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1.
BMC Vet Res ; 19(1): 145, 2023 Sep 06.
Artigo em Inglês | MEDLINE | ID: mdl-37674206

RESUMO

BACKGROUND: Idiopathic epilepsy (IE) is a common, chronic brain dysfunction in dogs. Recently, the effect of feeding a diet enriched with medium-chain triglycerides (MCTs) on seizure frequency has been evaluated in several studies in dogs with IE. However, most dogs with IE in previous studies were treated with phenobarbital as the main antiseizure medication (ASM). In Japan, zonisamide (ZNS) is the most prescribed ASM for dogs with IE. The interaction between ZNS and various nutrients including MCTs and the potential effects on treatment efficacy resulting from combining these therapies have not been previously studied. A prospective, randomized, double-blinded, placebo-controlled, crossover dietary study was conducted. Dogs (n = 7) treated with ZNS were fed either a placebo diet (PL) or Purina ProPlan Veterinary Diet NeuroCare (NC) for 3 months, after which treatments were crossed over and continued for another 3 months. Seizure frequency (seizures/month; sz/m), blood tests including concentrations of ZNS and ß-hydroxybutyric acid, and owner's visual analogue scale score were collected from all dogs for both treatment periods. RESULTS: There was no significant difference in the seizure frequency between PL (2.95 ± 0.80 sz/m) and NC (1.90 ± 0.57 sz/m) during the 6 months of trial. Three of 7 dogs showed ≥ 50% seizure reduction, and 1 of those 3 dogs achieved seizure freedom in NC period. However, 2 of 7 dogs had no changes in epileptic seizure frequency, 2 of 7 dogs had a deterioration in seizure frequency in the NC period. Feeding the MCT diet concurrent with ZNS showed no apparent adverse effects and did not affect ZNS concentration. CONCLUSIONS: This study indicated that the commercially available MCT-enriched diet (NC) can be safely used concurrently with ZNS for dogs with IE.


Assuntos
Doenças do Cão , Epilepsia , Cães , Animais , Zonisamida/uso terapêutico , Estudos Prospectivos , Epilepsia/tratamento farmacológico , Epilepsia/veterinária , Convulsões/tratamento farmacológico , Convulsões/veterinária , Dieta/veterinária , Triglicerídeos , Doenças do Cão/tratamento farmacológico
2.
J Inherit Metab Dis ; 45(3): 541-556, 2022 05.
Artigo em Inglês | MEDLINE | ID: mdl-35076099

RESUMO

Inherited errors of mitochondrial fatty acid ß-oxidation (FAO) are life threatening, even with optimum care. FAO is the major source of energy for heart and is critical for skeletal muscles especially during physiologic stress. Clinical trials revealed that triheptanoin (commercially known as Dojolvi; C7G), improved heart function and decreased hypoglycemia in long chain FAO disorders, but other symptoms including rhabdomyolysis persisted, suggesting suboptimal tissue distribution/utilization of heptanoic acid (C7) conjugates and/or rapid liver breakdown. In this study, medium branched chain fatty acids were tested as potential anaplerotic treatments in fibroblasts from patients deficient in very long chain acyl-CoA dehydrogenase (VLCAD), long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD), trifunctional protein (TFP), and carnitine palmitoyltransferase II (CPT II). Cells were cultured to near confluency and treated with C7, 2,6-dimethylheptanoic acid (dMC7), 6-amino-2,4-dimethylheptanoic acid (AdMC7), or 4,8-dimethylnonanoic acid (dMC9) for 72 h and targeted metabolomics performed. The profile of TCA cycle intermediates was improved in cells treated with these branched chain fatty acids compared with C7. Intracellular propionate was higher in AdMC7 treated cells compared with C7 in VLCAD, LCHAD, and TFP deficient cell lines. With AdMC7 treatment, succinate was higher in CPT II and VLCAD deficient cells, compared with C7. Malate and glutamate were consistently higher in AdMC7 treated VLCAD, LCHAD, TFP, and CPT II deficient cells compared with the C7 treatment. The results provide the impetus to further evaluate and consider branched chain fatty acids as viable anaplerotic therapy for fatty acid oxidation disorders and other diseases.


