A randomized, double-blind trial of triheptanoin for drug-resistant epilepsy in glucose transporter 1 deficiency syndrome.

OBJECTIVE: This study was undertaken to evaluate efficacy and long-term safety of triheptanoin in patients >1 year old, not on a ketogenic diet, with drug-resistant seizures associated with glucose transporter 1 deficiency syndrome (Glut1DS). METHODS: UX007G-CL201 was a randomized, double-blind, placebo-controlled trial. Following a 6-week baseline period, eligible patients were randomized 3:1 to triheptanoin or placebo. Dosing was titrated to 35% of total daily calories over 2 weeks. After an 8-week placebo-controlled period, all patients received open-label triheptanoin through Week 52. RESULTS: The study included 36 patients (15 children, 13 adolescents, eight adults). A median 12.6% reduction in overall seizure frequency was observed in the triheptanoin arm relative to baseline, and a 13.5% difference was observed relative to placebo (p = .58). In patients with absence seizures only (n = 9), a median 62.2% reduction in seizure frequency was observed in the triheptanoin arm relative to baseline. Only one patient with absence seizures only was present in the control group, preventing comparison. No statistically significant differences in seizure frequency were observed. Common treatment-emergent adverse events included diarrhea, vomiting, abdominal pain, and nausea, mostly mild or moderate in severity. No serious adverse events were considered to be treatment related. One patient discontinued due to status epilepticus. SIGNIFICANCE: Triheptanoin did not significantly reduce seizure frequency in patients with Glut1DS not on the ketogenic diet. Treatment was associated with mild to moderate gastrointestinal treatment-related events; most resolved following dose reduction or interruption and/or medication for treatment. Triheptanoin was not associated with any long-term safety concerns when administered at dose levels up to 35% of total daily caloric intake for up to 1 year.

Nutritional Interventions for Patients with Mitochondrial POLG-Related Diseases: A Systematic Review on Efficacy and Safety.

Ketogenic diet is recommended as a treatment to reduce seizure frequency in patients with intractable epilepsy. The evidence and safety results are sparse for diet interventions in patients with pathogenic polymerase gamma (POLG) variants and intractable epilepsy. The aim of this systematic review is to summarize the efficacy of diet treatment on seizure frequency, clinical symptoms, and potential deleterious effect of liver involvement in patients with mitochondrial diseases caused by pathogenic POLG variants. Literature was searched in PubMed, Embase; and Cochrane in April 2022; no filter restrictions were imposed. The reference lists of retrieved studies were checked for additional literature. Eligibility criteria included verified pathogenic POLG variant and diet treatment. Overall, 880 studies were identified, providing eight case-reports representing nine patients eligible for inclusion. In eight of nine cases, clinical symptoms were improved; six out of nine cases reported improvements in seizure frequency. However, increasing levels of liver enzymes after initiating ketogenic diet were found in four of the nine cases, with one case revealing decreased levels of liver enzymes after initiating long-chain triglyceride restriction. Viewed together, the studies imply that ketogenic diet can have a positive impact on seizure frequency, but may induce progression of liver impairment in patients with pathogenic POLG variants.

Comparative Effectiveness of Insecticides for Use Against the House Fly (Diptera: Muscidae): Determination of Resistance Levels on a Malaysian Poultry Farm.

