Nutritional and metabolic support in patients with amyotrophic lateral sclerosis

Amyotrophic lateral sclerosis (ALS) is the most common form of progressive motor neuron disease and the most devastating neurodegenerative disorder. ALS is characterized by progressive paralysis and respiratory failure leading to death within 3 to 5 years after its onset. Protein-energy malnutrition is a frequent finding in ALS. The pathogenesis of protein-energy malnutrition in ALS is multifactorial. Muscle atrophy, hypophagia, dysphagia, and hypermetabolism play a role in determining the deterioration of nutritional status. A multidisciplinary approach is crucial to set an appropriate plan for metabolic and nutritional support in ALS. Nutritional management incorporates a continuous assessment and implementation of dietary modifications throughout the duration of the disease. The nutritional and metabolic approaches to ALS should start when the diagnosis of ALS is made and should become an integral part of the continuous care to the patient, including nutritional surveillance, dietary counseling, management of dysphagia, and enteral nutrition when needed. Parenteral nutrition is rarely indicated. Standard polymeric enteral formulas are routinely used, usually providing 25 to 30 kcal/kg and protein 0.8 to 1.2 g /kg per day. The use of fiber-enriched formulas may help prevent constipation. However, considering the complex metabolic abnormalities of ALS, standard and/or fiber-enriched formulas might not be sufficient to achieve optimal metabolic and nutritional support. Based on the most recent clinical and experimental evidence, it is tempting to hypothesize that personalized nutritional support including specific nutritional substrates could act on disease progression and improve the quality of life and the response to the few and yet scarcely effective, currently available pharmacologic therapies.     

Dietary Lipids and Dyslipidemia in Chronic Kidney Disease

The progression of chronic kidney disease (CKD) leads to altered lipid metabolism. CKD patients exhibit high blood triglyceride (TG) levels, reduced concentrations and functionality of high-density lipoproteins (HDL), and elevated levels of atherogenic small, dense, low-density lipoproteins (sdLDL). Disorders of lipid metabolism and other metabolic disturbances place CKD patients at high risk for cardiovascular disease (CVD). Extensive evidence supports the cardioprotective effects of unsaturated fatty acids, including their beneficial effect on serum cholesterol and TG levels. Dietary lipids might therefore be especially important in the nutritional management of CKD. We review current dietary recommendations for fat intake by CKD patients and suggest potential nutritional interventions by emphasizing dietary lipids that might improve the blood lipid profile and reduce cardiovascular risk in CKD.     

Nutritional risk and the metabolic syndrome in women: opportunities for preventive intervention from the Framingham Nutrition Study

BACKGROUND: Diet is recognized as a key factor in the cause and management of the metabolic syndrome (MetS). However, policies to guide preventive clinical nutrition interventions of the condition are limited. OBJECTIVES: We examined the relation between dietary quality and incident MetS in adult women and identified foci for preventive nutrition interventions. DESIGN: This was a prospective study of 300 healthy women (aged 30-69 y) in the Framingham Offspring-Spouse study who were free of MetS risk factors at baseline. The development of individual MetS traits and overall MetS status during 12 y of follow-up were compared in women by tertile of nutritional risk, based on intake of 19 nutrients. Multivariate logistic regression models considered age, smoking, physical activity, and menopausal status. RESULTS: Baseline age-adjusted mean nutrient intake and ischemic heart disease risk profiles differed by tertile of nutritional risk. Women with higher nutritional risk profiles consumed more dietary lipids (total, saturated, and monounsaturated fats) and alcohol and less fiber and micronutrients; they had higher cigarette use and waist circumferences. Compared with women with the lowest nutritional risk, those in the highest tertile had a 2- to 3-fold risk of the development of abdominal obesity and overall MetS during 12 y of follow-up [odds ratio: 2.3 (95% CI: 1.2, 4.3) and 3.0 (95% CI: 1.2, 7.6), respectively]. CONCLUSIONS: Higher composite nutritional risk predicts the development of abdominal obesity and MetS during long-term follow-up in healthy women, independent of lifestyle and ischemic heart disease risk factors. Preventive nutrition interventions for obesity and MetS risk reduction should focus on the overall nutritional quality of women's dietary profiles.     

