The objective of the present study was to evaluate the effect of electroporation on the membrane properties of lactobacilli and their ability to remove cholesterol in vitro. The growth of lactobacilli cells treated at 7.5kV/cm for 4ms was increased by 0.89 to 1.96 log(10) cfu/mL upon fermentation at 37°C for ...

Studies have shown a negative association between cellular cholesterol efflux and coronary artery disease (CAD). Standard protocol for quantitating cholesterol efflux involves labeling cells with [3H]-cholesterol and measuring release of the labeled sterol. Using [3H]-cholesterol is not ideal for the development of a high-throughput assay to screen large numbers of ...

An automated fluorescence microscopy assay using a non-toxic cholesterol-binding protein, toxin domain 4 (D4) (Shimada et al. Eur J Biochem 269: 6195 (2002)), was developed, in order to identify chemical compounds modifying intracellular cholesterol metabolism and distribution. Using this method, we screened a library of 1056 compounds and identified 35 ...

Free cholesterol is the predominant form of cholesterol in the neural retina. The vertebrate neural retina exhibits its own capacity to synthesize cholesterol and meets its demand also by taking it from the circulation. Defects in cholesterol synthesis and trafficking in the neural retina has detrimental consequences on its structure ...

N-6 and n-3 polyunsaturated fatty acids (PUFAs) have been shown to prevent tissue release of inflammatory molecules. We have shown that a combination of n-6 and n-3 PUFAs is more efficient than single supplementations on the long-term consequences of intraocular pressure elevation. We hypothesized that such an association is also ...

To describe patient selection, treatment administration, response evaluation, and side effect management associated with sapropterin therapy in infants and children aged <4 years. Six case reports are presented from 4 US metabolic clinics treating phenylketonuria with sapropterin in patients aged 7 months to 4 years. Outcomes included blood phenylalanine (Phe) ...

The ketogenic diet (KD) is an effective treatment for refractory epilepsy in children. It has been little studied in adults. We evaluated the efficacy of, safety of, and compliance with adjunctive KD treatment in adults with refractory epilepsy in a prospective open-label pilot study. Seizure frequency was evaluated for 4 ...

The use of amaranth, quinoa and buckwheat for the production of gluten-free pasta was investigated in the present study. The aim of the work was to produce pasta of good textural quality, in particular, low cooking loss, optimal cooking weight and texture firmness. The results demonstrated that pasta produced from ...

Age-related macular degeneration (AMD) represents the major cause of vision loss in industrialized nations. Laminar deposits in Bruch's membrane (BM) are among the first prominent histopathologic features, along with drusen formation, and have been found to contain oxidized lipids. Increases in concentrations of oxidized LDL (oxLDL) in plasma are observed ...

The ketogenic diet is an effective medical therapy for intractable childhood epilepsy. However, it has drawbacks in that it restricts calories, fluids and protein. The Atkins diet may also induce ketosis without those restrictions. Our objective was to evaluate the efficacy of a modified Atkins diet in children with intractable ...

Phenylketonuria is the most prevalent disorder caused by an inborn error in aminoacid metabolism. It results from mutations in the phenylalanine hydroxylase gene. Phenotypes can vary from a very mild increase in blood phenylalanine concentrations to a severe classic phenotype with pronounced hyperphenylalaninaemia, which, if untreated, results in profound and ...

The aim of this study was to assess how the ketogenic diet influences the blood levels of antiepileptic drugs in the first month of treatment in a pediatric population with drug-resistant epilepsy. The plasma concentrations of antiepileptic drugs were investigated in an open study on 36 consecutive children and adolescents ...

Traumatic brain injury (TBI) frequently leads to epilepsy. The process of epileptogenesis - the development of that seizure state - is still poorly understood, and effective antiepileptogenic treatments have yet to be identified. The ketogenic diet (KD) has been shown to be effective as an antiepileptic therapy, but has not ...

The modified Atkins diet is a high-fat, low-carbohydrate treatment for intractable childhood epilepsy. As data suggest that a stricter diet onset can be more effective, we added a ketogenic supplement to the modified Atkins diet during its initial month. Thirty children with intractable epilepsy were prospectively started on the modified ...

The ketogenic diet has long been used to treat epilepsy, but its mechanism is not yet clearly understood. To explore the potential mechanism, we analyzed the changes in gene expression induced by the ketogenic diet in the rat kainic acid (KA) epilepsy model. KA-administered rats were fed the ketogenic diet ...

Fever induced refractory epileptic encephalopathy in school age children (FIRES) is a devastating condition initiated by prolonged perisylvian refractory status epilepticus (SE) triggered by fever of unknown cause. SE may last more than 1 month, and this condition may evolve into pharmacoresistant epilepsy associated with severe cognitive impairment. We aimed ...