Assuntos
Acil-CoA Desidrogenase de Cadeia Longa , Erros Inatos do Metabolismo Lipídico , Carnitina O-Palmitoiltransferase/genética , Carnitina O-Palmitoiltransferase/metabolismo , Ciclo do Ácido Cítrico , Ácidos Graxos/metabolismo , Humanos , Erros Inatos do Metabolismo Lipídico/metabolismo , Oxirredução
3.
Compr Rev Food Sci Food Saf ; 21(2): 843-867, 2022 03.
Artigo em Inglês | MEDLINE | ID: mdl-35181994

RESUMO

Medium chain triglycerides (MCT) are esters of fatty acids with 6 to 12 carbon atom chains. Naturally, they occur in various sources; their composition and bioactivity are source and extraction process-linked. The molecular size of MCT oil permits unique metabolic pathways and energy production rates, making MCT oil a high-value functional food. This review details the common sources of MCT oil, presenting critical information on the various approaches for MCT oil extraction or synthesis. Apart from conventional techniques, non-thermal processing methods that show promising prospects are analyzed. The biological effects of MCT oil are summarized, and the range of need-driven modification approaches are elaborated. A section is devoted to highlighting the recent trends in the application of MCT oil for food, nutraceuticals, and allied applications. While much is debated about the role of MCT oil in human health and wellness, there is limited information on daily requirements, impact on specific population groups, and effects of long-term consumption. Nonetheless, several studies have been conducted and continue to identify the most effective methods for MCT oil extraction, processing, handling, and storage. A knowledge gap exists and future research must focus on technology packages for scalability and sustainability.


Assuntos
Suplementos Nutricionais , Ácidos Graxos , Indústria Alimentícia , Humanos , Triglicerídeos/química
4.
J Nutr ; 151(10): 2949-2956, 2021 10 01.
Artigo em Inglês | MEDLINE | ID: mdl-34255085

RESUMO

BACKGROUND: Dietary saturated fat raises total cholesterol and LDL cholesterol levels. It is unclear whether these effects differ by the fatty acid chain lengths of saturated fats; particularly, it is unclear whether medium-chain fatty acids increase lipid levels. OBJECTIVES: We conducted a systematic review to determine the effects of medium-chain triglyceride (MCT) oil, consisting almost exclusively of medium-chain fatty acids (6:0-10:0), on blood lipids. METHODS: We searched Medline and Embase through March 2020 for randomized trials with a minimum 2-week intervention period that compared MCT oil with another fat or oil. Outcomes were total cholesterol, LDL cholesterol, HDL cholesterol, and triglyceride levels. Included studies were restricted to adults above 18 years of age. Studies conducted in populations receiving enteral or parenteral nutrition were excluded. Data were pooled using a random-effects meta-analysis. RESULTS: Seven articles were included in the meta-analysis; LDL cholesterol and HDL cholesterol were reported in 6 studies. MCT oil intake did not affect total cholesterol (0.04 mmol/L; 95% CI, -0.11 to 0.20; I2 = 33.6%), LDL cholesterol (0.02 mmol/L; 95% CI, -0.13 to 0.17; I2 = 28.7%), or HDL cholesterol (-0.01 mmol/L; 95% CI, -0.10 to 0.09; I2 = 74.1%) levels, but did increase triglycerides (0.14 mmol/L; 95% CI, 0.01-0.27; I2 = 42.8%). Subgroup analyses showed that the effects of MCT oil on total cholesterol and LDL cholesterol differed based on the fatty acid profile of the control oil (Pinteraction = 0.003 and 0.008, respectively), with MCT oil increasing total cholesterol and LDL cholesterol when compared to a comparator consisting predominantly of unsaturated fatty acids, and with some evidence for reductions when compared to longer-chain SFAs. CONCLUSIONS: MCT oil does not affect total cholesterol, LDL cholesterol, or HDL cholesterol levels, but does cause a small increase in triglycerides.


Assuntos
Colesterol , Lipídeos , HDL-Colesterol , Gorduras na Dieta , Humanos , Ensaios Clínicos Controlados Aleatórios como Assunto , Triglicerídeos
5.
Int J Mol Sci ; 22(22)2021 Nov 15.
Artigo em Inglês | MEDLINE | ID: mdl-34830192

RESUMO

Recently, type 2 diabetes mellitus (T2DM) has been reported to be strongly associated with Alzheimer's disease (AD). This is partly due to insulin resistance in the brain. Insulin signaling and the number of insulin receptors may decline in the brain of T2DM patients, resulting in impaired synaptic formation, neuronal plasticity, and mitochondrial metabolism. In AD patients, hypometabolism of glucose in the brain is observed before the onset of symptoms. Amyloid-ß accumulation, a main pathology of AD, also relates to impaired insulin action and glucose metabolism, although ketone metabolism is not affected. Therefore, the shift from glucose metabolism to ketone metabolism may be a reasonable pathway for neuronal protection. To promote ketone metabolism, medium-chain triglyceride (MCT) oil and a ketogenic diet could be introduced as an alternative source of energy in the brain of AD patients.