In this study, the toxicology of two commercial larvicides--cyromazine (Neporex 50SP) and ChCy (combination of chlorpyrifos and cypermethrin, Naga 505)--and five commercial adulticides--thiamethoxam (Agita 10WG), cyfluthrin (Responsar WP), lambda-cyhalothrin (Icon 2.8EC), fipronil (Regent 50SC), and imidacloprid (Toxilat 10WP)--was examined against the WHO/VCRU (World Health Organization/ Vector Control Research Unit) susceptible strain and the AYTW (Ayer Tawar) field strain of house fly, Musca domestica L. These pesticides were administered topically, in the diet, or as a dry residue treatment on plywood. Probit analysis using at least five concentrations and the concentration that was lethal to 50% (LC(50)) of the organisms was applied to compare the toxicology and resistance levels of the AYTW population to different insecticides. In the larvicide laboratory study, ChCy was more effective than cyromazine, with a significantly lower LC(50) value when administered topically or in the diet, although the AYTW population was susceptible to both larvicides with a resistance ratio (RR) <10. For the adulticide laboratory study, cyfluthrin and fipronil exhibited the lowest LC50 values of the adulticides, indicating that they are both effective at controlling adult flies, although lambda-cyhalothrin showed moderate resistance (RR = 11.60 by topical application; 12.41 by plywood treatment). Further investigation of ChCy, cyromazine, cyfluthrin, and fipronil under field conditions confirmed that ChCy and cyromazine strikingly reduced larval density, and surprisingly, ChCy also exhibited adulticidal activity, which significantly reduced adult fly numbers compared with the control group. Cyfluthrin and fipronil were also confirmed to be effective, with a significant reduction in adult fly numbers compared with the control group.

A re-evaluation of life-long severe galactose restriction for the nutrition management of classic galactosemia.

The galactose-restricted diet is life-saving for infants with classic galactosemia. However, the benefit and extent of dietary galactose restriction required after infancy remain unclear and variation exists in practice. There is a need for evidence-based recommendations to better standardize treatment for this disorder. This paper reviews the association between diet treatment and outcomes in classic galactosemia and evaluates the contribution of food sources of free galactose in the diet. Recommendations include allowing all fruits, vegetables, legumes, soy products that are not fermented, various aged cheeses and foods containing caseinates. Further research directions are discussed.

A study of the low-protein diet in delaying the course of chronic kidney disease.

A low-protein diet (LPD) has become an important way to delay the progression of chronic kidney disease (CKD) and to delay the need for dialysis. A review of the literature reveals the low-protein diet's influence on the course of chronic kidney disease. An artificial low-protein food, wheat starch, for example, can not only increase the high-quality protein intake ratio, but can ensure adequate energy intake on a low-protein diet while meeting the nutritional needs of the body, effectively reducing the burden on the damaged kidneys. The purpose of this review is to provide a reference for the clinical implementation of diet and nutrition therapy in patients with chronic kidney disease.

Quality of life in children treated with restrictive diet for inherited metabolic disease.

BACKGROUND: The aim of this study was to investigate the quality of life (QoL) of a group of patients with inherited metabolic diseases (IMD) who were treated with restrictive diet. METHOD: A total of 68 patients (35 boys, 51.5%; 33 girls, 48.5%) with IMD (organic acidemia [OA], n = 14; disorder of carbohydrate metabolism [CMD], n = 33; and disorder of amino acid metabolism [AMD], n = 21) and their parents were inteviewed. Both parents completed a QoL Scale for Metabolic Diseases-Parent Form, a KINDL parent questionnaire, and a depression form. All patients aged ≥4 years completed a questionnaire themselves, including the KINDL-Kid and KINDL-Kiddo self-reports. The semi-standardized interviews were carried out with patients and their parents in a clinical setting. RESULTS: The patients with bad diet compliance had lower scores for school labeling and perception of disease on both the parent and child questionnaire forms (P < 0.05). The patients were then divided into three groups (OA, CMD, AMD) for further analysis. Differences were seen between groups with regard to scores of physical function and school performance according to QoL Scale for Metabolic Diseases-Parent Form (P < 0.01). According to parent perceptions, the CMD patients had better QoL with regard to emotional wellbeing. CONCLUSION: As negative effects of the disease increased, the QoL of IMD patients and their parents decreased in terms of emotional, physical, and cognitive function. Application of expanded newborn scanning programs, early diagnosis, regular follow up, and family education would lessen the effects of the disease and improve the QoL of both families and children.