The Marsh classification is a semiquantitative method for the diagnosis and monitoring of changes in duodenal biopsies in celiac disease. We have explored the possibility that quantitative changes in villous area and crypt length (morphometry) may provide better information on changes in duodenal morphology, particularly after the introduction of a ...

The effect of the ketogenic diet on behavior and cognition is unclear. We addressed this issue in rats behaviorally and electrophysiologically.We fed postnatal day 21 rats a standard diet (SD), ketogenic diet (KD), or calorie-restricted diet (CR) for 2–3 weeks. CR controlled for the slower weight gain experienced by KD-fed ...

Dietary therapies are established as beneficial for symptomatic generalized epilepsies such as Lennox-Gastaut syndrome; however, the outcome for idiopathic generalized epilepsy has never been specifically reported. The efficacy of the ketogenic and modified Atkins diet for childhood and juvenile absence epilepsy was evaluated from both historical literature review and patients ...

PURPOSE: Although the number of antiepileptic drugs (AEDs) is increasing, none displays neuroprotective or antiepileptogenic properties that could prevent status epilepticus (SE)-induced drug-resistant epilepsy. Ketogenic diet (KD) and calorie restriction (CR) are proposed as alternative treatments in epilepsy. Our goal was to assess the neuroprotective or antiepileptogenic effect of these ...

Heart muscle is metabolically versatile, converting energy stored in fatty acids, glucose, lactate, amino acids, and ketone bodies. Here, we use mouse models in ketotic nutritional states (24 h of fasting and a very low carbohydrate ketogenic diet) to demonstrate that heart muscle engages a metabolic response that limits ketone ...

QUESTION: Families of children with epilepsy now ask about the ketogenic diet at clinic visits. Is it effective and safe to use for treatment of epilepsy? ANSWER: The ketogenic diet can be considered an option for children with intractable epilepsy who use multiple antiepileptic drugs. It is a treatment of ...

To report a case of a woman presenting with primary infertility who on investigation was found to have celiac disease and had a successful conception when on a gluten-free diet for a period of 8 months. Case report. Tertiary care university hospital. A 30-year-old female patient with primary infertility. Gluten-free ...

The role of pharmacological and surgical management of epilepsy continues to expand, but these treatments are often associated with significant side effects and morbidity. As a result, many patients with epilepsy and their physicians alike have gained interest in the role of vitamins and other dietary supplements for seizure management. ...

In this paper, we report on a 5-year-old girl who developed a renal stone while following the ketogenic diet to treat refractory seizure disorder. Three months after initiating the ketogenic diet, she developed severe abdominal pain and vomiting. The spot urine calcium-to-creatinine (Ca/Cr) ratio and 24-hour urine evaluation showed hypercalciuria. ...

Calorie restriction can be anticonvulsant in animal models. The ketogenic diet was designed to mimic calorie restriction and has been assumed to work by the same mechanisms. We challenged this assumption by profiling the effects of these dietary regimens in mice subjected to a battery of acute seizure tests. Juvenile ...

BACKGROUND: In 2000, a survey showed that use of the ketogenic diet as a treatment for intractable epilepsy in the UK was low. Subsequently, the number of medical publications supporting its efficacy has increased and demand from parents for this treatment has also increased. This survey was undertaken to determine ...

BACKGROUND: Human beta-defensins (hBDs) are small cationic, widely expressed proteins involved in innate immunity that exert strong bactericidal activity toward various pathogens. However, the role of hBDs in various diseases to which bacterial infection add severity, as it is in celiac disease (CD), is not yet clear. We analyzed the ...

The ketogenic diet (KD) is a high fat, restricted carbohydrate regime that has been used as a treatment for seizures since the 1920s, when it was designed to induce a similar metabolic response to fasting. A modification of this early classical version of the KD was introduced in the 1970s ...

In this study, we compared corn gluten hydrolyzates, BCAAs, and leucine for their effects on body weight reduction in high fat-induced obese rats in order to determine the major active components in the corn gluten hydrolyzates. After obesity was induced for 13 weeks with high fat diet, the overweight-induced SD ...

Ketogenic diet (KD) is a high-fat, low-protein and low-carbohydrate diet. It is reported that KD can provide the neuroprotection for the neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease (PD) and amyotrophic lateral sclerosis. The main clinical symptom of PD is motor dysfunction derived from the loss of dopaminergic neurons ...

Mitochondrial dysfunction is a major cause of neurodegenerative and neuromuscular diseases of adult age and of multisystem disorders of childhood. However, no effective treatment exists for these progressive disorders. Cell culture studies suggested that ketogenic diet (KD), with low glucose and high fat content, could select against cells or mitochondria ...

PURPOSE: The ketogenic diet has well-established short- and long-term outcomes for children with intractable epilepsy, but only for those actively receiving it. However, no information exists about its long-term effects years after it has been discontinued. METHODS: Living subjects were identified who were treated at the Johns Hopkins Hospital with ...