Assuntos
Doença de Alzheimer/dietoterapia , Doença de Alzheimer/epidemiologia , Óleo de Coco/uso terapêutico , Diabetes Mellitus Tipo 2/dietoterapia , Diabetes Mellitus Tipo 2/epidemiologia , Dieta Cetogênica/métodos , Óleo de Palmeira/uso terapêutico , Doença de Alzheimer/metabolismo , Peptídeos beta-Amiloides/metabolismo , Animais , Comorbidade , Diabetes Mellitus Tipo 2/metabolismo , Metabolismo Energético , Glucose/metabolismo , Humanos , Insulina/metabolismo , Resistência à Insulina , Cetonas/metabolismo
6.
Molecules ; 25(13)2020 Jun 30.
Artigo em Inglês | MEDLINE | ID: mdl-32629796

RESUMO

The feasibility of the use of two lipid sources and their impact on the cannabinoid profile, terpene fingerprint, and degradation products in medical cannabis oil preparations during 3 months of refrigerated storage time were investigated. LCHRMS-Orbitrap® and HS-SPME coupled to GC-MS for the investigation of targeted and untargeted cannabinoids, terpenes, and lipid degradation products in Bedrocan® and Bediol® macerated oils were used as analytical approaches. As regards the cannabinoid trend during 90 days of storage, there were no differences between PhEur-grade olive oil (OOPH) and medium-chain triglycerides oil (MCT oil) coupled to a good stability of preparations for the first 60 days both in Bedrocan® and Bediol® oils. MCT lipid source extracted a significant concentration of terpenes compared to olive oil. Terpenes showed a different scenario since MCT oil displayed the strongest extraction capacity and conservation trend of all compounds during the shelf life. Terpenes remained stable throughout the entire storage period in MCT formulations while a significant decrease after 15 and 30 days in Bediol® and Bedrocan® was observed in olive oil. Therefore, MCT oil could be considered a more suitable lipid source compared to olive oil involved in the extraction of medical cannabis for magistral preparations.


Assuntos
Canabinoides/análise , Cannabis/química , Maconha Medicinal/análise , Extratos Vegetais/química , Óleos de Plantas/química , Triglicerídeos/química , Maconha Medicinal/química
7.
Cureus ; 16(6): e62556, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-39027784

RESUMO

Background and objective Butter coffee drinks, mainly a form of a saturated fat diet, are widely accepted as a "healthy energy-boosting drink", especially in the young and healthy military population. The objective of our study was to determine the effects of medium-chain triglyceride (MCT) oil and butter on lipid profile, especially apolipoprotein B (ApoB), low-density lipoprotein (LDL)-cholesterol (LDL-C), high-density lipoprotein (HDL)-cholesterol (HDL-C), and other risk factors for coronary heart disease, such as BMI, BP, fasting blood glucose, HbA1c, and high-sensitivity C-reactive protein (hs-CRP) levels in healthy adults. Materials and methods We conducted a prospective study of 60 subjects who were randomized to one of the two following regimens: (1) coffee or (2) coffee with butter plus MCT oil combination. The primary outcome was the effect on ApoB. Secondary outcomes were as follows: non-HDL-C, LDL-C, triglycerides, BP, waist circumference, fasting blood glucose, and HbA1c. These parameters were evaluated at the baseline and after 12 weeks. The Mann-Whitney U test was utilized for analysis of the results. Results While 60 subjects were recruited for the study, only 41 completed it, meeting the minimum required sample size (17 per group) necessary to achieve the desired effect size: 21 males (nine in the control group and 12 in the experimental group) and 20 females (10 in each group). Anthropometric measures were similar between the two groups at baseline, and so were age and BMI (average age: 33.00 ± 5.84 years among controls and 30.86 ± 6.14 years in the experimental group; BMI: 27.35 ± 4.63 kg/m2 vs. 25.74 ± 2.70 kg/m2). The pulse rate was 69.35 ± 10.98 in the control vs. 70.68 ± 10.32 bpm in the experimental group. The waist size was also similar in both groups. Baseline lab findings were as follows: ApoB: 89.85 ± 17.52 (control), 81.60 ± 12.84 mg/dL (experimental); hs-CRP: 0.18 ± 0.27 (control), 0.17 ± 0.27 mg/L (experimental); LDL-C 113.65 ±23.71 (control), 106.50 ± 18.99 mg/dL (experimental); HDL-C 57.35 ± 14.63 (control), 62.41 ± 16.15 mg/dL (experimental); and triglycerides: 76.00 ± 31.30 (control), 56.77 ± 14.77 mg/dL (experimental), and these values were similar. The values after 12 weeks of intervention were as follows: BMI: 27.37 ± 5.24 (control), 26.36 ± 3.55 (experimental); pulse rate: 78.88 ± 14.00 (control), 74.20 ± 11.90 bpm (experimental); ApoB 87.1 ± 17.38 (control), 85.7 ±20.59 mg/dL (experimental); hs-CRP 0.26 ± 0.22 (control), 0.15 ± 0.14 mg/L (experimental); LDL-C 111.59 ± 20.35 (control), 114.10 ± 26.99 mg/dL (experimental); HDL-C 57.71 ± 12.93 (control), 64.85 ± 13.32 mg/dL (experimental); and triglycerides: 74.71 ± 25.39 (control), 60.80 ± 15.77 mg/dL (experimental). Conclusion At a significance level of 5%, there was no difference between the two groups, either at the baseline or at 12 weeks of intervention. Based on our findings, adding MCT oil and butter to coffee may be safe. However, further studies with larger sample sizes and longer duration are needed to validate our findings.

