Use of Branched-Chain Amino Acids as a Potential Treatment for Improving Nutrition-Related Outcomes in Advanced Chronic Liver Disease.

Advanced chronic liver disease (ACLD) represents a complex and multifactorial clinical entity characterized by liver dysfunction and associated complications. In recent years, the significance of nutritional status in ACLD prognosis has gained considerable attention. This review article delves into the multifactorial pathogenesis of malnutrition in ACLD and its profound consequences for health outcomes. We explore the clinical implications of secondary sarcopenia in ACLD and highlight the critical relevance of frailty in both decompensated and compensated ACLD. A specific focus of this review revolves around branched-chain amino acids (BCAAs) and their pivotal role in managing liver disease. We dissect the intricate relationship between low Fischer's ratio and BCAA metabolism in ACLD, shedding light on the molecular mechanisms involved. Furthermore, we critically evaluate the existing evidence regarding the effects of BCAA supplementation on outcomes in ACLD patients, examining their potential to ameliorate the nutritional deficiencies and associated complications in this population.

Nutritional status of children affected by X-linked adrenoleukodystrophy.

BACKGROUND: Adrenoleukodystrophy (ALD) is a rare X-linked metabolic disorder that causes the accumulation of very-long-chain fatty acids (VLCFAs) (C26:0) and the subsequent variety of clinical and neurological symptoms. Little is known about nutritional status and dietary habits of children affected by ALD, and so the present study aimed to assess nutritional status and food intake in children with ALD, also exploring the relationship between food intake and the consumption of disease-specific dietary supplements to reduce blood C26:0 concentrations and increase monounsaturated fatty acids (C26:1). METHODS: All patients underwent a clinical and neurological evaluation and a comprehensive nutritional assessment. The association of VLCFA concentrations with dietary lipids was assessed. RESULTS: Nine boys (11.49 ± 3.61 years) were enrolled in a cross-sectional study. All patients were normal weight, with normal resting energy expenditure. Only six of nine patients followed the low-fat diet and dietary supplements. An inverse association was found between the food intake of polyunsaturated lipids and C26:0; conversely, the C26:0 was positively associated with the dietary saturated lipids. When consumed, dietary supplement consumption correlated positively with C26:1 (ρ = 0.917, p = 0.029) and no correlation was found with C26:0 (ρ = 0.410, p = 0,493). CONCLUSIONS: No children were found to be malnourished or overweight or obese; however, half of the children reported excessive body fat, probably as a result of the pharmacotherapies. A low-fat diet could be adjuvant in the management of the accumulation of VLCFAs, but poor dietary compliance to disease-specific nutritional guidelines appears to be a major problem of this condition and underlines the need for a structured and personalised nutritional management in ALD disease.

Moringin, A Stable Isothiocyanate from Moringa oleifera, Activates the Somatosensory and Pain Receptor TRPA1 Channel In Vitro.

Moringa oleifera Lam. is a tropical plant widely used in traditional medicines and as a food supplement. It is characterized by the presence of glucosinolates and isothiocyanates; the stable isothiocyanate 4-[(α-l-rhamnosyloxy)benzyl]isothiocyanate (moringin) has been widely studied for its bioactivity as hypoglycemic, antimicrobial, anticancer and in particular for its involvement in nociception and neurogenic pain. Moringa extracts and pure moringin were submitted to in vitro assays with the somatosensory TRPA1 ion channel, proving that moringin is a potent and effective agonist of this receptor involved in nociceptive function and pain states. Moringin do not activate or activates very weakly the vanilloids somatosensory channels TRPV1,2,3 and 4, and the melastatin cooling receptor TRPM8. The comparison of moringin's activity with other known agonists of natural origin is also discussed.

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

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

Impact of the Ketogenic Diet on Linear Growth in Children: A Single-Center Retrospective Analysis of 34 Cases.