Diet and health: scientific concepts and principles conference commentary

The conference concluded that dietary guidelines are needed, but that they cannot apply to everyone in the population nor to the same extent. Furthermore, a mechanism or structure is needed in which the guidelines can be periodically reviewed and updated. In making dietary recommendations, genetic variability, age, sex and body size need to be considered. Survival, extension of life span, relief of selected symptoms and biochemical improvements of risk factors are some of the criteria to be used in assessing the role of diet in health and disease. The most important questions that remain unresolved in the nutritional sciences concern the relation of diet to the development of chronic disease. The science of nutritional epidemiology needs to be strengthened and etiologic research in epidemiology ought to be performed and evaluated with the same standards used in other branches of science. Therefore, there is a need to establish guidelines for the validity of methods in assessing dietary intake and for a minimum acceptable correlation for the reproducibility and repeatability of dietary intake data. Research on the role of genetic variation and nutrition, the metabolic effects of omega-3 fatty acids and their relationship to omega-6 fatty acids, the energy expenditure after weight reduction and improving the scientific base of nutritional epidemiology will enhance the scientific basis of nutrition and our understanding of the role of diet in health and disease.     

Troubles nutritionnels lors de la sclérose latérale amyotrophique (SLA)

Amyotrophic lateral sclerosis (ALS) is a rare neuromuscular disease, with a severe prognostic. Its consequences are not only neurological, but also nutritional, linked with a decrease of feeding, caused by swallowing and salivation disorders, a frequent anorexia or constipation, troubles due to a possible respiratory insufficiency, and sometimes important difficulties for going shopping, cooking and more simply putting the food into the mouth. Although fat-free mass is usually decreased during ALS, a paradoxical hypermetabolism is present in 50 to 70% cases, favouring nutritional alterations. The tools used for assessment, sometimes specific to the disability, are anthropometric criteria, body impedance analysis (BIA), dietetic surveys, dual X-ray absorptiometry and indirect calorimetry. Alimentary intakes, weight, fat-free mass and body mass index are progressively reduced, leading to malnutrition. BIA phase angle is largely decreased, reflecting severe cellular alterations. Disorders of lipid status are possible but inconsistent across studies and perhaps linked with cultural feeding habits. The loss of weight at diagnosis and malnutrition during evolution are independent prognostic factors for survival, justifying early nutritional assessment and care. During evolution of ALS, a higher level of fat mass seems to be a positive factor for survival. French, European and American recommendations give precisions on follow-up, modalities of fight against the causes of malnutrition, nutritional care, mainly including the use of oral energy and protein-enriched nutritional supplements and enteral nutrition.     

Nutrition and Kidney Stone Disease

The prevalence of kidney stone disease is increasing worldwide. The recurrence rate of urinary stones is estimated to be up to 50%. Nephrolithiasis is associated with increased risk of chronic and end stage kidney disease. Diet composition is considered to play a crucial role in urinary stone formation. There is strong evidence that an inadequate fluid intake is the major dietary risk factor for urolithiasis. While the benefit of high fluid intake has been confirmed, the effect of different beverages, such as tap water, mineral water, fruit juices, soft drinks, tea and coffee, are debated. Other nutritional factors, including dietary protein, carbohydrates, oxalate, calcium and sodium chloride can also modulate the urinary risk profile and contribute to the risk of kidney stone formation. The assessment of nutritional risk factors is an essential component in the specific dietary therapy of kidney stone patients. An appropriate dietary intervention can contribute to the effective prevention of recurrent stones and reduce the burden of invasive surgical procedures for the treatment of urinary stone disease. This narrative review has intended to provide a comprehensive and updated overview on the role of nutrition and diet in kidney stone disease.     