8.
Foods ; 13(7)2024 Apr 02.
Artigo em Inglês | MEDLINE | ID: mdl-38611395

RESUMO

Glycogen storage diseases (GSDs) are a group of carbohydrate metabolism disorders, most of which are inherited in autosomal recessive patterns. GSDs are of two types: those that have to do with liver and hypoglycaemia (hepatic GSDs) and those that are linked to neuromuscular presentation. This study aims to assess the impact of dietary intervention, including medium-chain triglyceride (MCT) oil, on anthropometric measurements, body composition analysis and metabolic parameters among Jordanian children and is expected to be the first in the country. A sample of 38 children with glycogen storage disease type 1 (GSD-1) (median age = 6.4 years) were on a diet that included uncooked cornstarch therapy and a fructose-, sucrose- and lactose-restricted diet. Patients started to take MCT oil along with the prescribed diet after the first body composition test. Patients' nutritional status was re-evaluated three months later. The study results show that the percentage of patients who suffered from hypoglycaemia at the beginning of the study decreased significantly from 94.7% to 7.9% (p < 0.0001). The serum levels of triglycerides, cholesterol, uric acid and lactate decreased significantly after three months of intervention (100-71.1%, 73.7-21.1%, 97.4-52.6% and 94.7-18.4%, respectively). In contrast, there was no statistical difference in neutrophil count. Regarding clinical parameters, liver span was significantly reduced from (16.01 ± 2.65 cm) to (14.85 ± 2.26 cm) (p < 0.0001). There were significant improvements in growth parameters, including height-for-age and BMI-for-age for children aged ≥2 years (p = 0.034 and p = 0.074, respectively). Significant improvements in skeletal muscle mass and bone mineral content were also noticed at the end of the trial (p ≤ 0.05). In conclusion, medium-chain triglyceride therapy is found to improve biochemical and growth parameters in children with GSD-1 in Jordan.

9.
Food Res Int ; 174(Pt 1): 113595, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37986458

RESUMO

The health benefits of curcumin have been demonstrated by several clinical studies, but its low bioavailability compromises its functionality. In this regard, emulsions have proven to be effective encapsulation systems for curcumin. Nevertheless, emulsions with a high oil content (50%) may offer some advantages due to the large amount of compound they can incorporate. Therefore, the aim of this work was to study the pharmacokinetics and biodistribution of curcumin when carried in optimized emulsions containing 50% MCT oil and a plant-based emulsifier (soybean lecithin) at 2 h or 4 h post-oral administration to rats. The most stable emulsion was obtained using 50% of oil and a surfactant-oil-ratio 0.1, through a microfluidization process. After the oral administration of the systems (150 mg curcumin/kg body weight), curcumin glucuronide was the main compound present in plasma (AUC0-t = 1556.3 ng·h·ml-1), especially at 2-4 h post-administration. The total curcuminoid bioavailability was increased by 10.6-fold when rats were fed with the curcumin emulsion rather than with a control suspension. Moreover, rats fed with the emulsion showed the highest accumulation of free curcuminoids, which present the highest biological activity, in the liver (129 ng curcumin/g tissue) and brown adipose tissue (193 ng curcumin/g tissue). The obtained results are of great interest since the presence of curcumin in the brown adipose tissue has been shown to play a relevant role in the prevention of obesity and its related metabolic disorders.