Data on the impact of the ketogenic diet (KD) on children's growth remain controversial. Here, we retrospectively investigated the occurrence of linear growth retardation in 34 children (47% males; age range: 2-17 years) diagnosed with drug-resistant epilepsy (DRE; n = 14) or glucose transporter type 1 deficiency syndrome (GLUT1-DS; n = 20) who had been treated with the KD for 12 months. The general characteristics of children with and without growth retardation were also compared. All participants received a full-calorie, traditional KD supplemented with vitamins, minerals, and citrate. Most children (80%; 11/14 in the DRE subgroup and 16/20 in the GLUT1-DS subgroup) treated with the KD did not show growth retardation at 12 months. Although participants with and without delay of growth did not differ in terms of baseline clinical characteristics, dietary prescriptions, or supplementation patterns, marked ketosis at 12 months tended to occur more frequently in the latter group. Altogether, our results indicate that growth retardation may occur in a minority of children treated with the KD. However, further research is required to identify children at risk and to clarify how increased ketones levels may affect endocrine pathways regulating growth during KD administration.

Nutritional characterisation of Zambian Moringa oleifera: acceptability and safety of short-term daily supplementation in a group of malnourished girls.

In Zambia, chronic malnutrition still is one of the most common problem among children. To fight against malnutrition, the easiest short-term solution could be to combine specific types of food with affordable local plants. A large variety of natural food resources grow in Zambia, such as Moringa oleifera (MO), whose leaves are known for their health benefits, but are not consumed much by local populations. We analysed Zambian MO powder obtained from dried leaves and found that it contains large amounts of protein, minerals and vitamins, such as iron, calcium and carotenoids. These characteristics make MO a good and sustainable complementary solution to malnutrition. We also evaluated the acceptability and the safety of dietary supplementation with MO powder in malnourished children for 30 days. A daily dose of 14 g daily was safe and well accepted. Its regular use in the menu of local populations may be viable proposition.

Effect of Moringa oleifera Leaf Powder on Postprandial Blood Glucose Response: In Vivo Study on Saharawi People Living in Refugee Camps.

The hypoglycemic effect in humans of Moringa oleifera (MO) leaf powder has, to date, been poorly investigated. We assessed the chemical composition of MO leaf powder produced at Saharawi refugee camps, its in vitro ability to inhibit α-amylase activity, and its sensory acceptability in food. We then evaluated its effect on postprandial glucose response by randomly administering, on 2 different days, a traditional meal supplemented with 20 g of MO leaf powder (MOR20), or not (control meal, CNT), to 17 Saharawi diabetics and 10 healthy subjects. Capillary glycaemia was measured immediately before the meal and then at 30 min intervals for 3 h. In the diabetic subjects the postprandial glucose response peaked earlier with MOR20 compared to CNT and with lower increments at 90, 120, and 150 min. The mean glycemic meal response with MOR20 was lower than with CNT. The healthy subjects showed no differences. Thus, MO leaf powder could be a hypoglycemic herbal drug. However, given the poor taste acceptability of the 20 g MO meal, lower doses should be evaluated. Moreover, the hypoglycemic effects of MO leaf powder should also be demonstrated by trials evaluating its long-term effects on glycaemia.

Adherence to the Mediterranean Dietary Pattern and Incidence of Nephrolithiasis in the Seguimiento Universidad de Navarra Follow-up (SUN) Cohort.

