory epilepsy could be at risk of malnutrition because of feeding difficulties (anorexia, chewing, swallowing difficulties or vomiting) and chronic use of anticonvulsants, which may affect food intake and energy metabolism. Moreover, their energy requirement may be changed as their disabilities would impede normal daily activities. The aim of the present study was to evaluate nutritional status, energy metabolism and food intake in children with refractory epilepsy. Methods 17 children with refractory epilepsy (13 boys and 4 girls; mean age 9 ± 3,2 years; Body Mass Index 15,7 ± 3,6) underwent an anthropometric assessment, body composition evaluation by dual-energy X-ray absorptiometry, detailed dietetic survey and measurement of resting energy expenditure by indirect calorimetry. Weight-for-age, height-for-age (stunting) and weight-for-height (wasting) were estimated compared to those of a reference population of the same age. Results 40% of children were malnourished and 24% were wasted. The nutritional status was worse in the more disabled children. Dietary intake resulted unbalanced (18%, 39%, 43% of total daily energy intake derived respectively from protein, lipid and carbohydrate). Adequacy index [nutrient daily intake/recommended allowance (RDA) × 100] was < 60% for calcium iron and zinc. Conclusion many children with refractory epilepsy would benefit from individual nutritional assessment and management as part of their overall care.

Background Excess body fat is a major risk factor for disease primarily due to its endocrine activity. In recent years several criteria have been introduced to evaluate this factor. Nevertheless, treatment need is currently assessed only on the basis of an individual's Body Mass Index (BMI), calculated as body weight (in kg) divided by height in m2. The aim of our study was to determine whether application of the BMI, compared to adiposity-based criteria, results in underestimation of the number of subjects needing lifestyle intervention. Methods We compared treatment need based on BMI classification with four adiposity-based criteria: percentage body fat (%BF), considered both alone and in relation to metabolic syndrome risk (MS), waist circumference (WC), as an index of abdominal fat, and Body Fat Mass Index (BFMI, calculated as fat mass in kg divided by height in m2) in 63 volunteers (23 men and 40 women, aged 20 – 65 years). Results According to the classification based on BMI, 6.3% of subjects were underweight, 52.4% were normal weight, 30.2% were overweight, and 11.1% were obese. Agreement between the BMI categories and the other classification criteria categories varied; the most notable discrepancy emerged in the underweight and overweight categories. BMI compared to almost all of the other adiposity-based criteria, identified a lower percentage of subjects for whom treatment would be recommended. In particular, the proportion of subjects for whom clinicians would strongly recommend weight loss on the basis of their BMI (11.1%) was significantly lower than those identified according to WC (25.4%, p = 0.004), %BF (28.6%, p = 0.003), and MS (33.9%, p = 0.002). Conclusion The use of the BMI alone, as opposed to an assessment based on body composition, to identify individuals needing lifestyle intervention may lead to unfortunate misclassifications. Population-specific data on the relationships between body composition, morbidity, and mortality are needed to improve the diagnosis and treatment of at-risk individuals.

Background Attrition is an important but understudied issue that plays a vital role in the successful treatment of obesity. To date, most studies focusing on attrition rates and/or its predictors have been based on pretreatment data routinely collected for other purposes. Our study specifically aims at identifying the predictors of drop-out focusing on empirically or theoretically-based factors. Methods We conducted a retrospective observational study in an academic outpatient clinical nutrition service in Pavia, Italy. We examined a total of 98 adult obese patients (36 males, 62 females) who underwent a 6-month dietary behavioral weight-loss treatment at our Center. Pre-treatment and treatment-related variables were collected or calculated from clinical charts in order to discriminate those subjects who completed treatment from those who abandoned it before its completion. Multivariable regression analysis was used to identify the independent predictors of drop-out. Results The drop-out rates were 21% at 1 month and 57% at 6 months. Compared with completers, noncompleters were significantly younger in terms of age at first dieting attempt (24.0 ± 10.7 vs. 31.3 ± 11.2 years, P = 0.005), had lower diastolic blood pressure (87.8 ± 9.7 vs. 92.7 ± 11.4 mmHg, P = 0.022), had a lower baseline body fat percentage (38.5 ± 6.4 vs. 41.2 ± 4.4% weight, P = 0.015), and had a lower percentage of early weight loss (-1.8 ± 1.8% vs. -3.1 ± 2.1%, P = 0.035). Moreover, noncompleters significantly differed from completers with regard to type of referral (34.1% vs. 53.3% sent by a physician, P = 0.036) and SCL-90 anger-hostility subscale (0.83 ± 0.72 vs. 0.53 ± 0.51, P = 0.022). A multivariable logistic regression analysis including pre-treatment variables showed that body fat percentage (P = 0.030) and SCL-90 anger-hostility subscale (P = 0.021) were independently associated with attrition. In a multivariable model considering both pre-treatment and treatment-related factors, attrition was found to be independently related to the age at first dieting attempt (P = 0.016) and the achievement of early weight loss (P = 0.029). Conclusions Our data confirm that psychopathological tracts, early dieting attempts, and a poor initial treatment response are key independent predictors of drop-out from obesity treatment.