Assuntos
Curcumina , Ratos , Animais , Emulsões , Distribuição Tecidual , Emulsificantes , Diarileptanoides , Triglicerídeos
10.
J Oleo Sci ; 72(1): 87-97, 2023 Jan 07.
Artigo em Inglês | MEDLINE | ID: mdl-36504191

RESUMO

The fatty acid composition of coconut oil was modified using enzyme catalyzed interesterification with the aim of obtaining a product more alike to commercial MCT oils. This modification was carried out with the aim to obtain a product with some of the health benefits shown by MCT oils. Initially, lipase B from Candida antarctica immobilized on acrylic resin and lipozyme TL IM were tested as enzyme catalysts for the reaction. The enzyme catalysts have shown similar performance and lipozyme TL IM has been chosen as the catalyst based on its lower cost. The effects of reaction time, oil to methyl octanoate ratio, and enzyme loading on the reaction performance have been investigated with response surface methodology (RSM) utilizing the Box-Behnken approach. The optimized reaction was scaled up to 20 g. The possibility to source the medium chain fatty acid esters from coconut oil fatty acid distillate using a simple procedure was demonstrated and the possibility to use these esters for the interesterification of coconut oil has been demonstrated as well.


Assuntos
Ácidos Graxos , Óleos de Plantas , Óleo de Coco , Esterificação , Ésteres , Catálise
11.
Nutr Rev ; 81(9): 1144-1162, 2023 08 10.
Artigo em Inglês | MEDLINE | ID: mdl-36633304

RESUMO

CONTEXT: In preclinical Alzheimer's disease (AD), the brain gradually becomes insulin resistant. As a result, brain glucose utilization is compromised, causing a cellular energy deficit that leads to the accumulation of free radicals, which increases inflammation and damages neurons. When glucose utilization is impaired, ketone bodies offer an alternative energy source. Ketone bodies are synthesized from fats, obtained from either the diet or adipose tissue. Dietary medium-chain fatty acids (MCFAs), which are preferentially metabolized into ketone bodies, have the potential to supply the insulin-resistant brain with energy. OBJECTIVE: This systematic review and meta-analysis aims to review the effect of MCFA supplements on circulating ketone bodies and cognition in individuals with subjective cognitive decline, mild cognitive impairment, and AD. DATA SOURCES: A comprehensive search of electronic databases was performed on August 12, 2019, to retrieve all publications meeting the inclusion criteria. Alerts were then set to identify any publications after the search date up until January 31, 2021. DATA EXTRACTION: Data were extracted by 2 authors and assessed by a third. In total, 410 publications were identified, of which 16 (n = 17 studies) met the inclusion criteria. DATA ANALYSIS: All studies assessing change in levels of blood ketone bodies due to MCFA supplementation (n = 12) reported a significant increase. Cognition outcomes (measured in 13 studies), however, varied, ranging from no improvement (n = 4 studies) to improvement (n = 8 studies) or improvement only in apolipoprotein E allele 4 (APOE ε4) noncarriers (n = 2 studies). One study reported an increase in regional cerebral blood flow in APOE ε4 noncarriers and another reported an increase in energy metabolism in the brain. CONCLUSION: MCFA supplementation increases circulating ketone body levels, resulting in increased brain energy metabolism. Further research is required to determine whether this MCFA-mediated increase in brain energy metabolism improves cognition. SYSTEMATIC REVIEW REGISTRATION: PROSPERO registration number CRD42019146967.


Assuntos
Doença de Alzheimer , Humanos , Doença de Alzheimer/prevenção & controle , Apolipoproteína E4 , Ácidos Graxos/metabolismo , Corpos Cetônicos/metabolismo , Corpos Cetônicos/uso terapêutico , Insulina , Glucose/metabolismo
12.
Gels ; 8(11)2022 Oct 31.
Artigo em Inglês | MEDLINE | ID: mdl-36354613

RESUMO

Medium Chain Triglyceride (MCT) oil was successfully combined with Glyceryl Monostearate (GMS) and Glyceryl Monoolein (GMO) to form oleogels that were subsequently whipped to form stable oleofoams. The co-crystallization of GMS and GMO at a ratio of 20:1, 20:2.5, and 20:5 within MCT oil was studied through Differential Scanning Calorimetry (DSC), X-ray Diffraction analysis (XRD), rheological analysis, Fluorescence Recovery after Photobleaching (FRAP), Fourier Transform Infrared Spectroscopy (FTIR), and polarized microscopy. The addition of 5% GMO resulted in the production of more stable oleogels in terms of crystal structure and higher peak melting point, rendering this formulation suitable for pharmaceutical applications that are intended to be used internally and those that require stability at temperatures close to 40 °C. All formulations were whipped to form oleofoams that were evaluated for their storage stability for prolonged period at different temperatures. The results show that oleofoams containing 5% MGO retained their foam characteristics even after 3 months of storage under different temperature conditions.