Frailty-Preventing Effect of an Intervention Program Using a Novel Complete Nutritional "COMB-FP Meal": A Pilot Randomized Control Trial.

Frailty is a huge concern for the aging population, and dietary nutrition is considered a key factor in the prevention of aging. To solve the problem of frailty in the aging population, we developed a novel dietary intervention program using a novel COMpletely Balanced for Frailty Prevention (COMB-FP) meal, based on the Dietary Reference Intake for Japanese; in addition, we conducted a pilot randomized control trial comparing an exercise program only (control group) with exercise plus the COMB-FP meal program (test group). We included 110 male and female healthy volunteers with pre-frailty or frailty; the trial lasted for 12 weeks. Two daily meals were replaced with the COMB-FP meals during the trial in the test group. Walking speed and cognitive function were significantly improved in the test group compared with the control group. We observed a similar pattern in other frailty-related outcomes, such as occupancy of the microbiome, World Health Organization well-being index (WHO-5), and oxidative stress. Our study might indicate the importance of a well-balanced intake of nutrients for frailty prevention.

Macronutrient composition and its effect on body composition changes during weight loss therapy in patients with non-alcoholic fatty liver disease: Secondary analysis of a randomized controlled trial.

OBJECTIVES: Dietary composition may affect body composition during weight loss therapy. We tested the hypothesis of whether dietary macronutrient composition influences the reduction of total abdominal adipose tissue, subcutaneous adipose tissue (SAT), or visceral adipose tissue (VAT) during weight loss. METHODS: Dietary macronutrient composition and body composition were analyzed as a secondary outcome of a randomized controlled trial of 62 participants with non-alcoholic fatty liver disease. Patients were randomly assigned to a calorie-restricted intermittent fasting (5:2), calorie-restricted low-carbohydrate high-fat (LCHF), or healthy lifestyle advice (standard-of-care) diet in a 12-wk intervention phase. Dietary intake was assessed by self-reported 3-d food diaries and by characterization of total plasma fatty acid profile. Percentage of energy intake (E%) from different macronutrients was calculated. Body composition was assessed by magnetic resonance imaging and anthropometric measurements. RESULTS: The macronutrient composition differed significantly between the 5:2 (fat 36 E% and carbohydrates 43 E%) and the LCHF (fat 69 E% and carbohydrates 9 E%) groups (P < 0.001). Weight loss was similar in the 5:2 and LCHF groups (-7.2 [SD = 3.4] kg versus 8.0 [SD = 4.8] kg; P = 0.44) and significantly larger than for standard of care (-2.5 kg [SD = 2.3]; P < 0.001). The volume of total abdominal fat, adjusted for height, decreased on average by 4.7% (standard of care), 14.3% (5:2), and 17.7% (LCHF), with no significant differences between the 5:2 and LHCF groups (P = 0.32). VAT and SAT, adjusted for height, decreased on average by 17.1% and 12.7% for 5:2, respectively, and by 21.2% and 17.9% for LCHF, with no significant group differences (VAT [P = 0.16] and SAT [P = 0.10]). VAT was mobilized to a greater extent than SAT in all diets. CONCLUSIONS: The 5:2 and LCHF diets had similar effects on changes in intraabdominal fat mass and anthropometrics during weight loss. This might indicate that overall weight loss is more important than diet composition to achieve changes in total abdominal adipose tissue, VAT, or SAT. The results of the present study suggest that there is a need for further studies on the effect of diet composition on body composition changes during weight loss therapy.

The impact of a structured weight-loss treatment on physical fitness in patients with psoriatic arthritis and obesity compared to matched controls: a prospective interventional study.