Uremic Sarcopenia and Its Possible Nutritional Approach

Uremic sarcopenia is a frequent condition present in chronic kidney disease (CKD) patients and is characterized by reduced muscle mass, muscle strength and physical performance. Uremic sarcopenia is related to an increased risk of hospitalization and all-causes mortality. This pathological condition is caused not only by advanced age but also by others factors typical of CKD patients such as metabolic acidosis, hemodialysis therapy, low-grade inflammatory status and inadequate protein-energy intake. Currently, treatments available to ameliorate uremic sarcopenia include nutritional therapy (oral nutritional supplement, inter/intradialytic parenteral nutrition, enteral nutrition, high protein and fiber diet and percutaneous endoscopic gastrectomy) and a personalized program of physical activity. The aim of this review is to analyze the possible benefits induced by nutritional therapy alone or in combination with a personalized program of physical activity, on onset and/or progression of uremic sarcopenia.     

Relationship between Dietary Fiber Intake and the Prognosis of Amytrophic Lateral Sclerosis in Korea

The gut microbiota has been suggested as an important factor in the pathogenic mechanisms of amyotrophic lateral sclerosis (ALS). This study aimed to investigate whether the intake of different kinds of dietary fiber was related to the disease progression rate (∆FS) and survival time. In total, 272 Korean sporadic ALS patients diagnosed according to the revised EI Escorial criteria were recruited starting in March 2011 and were followed until the occurrence of events or the end of September 2020. The events included percutaneous endoscopic gastrostomy, tracheostomy, and death. Dietary fiber intake was calculated based on a 24-h dietary recall and classified according to five major fiber-rich foods: vegetables, fruits, grains, legumes, and nuts/seeds. Among the total participants, the group with ∆FS values lower than the mean ∆FS (0.75) was noted in the highest tertiles of total and vegetable fiber intake. Participants in the highest tertile for vegetable fiber intake showed longer survival in the Kaplan–Meier analysis (p = 0.033). Notably, vegetable fiber intake was negatively correlated with pro-inflammatory cytokine (interleukin (IL)-1β, IL-6, and monocyte chemoattractant protein-1) levels in the cerebrospinal fluid. This study showed that vegetable fiber intake could influence the disease progression rate and survival time. Further clinical trials are needed to confirm whether dietary fiber supplementation improves the prognosis of ALS.     

营养干预在代谢相关性脂肪肝疾病进展中的作用机制研究进展

代谢紊乱相关性脂肪肝(Metabolic-associated fatty liver disease, MAFLD)被认为是一种包括遗传、生理和环境因素在内的多因素疾病,其中不同的因子在各种通路重叠,导致代谢损伤和肝损伤。MAFLD的主要危险因素是超重/肥胖、胰岛素抵抗/2型糖尿病、高甘油三酯血症和相关的饮食行为诱因,主要是果糖饮料的摄入,坚持地中海饮食是MAFLD患者肝脏脂肪含量变化的重要预测因素。越来越多的证据表明,为MAFLD患者开具已被证明具有保肝作用的特定补充剂或营养保健品处方,以加速改善肝酶和肝脂肪变性的程度,或可预防或延缓MAFLD疾病进展。     

Nonalcoholic fatty liver disease

Nonalcoholic fatty liver disease (NAFLD) refers to the presence of hepatic steatosis not associated with a significant intake of ethanol. Insulin resistance is central to the pathogenesis of NAFLD; thus obesity, diabetes, and the metabolic syndrome are frequently associated with the disease. Consequently, as these metabolic conditions emerge as major health problems in Western society, it is now recognized that NAFLD is the most common chronic liver condition in the Western world. NAFLD is generally asymptomatic, although a minority of patients may present with evidence of progressive liver injury with complications of cirrhosis, liver failure, and hepatocellular carcinoma. Despite being common and potentially serious, relatively little is known about the natural history or prognostic significance of NAFLD. Although diabetes, obesity, and age are recognized risk factors for advanced liver disease, other significant factors leading to progressive liver injury remain to be identified. The treatment of NAFLD focuses upon modifying metabolic risk factors. Insulin-sensitizing and hepatoprotective drugs have been subjected to study trials, but as yet, no agent has conclusively been demonstrated to prevent disease progression. Management is further complicated by the inability to predict which patients will develop liver-related morbidity and thus benefit from treatment.     