13.
J Frailty Aging ; 11(1): 100-108, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-35122097

RESUMO

OBJECTIVES: Supplementation with 6 g/day of medium-chain triglycerides (MCTs) at dinnertime increases muscle function and cognition in frail elderly adults relative to supplementation with long-chain triglycerides. However, suitable timing of MCT supplementation during the day is unknown. DESIGN: We enrolled 40 elderly nursing home residents (85.9 ± 7.7 years) in a 1.5-month randomized intervention trial. Participants were randomly allocated to two groups: one received 6 g/day of MCTs at breakfast (breakfast group) as a test group and the other at dinnertime (dinner group) as a positive control group. MEASUREMENTS: Muscle mass, strength, function, and cognition were monitored at baseline and 1.5 months after initiation of intervention. RESULTS: Thirty-seven participants completed the study and were included in the analysis. MCT supplementation in breakfast and dinner groups respectively increased right arm muscle area from baseline by 1.1 ± 0.8 cm2 (P<0.001) and 1.6 ± 2.5 cm2 (P<0.001), left arm muscle area by 1.1 ± 0.7 cm2 (P<0.001) and 0.9 ± 1.0 cm2 (P<0.01), right knee extension time by 39 ± 42 s (P<0.01) and 20 ± 32 s (P<0.05), leg open and close test time by 1.74 ± 2.00 n/10 s (P<0.01) and 1.67 ± 2.01 n/10 s (P<0.01), and Mini-Mental State Examination score by 1.5 ± 3.0 points (P=0.06) and 1.0 ± 2.1 points (P=0.06). These increases between two groups did not differ statistically significantly. CONCLUSION: Supplementation with 6 g MCTs/day for 1.5 months, irrespective of ingestion at breakfast or dinnertime, could increase muscle mass and function, and cognition in frail elderly adults.


Assuntos
Cognição , Idoso Fragilizado , Idoso , Suplementos Nutricionais , Humanos , Força Muscular , Músculos , Triglicerídeos
14.
Indian J Hum Genet ; 17(1): 29-32, 2011 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-21814341

RESUMO

Very long chain acyl-CoA dehydrogenase (VLCAD) deficiency is one of the genetic defects of mitochondrial fatty acid beta-oxidation presenting in early infancy or childhood. If undiagnosed and untreated, VLCAD deficiency may be fatal, secondary to cardiac involvement. We assessed the effect of replacing part of the fat in the diet of a 2 ½-month-old male infant, who was diagnosed with VLCAD deficiency,with medium-chain triglyceride (MCT) oil and essential fats. The patient presented with vomiting, dehydration, and was found to have persistent elevation of liver function tests, hepatomegaly, pericardial and pleural effusion, right bundle branch block, and biventricular hypertrophy. Because of the cardiomyopathy, hepatomegaly, and an abnormal acylcarnitine profile and urine organic acids, he was suspected of having VLCAD deficiency. This was confirmed on acyl-coA dehydrogenase, very long chain (ACADVL) gene analysis. He was begun on an MCT oil-based formula with added essential fatty acids, uncooked cornstarch (around 1 year of age), and frequent feeds. By 7 months of age, cardiomyopathy had reversed and by 18 months of age, all cardiac medications were discontinued and hypotonia had improved such that physical therapy was no longer required. At 5 years of age, he is at the 50(th) percentile for height and weight along with normal development. Pediatricians need to be aware about the basic pathophysiology of the disease and the rationale behind its treatment as more patients are being diagnosed because of expansion of newborn screen. The use of MCT oil as a medical intervention for treatment of VLCAD deficiency remains controversial mostly because of lack of clear phenotype-genotype correlations, secondary to the genetic heterogeneity of the mutations. Our case demonstrated the medical necessity of MCT oil-based nutritional intervention and the need for the further research for the development of specific guidelines to improve the care of these patients.