BACKGROUND: Diet plays an important role in the pathogenesis of nephrolithiasis. Limited data are available to investigate the association between a Mediterranean dietary pattern and risk for nephrolithiasis. STUDY DESIGN: Prospective cohort study. SETTING & PARTICIPANTS: 16,094 men and women without a history of nephrolithiasis who participated in the Seguimiento Universidad de Navarra Follow-up (SUN) Project. PREDICTORS: A validated 136-item food frequency questionnaire was used to assess baseline adherence to a Mediterranean dietary pattern that is high in fruits, vegetables, nuts, fish, and legumes, but moderate in alcohol and low in meats, saturated fats, and sugars. A Mediterranean dietary pattern score was calculated and categorized into 3 groups (0-3, 4-6, and 7-9 points). Additional factors included in statistical models were sex, age, body mass index, smoking, physical activity, time spent watching television, following a medical nutritional therapy, water and energy intake, calcium and vitamin D supplementation, and history of hypertension or diabetes. OUTCOMES: Incidence of nephrolithiasis. Participants were classified as having incident nephrolithiasis if they reported a physician-made diagnosis of nephrolithiasis during follow-up. RESULTS: After a mean follow-up of 9.6 years, 735 new cases of nephrolithiasis were identified. The multivariable HRs of nephrolithiasis for the 2 highest categories of adherence to the Mediterranean dietary pattern, using the lowest category as the reference, were 0.93 (95% CI, 0.79-1.09) and 0.64 (95% CI, 0.48-0.87); P for trend=0.01. The risk for nephrolithiasis was lower with greater consumption of dairy products and vegetables and greater with higher monounsaturated fatty acid to saturated fatty acid ratio. LIMITATIONS: No information for kidney stone composition. CONCLUSIONS: Greater adherence to a Mediterranean dietary pattern was associated with reduced risk for incident nephrolithiasis. Additional longitudinal studies are needed.

Moringa oleifera Seeds and Oil: Characteristics and Uses for Human Health.

Moringa oleifera seeds are a promising resource for food and non-food applications, due to their content of monounsaturated fatty acids with a high monounsaturated/saturated fatty acids (MUFA/SFA) ratio, sterols and tocopherols, as well as proteins rich in sulfated amino acids. The rapid growth of Moringa trees in subtropical and tropical areas, even under conditions of prolonged drought, makes this plant a reliable resource to enhance the nutritional status of local populations and, if rationalized cultivation practices are exploited, their economy, given that a biodiesel fuel could be produced from a source not in competition with human food crops. Despite the relatively diffuse use of Moringa seeds and their oil in traditional medicine, no pharmacological activity study has been conducted on humans. Some encouraging evidence, however, justifies new efforts to obtain clear and definitive information on the benefits to human health arising from seed consumption. A critical review of literature data concerning the composition of Moringa oil has set in motion a plan for future investigations. Such investigations, using the seeds and oil, will focus on cultivation conditions to improve plant production, and will study the health effects on human consumers of Moringa seeds and their oil.

Nutritional Characterization and Phenolic Profiling of Moringa oleifera Leaves Grown in Chad, Sahrawi Refugee Camps, and Haiti.

Moringa oleifera is a plant that grows in tropical and subtropical areas of the world. Its leaves are rich of nutrients and bioactive compounds. However, several differences are reported in the literature. In this article we performed a nutritional characterization and a phenolic profiling of M. oleifera leaves grown in Chad, Sahrawi refugee camps, and Haiti. In addition, we investigated the presence of salicylic and ferulic acids, two phenolic acids with pharmacological activity, whose presence in M. oleifera leaves has been scarcely investigated so far. Several differences were observed among the samples. Nevertheless, the leaves were rich in protein, minerals, and ß-carotene. Quercetin and kaempferol glycosides were the main phenolic compounds identified in the methanolic extracts. Finally, salicylic and ferulic acids were found in a concentration range of 0.14-0.33 and 6.61-9.69 mg/100 g, respectively. In conclusion, we observed some differences in terms of nutrients and phenolic compounds in M. oleifera leaves grown in different countries. Nevertheless, these leaves are a good and economical source of nutrients for tropical and sub-tropical countries. Furthermore, M. oleifera leaves are a source of flavonoids and phenolic acids, among which salicylic and ferulic acids, and therefore they could be used as nutraceutical and functional ingredients.

Cultivation, Genetic, Ethnopharmacology, Phytochemistry and Pharmacology of Moringa oleifera Leaves: An Overview.