OBJECTIVES: To evaluate the effects of weight loss treatment on physical fitness in patients with psoriatic arthritis (PsA) and obesity compared to matched controls. METHODS: In total, 46 patients with PsA (CASPAR) and BMI ≥ 33 kg/m2 and 52 obese persons were included in this 12-month prospective open intervention study with a very low energy diet (640 kcal/day), followed by structured reintroduction of an energy-restricted diet and brief support for physical activity. The primary outcome was muscle strength assessed with hand-grip strength (Grippit) and leg muscle strength (timed stand test). Secondary outcomes were cardiorespiratory fitness, body composition, and physical functioning (SF-36PCS). Outcomes were assessed at baseline, 6 (M6), and 12 months (M12). Nonparametric statistics were used. RESULTS: Median weight reduction at M6 was 18.9 kg in patients and 23.0 kg in controls, (p = 0.546). At M12, patients' median weight loss from baseline was 16.1 kg, corresponding with significant loss of total fat mass (- 30.1%), and lean mass (total - 7.0%, arm - 13.7%, and leg - 6.0%). Leg muscle strength improved in patients and controls at M6 (p < 0.001) and remained improved at M12 (p < 0.01), while hand-grip strength was unchanged in both groups. Cardiorespiratory fitness increased in controls at M6 (p = 0.018) and M12 (p = 0.028) but not in patients. Physical functioning improved in both groups at M6 (p < 0.001) and remained improved at M12 (p = 0.008) and (p < 0.01), respectively. CONCLUSION: The intervention resulted in positive effects on body weight and total body fat. Despite reduced lean body mass, the muscle strength did not deteriorate in patients with PsA and controls. TRIAL REGISTRATION: ClinicalTrials.gov identifier: NCT02917434, registered on September 21, 2016-retrospectively registered.

Treatment of NAFLD with intermittent calorie restriction or low-carb high-fat diet - a randomised controlled trial.

BACKGROUND & AIMS: The first-line treatment for non-alcoholic fatty liver disease (NAFLD) is weight reduction. Several diets have been proposed, with various effects specifically on liver steatosis. This trial compared the effects of intermittent calorie restriction (the 5:2 diet) and a low-carb high-fat diet (LCHF) on reduction of hepatic steatosis. METHODS: We conducted an open-label randomised controlled trial that included 74 patients with NAFLD randomised in a 1:1:1 ratio to 12 weeks' treatment with either a LCHF or 5:2 diet, or general lifestyle advice from a hepatologist (standard of care; SoC). The primary outcome was reduction of hepatic steatosis as measured by magnetic resonance spectroscopy. Secondary outcomes included transient elastography, insulin resistance, blood lipids, and anthropometrics. RESULTS: The LCHF and 5:2 diets were both superior to SoC treatment in reducing steatosis (absolute reduction: LCHF: -7.2% [95% CI = -9.3 to -5.1], 5:2: -6.1% [95% CI = -8.1 to -4.2], SoC: -3.6% [95% CI = -5.8 to -1.5]) and body weight (LCHF: -7.3 kg [95% CI = -9.6 to -5.0]; 5:2: -7.4 kg [95% CI = -8.7 to -6.0]; SoC: -2.5 kg [95% CI =-3.5 to -1.5]. There was no difference between 5:2 and LCHF (p = 0.41 for steatosis and 0.78 for weight). Liver stiffness improved in the 5:2 and SoC but not in the LCHF group. The 5:2 diet was associated with reduced LDL levels and was tolerated to a higher degree than LCHF. CONCLUSIONS: The LCHF and 5:2 diets were more effective in reducing steatosis and body weight in patients with NAFLD than SoC, suggesting dietary advice can be tailored to meet individual preferences. LAY SUMMARY: For a person with obesity who suffers from fatty liver, weight loss through diet can be an effective treatment to improve the condition of the liver. Many popular diets that are recommended for weight reduction, such as high-fat diets and diets based on intermittent fasting, have not had their effects on the liver directly evaluated. This study shows that both a low-carb high-fat and the 5:2 diet are effective in treating fatty liver caused by obesity. CLINICAL TRIALS REGISTRATION: This study is registered at Clinicaltrials.gov (NCT03118310).