15.
Rinsho Shinkeigaku ; 61(3): 200-203, 2021 Mar 25.
Artigo em Japonês | MEDLINE | ID: mdl-33627582

RESUMO

A 49-year-old woman with intellectual disability and a food preference for fried chicken entered a nursing home. After nursing home diet, she developed episodic attacks of hyperammonemic encephalopathy. Her characteristic food preference and the negative results for brain and liver imaging studies suggested urea cycle disorder. A high plasma citrulline level on amino acid analysis and a genetic test for citrine gene confirmed a citrine deficiency (adult-onset type II citrullinemia). Although a low-carbohydrate diet was insufficient, a combination therapy of a low-carbohydrate diet and a medium-chain triglyceride (MCT) oil was effective. MCT oil may be a promising treatment option.


Assuntos
Citrulinemia/tratamento farmacológico , Citrulinemia/etiologia , Casas de Saúde , Triglicerídeos/administração & dosagem , Citrulinemia/diagnóstico , Citrulinemia/genética , Diagnóstico Diferencial , Feminino , Heterozigoto , Humanos , Pessoa de Meia-Idade , Proteínas de Transporte da Membrana Mitocondrial/genética , Mutação , Óleos , Resultado do Tratamento , Triglicerídeos/química
16.
Food Chem ; 341(Pt 2): 128155, 2021 Mar 30.
Artigo em Inglês | MEDLINE | ID: mdl-33045587

RESUMO

This study investigated the impact of adding ß-carotene on the structure of fresh O/W emulsions with different oil phase (sunflower oil-LCT or NEOBEE®1053-MCT) and emulsifiers (WPI, Tween 80 - T80 or WPI/T80 mixture). In this sense, the behavior of emulsions through the gastrointestinal tract, the stability and bioaccessibility of ß-carotene were also assessed. The ß-carotene reduced the interfacial tension of the LCT/MCT-water systems. The addition of ß-carotene promoted an increase of viscoelasticity of LCT/MCT-T80 (0.5%WPI/0.5%T80 and 1%T80 w/w) interfaces, but an increase of WPI content reduced the viscoelasticity of interfacial layers (LCT/MCT-1% WPI). These changes in the interface properties influenced the mean droplet size and ζ-potential of the fresh emulsions. LCT systems presented similar bioaccessibility/stability of ß-carotene. However, ß-carotene entrapped within protein-coated MCT droplets was more stable than within T80-MCT systems. Our results show that ß-carotene interacted with other ingredients of emulsions changing their properties and behavior under gastrointestinal tract as well as the stability/bioaccessibility of ß-carotene.


Assuntos
Emulsões/química , Polissorbatos/química , beta Caroteno/química , Disponibilidade Biológica , Digestão , Emulsificantes/química , Óleo de Girassol , Tensão Superficial , Viscosidade , Água/química , Proteínas do Soro do Leite/química , beta Caroteno/farmacocinética
17.
Foods ; 10(4)2021 Mar 25.
Artigo em Inglês | MEDLINE | ID: mdl-33805896

RESUMO

High-moisture extrusion is a common process to impart an anisotropic, meat-like structure to plant proteins, such as wheat gluten. The addition of oil during the process promises to enhance the sensory properties of the meat analogs. In this study, the influence of oil on extrusion-relevant parameters as well as the structure-related characteristics of extruded wheat gluten was investigated. Oil was added directly to the extruder at different contents (0, 2, 4, 6%) and addition points (front/end of the extruder barrel). Process conditions, complex viscosity, Young's modulus and oil phase morphology were determined as a function of oil content and oil addition point. With increasing oil content, material temperature, die pressure, and complex viscosity decreased. The addition of oil at the end of the extruder barrel reduced this effect compared to the addition of oil in the front part of the extruder. It was observed that the extrudate's tensile strength is a function of material temperature, resulting in an increase in tensile strength with increasing material temperature. The oil was dispersed in the gluten matrix as small droplets with irregular shape. As the oil content increased, the size of the oil droplets increased, while the addition of oil at the end of the extruder resulted in a decrease in droplet size.