Whole-grain intake is inversely associated with the metabolic syndrome and mortality in older adults

BACKGROUND: Whole-grain intake has been inversely associated with the metabolic syndrome in middle-aged populations, but the association has not been investigated in older adults. The metabolic consequence of consuming high whole-grain diets may differ in elderly persons, who are prone to greater insulin resistance and impaired glucose tolerance. OBJECTIVE: The aim of the present study was to examine the cross-sectional association between whole- and refined-grain intake, cardiovascular disease risk factors, prevalence of the metabolic syndrome, and the incidence of cardiovascular disease mortality in the same cohort of older adults. DESIGN: The nutritional status of 535 healthy persons aged 60-98 y was determined from 1981 to 1984. The subjects kept a 3-d food record and had their blood tested for metabolic risk factors. The metabolic syndrome was defined based on criteria set by the third report of the National Cholesterol Education Program. The vital status of the subjects was identified in October 1995. RESULTS: The results showed a significant inverse trend between whole-grain intake and the metabolic syndrome (P for trend = 0.005) and mortality from cardiovascular disease (P for trend = 0.04), independent of demographic, lifestyle, and dietary factors. Fasting glucose concentrations and body mass index decreased across increasing quartile categories of whole-grain intake (P for trend = 0.01 and 0.03, respectively), independent of confounders, whereas intake of refined grain was positively associated with higher fasting glucose concentrations (P for trend = 0.04) and a higher prevalence of the metabolic syndrome (P for trend = 0.01). CONCLUSION: Whole-grain intake is a modifiable dietary risk factor, and older and young adults should be encouraged to increase their daily intake to > or = 3 servings/d.     

Effect of diet on progression of chronic renal disease

Changes in renal function induced by protein intake are thought to reflect evolutionary adaptation of the kidney. Excess dietary proteins over long periods may increase normal blood flow and glomerular filtration rate, requiring the continuous use of outer cortex's reserve glomeruli. According to the hyperfiltration theory, pressures and flows in outer cortical glomeruli contribute to continuous intrarenal capillary hypertension and predispose healthy people to progressive glomerular sclerosis and deterioration of renal function. Pressures and flows associated with the response to renal disease in turn may accelerate the development of sclerosis, leading to even more rapid loss of renal function. Restriction of dietary protein (less than or equal to 0.6 g/kg per day) and/or phosphorus seems to slow the rate of loss of renal function in patients with chronic renal insufficiency. Evidence for the role of lipids in the progression of renal disease is not clear. All of these dietary factors possibly play a role in the progression of renal disease; the relative importance of each factor varies, depending on the pathogenesis, stage, and mechanism of progression of the disease. Findings indicate that nutrition therapy can decrease rate of deterioration of renal function in patients with chronic renal diseases.     

Nutrition and dementia

The nutritional consequences of dementia and the role of diet in the etiology, treatment, and prevention of dementia are the subjects of this review. The major cause of dementia is Alzheimer's disease. Although it has been suggested that aluminum intake may cause this disease, the bulk of scientific evidence suggests that this is unlikely. Dietary supplements of choline and lecithin have been used to treat Alzheimer's disease but are ineffective. Alzheimer's disease patients are at risk of developing protein-energy malnutrition because of poor food intake and increased energy requirements. The second most common cause of dementia is multi-infarct dementia, caused by multiple strokes. Diet may play a role in the prevention of this form of dementia through effects on blood pressure and other risk factors. Control of risk factors may also prevent further progression of the dementia. Patients with multi-infarct dementia often have dysphagia. The third most common cause of dementia appears to be excessive alcohol intake, due both to the direct neurotoxic effects of alcohol and to the effects of alcohol on nutritional status. Alcoholic dementia may be at least partially reversible with abstinence and good nutrition. Other causes are vitamin B-12 and folate deficiencies; these are reversible dementias. In all types of dementia, adequate nutrition may improve physical well-being, help maximize the patient's functioning, and improve the quality of life.     