Moringa oleifera is an interesting plant for its use in bioactive compounds. In this manuscript, we review studies concerning the cultivation and production of moringa along with genetic diversity among different accessions and populations. Different methods of propagation, establishment and cultivation are discussed. Moringa oleifera shows diversity in many characters and extensive morphological variability, which may provide a resource for its improvement. Great genetic variability is present in the natural and cultivated accessions, but no collection of cultivated and wild accessions currently exists. A germplasm bank encompassing the genetic variability present in Moringa is needed to perform breeding programmes and develop elite varieties adapted to local conditions. Alimentary and medicinal uses of moringa are reviewed, alongside the production of biodiesel. Finally, being that the leaves are the most used part of the plant, their contents in terms of bioactive compounds and their pharmacological properties are discussed. Many studies conducted on cell lines and animals seem concordant in their support for these properties. However, there are still too few studies on humans to recommend Moringa leaves as medication in the prevention or treatment of diseases. Therefore, further studies on humans are recommended.

Adherence to the Mediterranean diet is inversely related to binge eating disorder in patients seeking a weight loss program.

BACKGROUND & AIM: The key factors influencing the development of Binge Eating Disorder (BED) are not well known. Adherence to the Mediterranean diet (MD) has been suspected to reduce the risk of several mental illnesses such as depression and anxiety. There are no existing studies that have examined the relationships between BED and MD. METHODS: Cross-sectional study of 1472 participants (71.3% women; mean age: 44.8 ± 12.7) at high risk of BED. A MD score (MED-score) was derived from a validated food frequency questionnaire and BED by Binge Eating Scale questionnaire (BES). Body mass index, waist circumference and total body fat (%) were assessed by anthropometric measurements. RESULTS: 376 (25.5%) cases of self reported BED were identified. 11.1% of participants had a good adherence to MD (MED-score ≥ 9). After adjustments for age, gender, nutritional status, education, and physical activity level, high MED-score was associated with lower odds for BED (odds ratios and 95% confidence intervals of a BED disorder for successive levels of MED-score were 1 (reference), 0.77 (0.44, 1.36), 0.66 (0.37, 1.15), 0.50 (0.26, 0.96), and 0.45 (0.22, 0.55) (P for trend: <0.01). Olive oil and nut consumption were associated with low risk of BED development whereas butter, cream, sweets and commercial bakery/sweets/cakes consumption increased the risk. We did not find a cumulative effect of depression and anxiety on MED-score in binge eaters. CONCLUSIONS: These results demonstrate an inverse association between MD and the development of BED in a clinical setting among subjects at risk of BED. Therefore, we should be cautious about generalizing the results to the whole population, although reverse causality and confounding cannot be excluded as explanation. Further prospective studies are warranted.

Relevance of Mediterranean diet and glucose metabolism for nephrolithiasis in obese subjects.

BACKGROUND: Nephrolithiasis is more frequent and severe in obese patients from different western nations. This may be supported by higher calcium, urate, oxalate excretion in obese stone formers. Except these parameters, clinical characteristics of obese stone formers were not extensively explored. AIMS: In the present paper we studied the relationship between obesity and its metabolic correlates and nephrolithiasis. MATERIALS AND METHODS: We studied 478 Caucasian subjects having BMI ≥ 25 kg/m². The presence of nephrolithiasis, hypertension, diabetes mellitus and metabolic syndrome were noted. They underwent measurements of anthropometry (BMI and waist circumference, body composition), serum variables (fasting glucose, serum lipids and serum enzymes) and Mediterranean diet (MedDiet) nutritional questionnaire. RESULTS: 45 (9.4%) participants were stone formers. Subjects with high serum concentrations of triglycerides (≥ 150 mg/dl), fasting glucose (> 100 mg/dl) and AST (>30 U/I in F or >40 U/I in M) were more frequent among stone formers than non-stone formers.Multinomial logistic regression confirmed that kidney stone production was associated with high fasting glucose (OR = 2.6, 95% CI 1.2-5.2, P = 0.011), AST (OR = 4.3, 95% CI 1.1-16.7, P = 0.033) and triglycerides (OR = 2.7, 95% CI 1.3-5.7, P = 0.01). MedDiet score was not different in stone formers and non-stone formers. However, stone formers had a lower consumption frequency of olive oil and nuts, and higher consumption frequency of wine compared with non-stone formers. CONCLUSIONS: Overweight and obese stone formers may have a defect in glucose metabolism and a potential liver damage. Some foods typical of Mediterranean diet may protect against nephrolithiasis.