BACKGROUND: Phenylketonuria is an inherited disease treated with dietary restriction of the amino acid phenylalanine. The diet is initiated in the neonatal period to prevent mental handicap; however, it is restrictive and can be difficult to follow. Whether the diet can be relaxed or discontinued during adolescence or should be ...

Celiac disease is a permanent intolerance to the gliadin fraction of wheat gluten and to similar barley and rye proteins that occurs in genetically susceptible subjects. After ingestion, degraded gluten proteins reach the small intestine and trigger an inappropriate T cell-mediated immune response, which can result in intestinal mucosal inflammation ...

A 13-year-old girl with controlled coeliac disease who had been on a gluten-free diet for the past 3 months was admitted with respiratory distress and hypoxia for the past week. Chest radiograph and CT scan showed bilateral widespread alveolar shadowing suggestive of a hypersensitive pneumonitis. There was a dramatic radiological ...

This is an empirical study of the way in which celiac disease patients manage the risk of gluten intake in their everyday life.The article examines naturally occurring conversational data in order to study how patients cope interactionally with constantly being at risk in their day-to-day living. They reject quitting the ...

BACKGROUND: Phenylketonuria is an inherited disease for which the main treatment is the dietary restriction of the amino acid phenylalanine. The diet has to be initiated in the neonatal period to prevent or reduce mental handicap. However, the diet is very restrictive and unpalatable and can be difficult to follow. ...

AIMS: To identify risk as well as protective factors related to compliance with the gluten-free diet in a cohort of teenagers with celiac disease (CD). PATIENTS AND METHODS: Two hundred four patients with CD (European Society for Pediatric Gastroenterology, Hepatology, and Nutrition criteria) older than or equal to 13 years ...

Phenylketonuria (PKU) was the first inherited metabolic disease in which treatment was found to prevent clinical features of the disorder; dietary management was established almost 60 years ago. The institution of a low-phenylalanine (Phe) diet in the first few weeks of life was made possible by Guthrie neonatal screening, which ...

Management of glioblastoma multiforme (GBM) has been difficult using standard therapy (radiation with temozolomide chemotherapy). The ketogenic diet is used commonly to treat refractory epilepsy in children and, when administered in restricted amounts, can also target energy metabolism in brain tumors. We report the case of a 65-year-old woman who ...

OBJECTIVE: To determine the effect of coeliac disease and treatment with a gluten-free diet on growth and glycaemic control in asymptomatic children with Type 1 diabetes. METHODS: Data were compared in children with coeliac disease diagnosed by annual antibody screening and jejunal biopsy and treated with a gluten-free diet (n ...

Ketogenic diets influence brain function and have been therapeutically used for anti-epileptic purposes. We investigated the effects of maternal ketogenic diets on the development of somatic and reflex responses in rat pups. These were born from mothers receiving: (i) normal fat (7%) + normal protein (17%); (ii) high-fat (55.4%) + ...

Prolonged status epilepticus (SE) can be refractory to conventional interventions, with high rates of subsequent morbidity and mortality. A high fat, low protein, low carbohydrate ketogenic diet (KD) has been used successfully to treat intractable epilepsy. However, its possible role in prolonged SE has not been well described. We report ...

BACKGROUND: We have shown that urine peptide increase is found in autism, and that some of these peptides have a dietary origin. To be explanatory for the disease process, a dietary effect on the brain must be shown to be possible and probable. METHODS: Diagnosis was based on DSM-III and ...

In recent years, there has been much focus on research on non-dietary treatments in phenylketonuria (PKU). However, diet is likely to remain the major treatment for many years to come, since it has continued to be developed and consistent improvements have occurred. For example, with protein substitute, studies have tried ...

For many years the neuromodulator adenosine has been recognized as an endogenous anticonvulsant molecule and termed a "retaliatory metabolite." As the core molecule of ATP, adenosine forms a unique link between cell energy and neuronal excitability. In parallel, a ketogenic (high-fat, low-carbohydrate) diet is a metabolic therapy that influences neuronal ...

A pilot prospective follow-up study of the role of the branched chain amino acids as additional therapy to the ketogenic diet was carried out in 17 children, aged between 2 and 7 years, with refractory epilepsy. All of these patients were on the ketogenic diet; none of them was seizure ...

Lifelong treatment of phenylketonuria (PKU) includes a phenylalanine (phe) restricted diet that provides sufficient phe for growth and maintenance plus phe-free amino acid formula to meet requirements for protein, energy and micronutrients. Phe tolerance (mg phe/kg body weight/day) is the amount of phe those with PKU can consume and maintain ...

AIM: The purpose of this study was to estimate the caloric intake and fat consumption in children with celiac disease (CD) following a gluten-free diet (GFD). PATIENTS AND METHODS: This study enrolled 100 subjects, including 50 children with CD on a gluten-free diet and a control group of 50 healthy ...