18.
Food Res Int ; 137: 109360, 2020 11.
Artigo em Inglês | MEDLINE | ID: mdl-33233063

RESUMO

The engineering of ingredients emerges as a strategy to design emulsified products aiming to control the lipid hydrolysis. In this context, oil-in-water (O/W) emulsions composed of different oil phases (Sunflower oil - LCT or NEOBEE® 1053 - MCT) and stabilized by whey protein isolate - WPI (1% w/w), Tween 80 - T80 (1% w/w) or varied ratios of WPI/T80 (0.9975%WPI/0.0025%T80; 0.75%WPI/0.25%T80; 0.5%WPI/0.5%T80 w/w) were produced and submitted to simulated gastrointestinal conditions. The lipolysis of LCT was influenced by the fatty acid chain length and emulsifier composition, while only the fatty acid chain length affected the lipolysis of MCT. The emulsions produced with LCT and 1%WPI or 09975%WPI/00025%T80 showed the highest release rate of free fatty acids (FFAs), but similar result was observed for the 0.5%WPI/0.5%T80 system. In the 0.5%WPI/0.5%T80 mixture, WPI and T80 worked together and achieved an improved performance during the gastric (stability similar as 1%T80 emulsion) and small intestinal phases (lipolysis similar as 1%WPI emulsion). The rational selection of ingredients is useful to design emulsions with improved performance as a delivery system since the emulsion structural stability during digestion, the oil type and interaction between lipase-interface had a marked impact on the efficiency of lipid digestion.


Assuntos
Emulsificantes , Tensoativos , Emulsões , Polissorbatos , Proteínas do Soro do Leite
19.
J Pediatr Pharmacol Ther ; 25(3): 220-227, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32265605

RESUMO

OBJECTIVES: Growth failure following surgical palliation of complex congenital heart defects (CHDs) is a prognosticator of poor outcomes. Many strategies for improving weight gain have been implemented in this population, with limited success. We recently described the potential of the anabolic steroid oxandrolone to improve weight gain following surgical repair of CHD when administered via a medium-chain triglyceride (MCT) oil suspension to the buccal mucosa. The current study evaluates the stability of oxandrolone in the MCT oil formulation, as well as the pharmacokinetics of oxandrolone when administered via buccal mucosa in both neonates and adults. METHODS: Stability was assessed by long-term storage of the preparation 1) at ambient conditions and 2) under photodegradative conditions for 3 days. Neonatal pharmacokinetic parameters were determined in a cohort of neonates following surgical CHD repair, whereas adult pharmacokinetics parameters were collected as part of a prospective study to evaluate the relative bioavailability of the oxandrolone in MCT oil formulation. RESULTS: We found that oxandrolone was stable in the MCT oil formulation for at least 1 month, although exposure to light hastened drug degradation. Both neonatal and adult oxandrolone pharmacokinetics were variable; however, oxandrolone in MCT oil was relatively well absorbed through the buccal mucosa (mean bioavailability = 62.5%). CONCLUSIONS: These data suggest that the variability in oxandrolone exposures is inherent to the drug, and not the formulation or route of administration. Combined, these data support further study of this novel oxandrolone in MCT oil formulation and its impact on growth following complex surgical repair of CHD in neonates.

20.
Int J Pharm ; 556: 9-20, 2019 Feb 10.
Artigo em Inglês | MEDLINE | ID: mdl-30529659

RESUMO

Coenzyme Q10 (CoQ10) is a mitochondrial respiratory cofactor and potent endogenous antioxidant. In CoQ10-deficient patients, early treatment with high-oral doses (5-50 mg/kg/day) can limit the progression of renal disease and the onset of neurological manifestations. Crystalline CoQ10 is lipophilic, water-insoluble, and poorly absorbed in the gut. Here, CoQ10 showed low bulk density, another important disadvantage in solid oral formulations. Thus, we propose the use of oleogels to maintain dissolved a high-dose of CoQ10 in medium-chain triglyceride (MCT) oil, using ethylcellulose (EC) for gelling, and a surfactant (sorbitan monostearate -SMS- or lecithin). "True gels" were only obtained with the surfactant presence. Thermoreversible oleogels with 1 g of dissolved CoQ10 per 5 g-disk were successfully developed with proved stability and solubility for 12 months (25.0 °C). SMS was better than lecithin as a surfactant because it allowed lower syneresis, higher CoQ10 retention for 12 months, and notably higher oxidative-stability of the MCT-oil, best immobilized by its true gel network. Plastic deformation without fracture was determined under compression, emulating the soft deformation behavior inside the mouth. SMS-oleogels allowed loading a maximal solubilized CoQ10 dose with maximal stability, and may be easier to swallow by CoQ10-deficient patients who suffer from secondary dysphagia.


Assuntos
Antioxidantes/administração & dosagem , Celulose/análogos & derivados , Tensoativos/química , Ubiquinona/análogos & derivados , Administração Oral , Antioxidantes/química , Celulose/química , Química Farmacêutica/métodos , Relação Dose-Resposta a Droga , Estabilidade de Medicamentos , Hexoses/química , Lecitinas/química , Compostos Orgânicos , Solubilidade , Triglicerídeos/química , Ubiquinona/administração & dosagem , Ubiquinona/química
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