Phaseolus vulgaris extract affects glycometabolic and appetite control in healthy human subjects.

Extracts of Phaseolus vulgaris (beans) are known to reduce glycaemia and food intake in rodents and humans. The present study evaluated the effects of a new, standardised and purified P. vulgaris extract (PVE), when employed as a supplement in a mixed balanced meal (60 % carbohydrates, 25 % lipids and 15 % protein), on glycometabolic and appetite control. To this end, a randomised, double-blind, placebo-controlled study was performed in twelve volunteers. Plasma glucose, insulin, C-peptide, ghrelin and satiety sensation ratings were assessed at baseline and during 3 h after meal consumption associated with PVE (100 mg) or placebo. Compared with placebo, PVE consumption resulted in lower increments in glucose (+15·4 (sem 5·4) v. 26·1 (SEM 7·3) %, P= 0·04 at 30 min), insulin (+981 (SEM 115) v. 1325 (SEM 240) %, P= 0·04 between 45 and 120 min) and C-peptide (+350 (SEM 27) v. 439 (SEM 30) %, P= 0·04 between 30 and 90 min). In the first 2 h, plasma ghrelin decreased similarly in both groups but did not rebound as in placebo thereafter (P= 0·04). Correspondingly, satiety sensation in the third hour was significantly reduced in the placebo but not in the PVE condition. PVE induced a lower desire to eat than placebo (P= 0·02) over the 3 h. In conclusion, PVE supplementation reduced postprandial glucose, insulin and C-peptide excursions, suppressed ghrelin secretion and affected satiety sensations, inducing a lower desire to eat. These results support that further studies are needed to prove the concept of employing PVE as a supplement in mixed balanced meals in obese, glucose-intolerant and diabetic subjects.

Effects on body composition of different short-term rehabilitation programs in long-stay hospitalized elderly women.

BACKGROUND AND AIMS: Long-stay hospitalized elderly women usually reduce daily living activities: this may lead to an accelerated worsening of body composition, cardio-metabolic condition and falls risk. Exercise training and electrical stimulation may ameliorate such condition. METHODS: The effects of 3 different short-term rehabilitation programs on lower limbs (kinesitherapy [KT], electrical stimulation [ES], KT combined with ES [KT+ES], 3 day/week for 6 weeks) were assessed on 40 hospitalized elderly women (82±7 yrs, 59.5±12.3 kg, 152±7 cm [m±SD]; n=10 for each intervention group and n=10 as control subjects [no intervention]). Segmental (upper and lower limbs) and total body fat mass (FM), fat free mass (FFM), bone mineral content (BMC) and density (BMD), resting energy expenditure (REE), maximum voluntary force (MVC) of leg extensor muscles and cardiorespiratory performance (6-min walking test [6MVT]) were evaluated before and after intervention. RESULTS: Compared to baseline, the distance covered by the 6MWT significantly increased after all rehabilitation programs (from 247±79 to 271±87 m, p<0.01), whereas significant increases in leg extensor muscles MVC were found after ES (+26%) and KT+ES (+16%) only, p<0.05. No changes in segmental and total body FM, FFM and REE were observed, whereas legs BMC significantly increased (2.7%) after KT only (p<0.05). CONCLUSIONS: Although all treatments increased cardiorespiratory performance, and KT and ES ameliorated muscle force, the proposed short-term exercise programs had poor effects on body composition. This suggests that longer programs, maybe combined with a tailored dietary intervention, should be administered to improve body composition in rehabilitating institutionalized elderly people.