Effect of Three Diets (Low-FODMAP, Gluten-free and Balanced) on Irritable Bowel Syndrome Symptoms and Health-Related Quality of Life.

Several studies have reported some efficacy of diets low in fermentable carbohydrates (Fermentable Oligo-, Di-, Monosaccharides and Polyols (FODMAPs)) in Irritable Bowel Syndrome (IBS). There is no evidence of its superiority compared to gluten-free and balanced diets in improving IBS patients' quality of life (QoL). The aim of this study is to assess whether different diets can improve QoL in IBS. Forty-two patients with IBS, according to Rome IV criteria, were enrolled. Low-FODMAP, gluten-free and balanced diets were proposed to each patient in the same succession. Each diet was followed for 4 weeks. The Bristol Stool Scale, the Visual Analogue Scale (VAS) for bloating and abdominal pain, and the SF12 questionnaire for health-related quality of life were applied at the beginning and at the end of each diet. Twenty-eight of the forty-two patients completed all the three diets. All the three diets reduced symptom severity (p < 0.01), bloating (p < 0.01) and abdominal pain (p < 0.01), and improved quality of life (p < 0.05); 3% of patients expressed a preference for the low-FODMAP diet, 11% for the gluten-free and 86% for the balanced diet (p < 0.01). The balanced diet improves QoL and VAS pain, provides an adequate quantity of FODMAPs and is more appreciated by patients. For these reasons, the balanced diet could be recommended to patients with irritable bowel syndrome.

Quality of Life in Chronic Ketogenic Diet Treatment: The GLUT1DS Population Perspective.

BACKGROUND: Glucose transporter type 1 deficiency syndrome (GLUT1DS) is a rare, genetically determined neurological disorder, for which Ketogenic Diet (KD) represents the gold standard life-long treatment. The aim of this study is to investigate health related quality of life in a well characterized cohort of patients affected by GLUT1DS treated with KD, evaluating factors that can influence patients' and parents' quality of life perception. METHODS: This is a double center exploratory research study. A postal survey with auto-administrable questionnaires was conducted among 17 subjects (aged 3-22 years) with diagnosis of GLUT1DS, receiving a stable KD treatment for more than 1 year. The Pediatric Quality of Life Inventory (PedsQL) 4.0 Generic Core Scales was adopted. Clinical variables analyzed in relation to quality of life were frequency of epileptic seizures and movement disorder since KD introduction, presence of intellectual disability (ID), and KD ratio. RESULTS: Quality of life global scores were impaired both in parents' and children's perspectives, with a significant concordance. Taking into consideration subscales, the average was 64.17 (range 10-100) for physical functioning, 74.23 (range 30-100) for emotional functioning, 62.64 (range 10-100) for social functioning, and 56 (range 15-92) for school functioning. CONCLUSIONS: In patients with GLUT1DS the quality of life perception is comparable to that of other patients with chronic disease. In our sample, the presence of movement disorder seems to be a crucial element in quality of life perception.

Intervenciones de enfermería para la atención integral del paciente adulto con hipertensión arterial / Nursing interventions for comprehensive care of adult patients with hypertension

Introducción: El CIE-10: I10X Hipertensión esencial, la define como enfermedad crónica asociada a estilos de vida que conducen a obesidad, sobrepeso y sedentarismo, consumo excesivo de alcohol, sal, tabaco y estrés, se caracteriza por niveles elevados y sostenidos de presión arterial iguales o mayores a 140 mmHg (sistólica) y sobre 90mmHg (diastólica)Métodos: se realizó búsqueda de documentos en PubMed y sitios web utilizando los términos MeSH: Classification, Complications, Diagnosis, Diet, therapy, Epidemiology, Etiology, Mortality, Nursing, Pharmacology Prevention, control and Therapy; en idiomas Inglés, Español y Portugués, publicados del 2008 al 2013, se obtuvieron 53 resultados, seleccionando 13 para su revisión, mismos que fueron aceptados a través de la aplicación de CASPE. Las evidencias y recomendaciones obtenidas fueron validadas con la escala de NICE y los puntos de buena práctica son aportaciones consensuadas por los autores de la guía de práctica clínica aplicando la enfermería basada en la evidencia.Resultados: Aumenta el riesgo de hipertensión la Ingesta elevada de sal y grasas, insuficiente consumo de frutas y hortalizas, ingestión dietética baja de calcio y potasio, alcoholismo, tabaquismo, estrés, sedentarismo. Conclusiones: Realizar medición de la presión, restringir ingesta de sal, moderar el consumo de alcohol, modificar la dieta diaria, reducción y control del peso, actividad física, abandono del tabaco, manejo de estrés y adherencia terapéutica. Clasificación, complicaciones, diagnóstico, la dieta, el tratamiento, epidemiología, etiología, mortalidad, enfermería, farmacología prevención, control y terapia

Introduction: The ICD-10: I10X Essential hypertension, defined as a chronic disease associated with lifestyles that lead to obesity, overweight and physical inactivity, excessive alcohol consumption, salt, snuff and stress, is characterized by high and sustained levels of pressure blood equal to or greater than 140 mmHg (systolic) and 90 mmHg (diastolic)Methods: document search was conducted in PubMed and websites using the MeSH terms: Classification, Complications, Diagnosis, Diet, therapy, Epidemiology, Etiology, Mortality, Nursing, Pharmacology Prevention, Control and Therapy; English, Spanish and Portuguese languages, published from 2008 to 2013, 53 results were obtained by selecting 13 for review, all of which were accepted through the application of Caspe. The evidence and recommendations obtained were validated with the scale of NICE and points of good practice are contributions agreed by the authors of the clinical practice guideline applying evidence-based nursing.Results: Increased risk of hypertension high salt and fat intake, low consumption of fruit and vegetables, low calcium and potassium, alcoholism, smoking, stress, sedentary lifestyle dietary intake.Conclusions: Perform measurement of pressure, restricting salt intake, moderate alcohol consumption, modifying the diet, weight reduction and control, physical activity, abandonment of snuff, stress management and adherence. classification, complications, diagnosis, diet, treatment, epidemiology, etiology, mortality, nursing, pharmacology prevention, control and therapy

Introdução: O ICD-10: I10X A hipertensão essencial, definida como uma doença crónica associada a estilos de vida que levam à obesidade, inatividade sobrepeso e físico, consumo excessivo de álcool, sal, rapé e stress, é caracterizada por níveis elevados e sustentados de pressão sanguínea igual ou superior a 140 mmHg (sistólica) e 90 mmHg (diastólica)Métodos: pesquisa de documentos foi realizada no PubMed e sites utilizando os termos MeSH: Classificação, complicações, diagnóstico, dieta, terapia, epidemiologia, etiologia, Mortalidade, Enfermagem, Farmacologia Prevenção, Controle e Terapia; Inglês, espanhol e português, publicados 2008-2013, 53 resultados foram obtidos, selecionando 13 para revisão, todos os quais foram aceites através da aplicação de Caspe. As evidências e recomendações obtidas foram validados com a escala de Nice e pontos de boa prática são contribuições acordadas pelos autores da prática clínica orientação aplicáveis enfermagem baseada em evidências.Resultados: Aumento do risco de alta sal hipertensão e ingestão de gordura, baixo consumo de frutas e legumes, baixo cálcio e potássio, alcoolismo, tabagismo, estresse, sedentarismo ingestão alimentar.Conclusões: Realizar medição de pressão, restringindo a ingestão de sal, o consumo moderado de álcool, modificação da dieta, redução de peso e controle, a atividade física, o abandono de rapé, gestão do stress e aderência. classificação, complicações, diagnóstico, dieta, tratamento, epidemiologia, etiologia, de mortalidade, de enfermagem, de prevenção de farmacologia, de controlo e de terapia