Nutrition markers in patients after heart surgery.

BACKGROUND: Nutrition support is an important aspect of multidisciplinary approaches in cardiology rehabilitation. However, little is known about the impact of a patient's nutrition status on recovery after elective heart surgery. The authors assessed changes in nutrition markers in patients undergoing postsurgical rehabilitation, and tested their correlation with systemic inflammatory responses and clinical outcomes to determine the adequacy of the prescribed dietary regimen. METHODS: Plasma concentrations of nutrition biomarkers were measured in 50 nondiabetic patients upon admission to a cardiology rehabilitation unit after coronary artery bypass grafting or mitral and/or aortic valve replacement (D0), and again 16 days later (D16). RESULTS: On D16, low plasma albumin increased, anemia improved, and high levels of inflammation markers declined. Vitamins remained stable within normal values, with the exception of vitamin B12, which decreased significantly from 516 +/- 341 to 445 +/- 212 ng/mL (P = .007). Blood glucose was >110 mg/dL in 51% of the patients at baseline; this proportion did not decline after rehabilitation. Overweight patients (body mass index >25 kg/m(2)) were prevalent (58%). They showed a slightly but not significantly greater inflammatory response and had a higher incidence of infective complications than the normal-weight group, but similar levels of nutrition markers. CONCLUSIONS: The standard dietary regimen followed during cardiological rehabilitation after major cardiovascular surgery aids recovery from surgical insult, but fails to normalize high glucose levels. Vitamin B12 supplementation should be encouraged because of its substantial reduction during the rehabilitation period.

Validation of food frequency questionnaire for assessing dietary macronutrients and calcium intake in Italian children and adolescents.

OBJECTIVES: To validate a food frequency questionnaire to determine calcium and macronutrient intake in healthy children and adolescents. MATERIALS AND METHODS: The questionnaire consisted of 16 main food groups containing the main foods composing the diet of Italian children, a total of 136 items and 23 questions about food habits, supplements and servings. Color photographs to identify serving size were included. The questionnaire was validated by comparing it with the 7-day weighed record method in 37 healthy volunteers in two different age groups: children aged 6 - 10 years (n = 18) and adolescents aged 16 - 20 years (n = 19). RESULTS: The food frequency questionnaire seemed to overestimate the nutrient intake when compared with 7-day weighed records; however the two methods were highly correlated for all the nutrients considered. The range of Pearson's correlations between energy and nutrient intake (protein, fat, carbohydrates and calcium) determined from food frequency questionnaire and 7-day weighed record were 0.5-0.6 in children and 0.5-0.8 in young adults. Agreement between the two methods in classifying relative intakes was examined by determining the frequency of similar classification into terciles. Two thirds of the subjects were correctly classified for most nutrients, with a very low (2% to 5%) percentage of grossly misclassifications. DISCUSSION: The food frequency questionnaire has proved adequate to rank a pediatric population in terms of calcium and macronutrient intakes. The ranking of subjects is extremely useful for epidemiologic studies evaluating the relation between nutrient intake and disease.

[Nutritional status and bone mineral mass in children treated with ketogenic diet]. / Stato nutrizionale e mineralizzazione ossea durante trattamento con dieta chetogenica.

Ketogenic diet (KD) is a high fat (90%), low carbohydrate (3%) diet used to treat refractory seizures in child. This highly unbalance diet could damage nutritional status. The aim of this study is to evaluate if KD can affect on growth and on mineral status in child. Seven child (1 females and 6 males) age between 3-16 years were retrospectively studied to assess nutritional status during KD; we evaluated anthropometric measurements (weight, height, skinfold and circumferences), bone mineral content and bone mineral density, using x-ray energy absorptiometry (DXA) and some biochemical parameters. We have not found any short term modifications (six months) concerning growth, and biochemical parameters studied. KD could worsen bone mineral status.