Gout and Diet: A Comprehensive Review of Mechanisms and Management

Gout is well known as an inflammatory rheumatic disease presenting with arthritis and abnormal metabolism of uric acid. The recognition of diet-induced systemic metabolic pathways have provided new mechanistic insights and potential interventions on gout progression. However, the dietary recommendations for gouty patients generally focus on food categories, with few simultaneous considerations of nutritional factors and systemic metabolism. It is worthwhile to comprehensively review the mechanistic findings and potential interventions of diet-related nutrients against the development of gout, including purine metabolism, urate deposition, and gouty inflammation. Although piecemeal modifications of various nutrients often provide incomplete dietary recommendations, understanding the role of nutritional factors in gouty development can help patients choose their healthy diet based on personal preference and disease course. The combination of dietary management and medication may potentially achieve enhanced treatment effects, especially for severe patients. Therefore, the role of dietary and nutritional factors in the development of gout is systematically reviewed to propose dietary modification strategies for gout management by: (1) reducing nutritional risk factors against metabolic syndrome; (2) supplementing with beneficial nutrients to affect uric acid metabolism and gouty inflammation; and (3) considering nutritional modification combined with medication supplementation to decrease the frequency of gout flares.     

Dietary patterns and the metabolic syndrome in obese and non-obese Framingham women

OBJECTIVES: To examine the relationship between habitual dietary patterns and the metabolic syndrome (MetS) in women and to identify foci for preventive nutrition interventions. RESEARCH METHODS AND PROCEDURES: Dietary patterns, nutrient intake, cardiovascular disease (CVD), and MetS risk factors were characterized in 1615 Framingham Offspring-Spouse Study (FOS) women. Dietary pattern subgroups were compared for MetS prevalence and CVD risk factor status using logistic regression and analysis of covariance. Analyses were performed overall in women and stratified on obesity status; multivariate models controlled for age, apolipoprotein E (APOE) genotypes, and CVD risk factors. RESULTS: Food and nutrient profiles and overall nutritional risk of five non-overlapping habitual dietary patterns of women were identified including Heart Healthier, Lighter Eating, Wine and Moderate Eating, Higher Fat, and Empty Calories. Rates of hypertension and low high-density lipoprotein levels were high in non-obese women, but individual MetS risk factor levels were substantially increased in obese women. Overall MetS risk varied by dietary pattern and obesity status, independently of APOE and CVD risk factors. Compared with obese or non-obese women and women overall with other dietary patterns, MetS was highest in those with the Empty Calorie pattern (contrast p value: p<0.05). DISCUSSION: This research shows the independent relationship between habitual dietary patterns and MetS risk in FOS women and the influence of obesity status. High overall MetS risk and the varying prevalence of individual MetS risk factors in female subgroups emphasize the importance of preventive nutrition interventions and suggest potential benefits of targeted behavior change in both obese and non-obese women by dietary pattern.     

Nutrition and immune function in human immunodeficiency virus infection

The triad of human immunodeficiency virus (HIV) infection, nutritional status and immune function are intimately related, each factor having effects on the others. The dominant effect in this three-way relationship is the effect of HIV infection on nutritional status, an effect which, until the advent of potent anti-retroviral drugs, has been manifest primarily as wasting. Recently, more complex metabolic abnormalities have become apparent, particularly fat redistribution syndromes, hyperlipidaemia and hypercholesterolaemia. For the converse effect, the effect of nutritional state on HIV disease progression, there is good evidence that clinical outcome is poorer in individuals with compromised nutrition. However, the beneficial effects of nutritional support have been more difficult to demonstrate. For macronutrients, effective macronutrient supply improves survival in severely-malnourished individuals and may have beneficial effects in less-severely-affected individuals. Micronutrient deficiencies appear to be involved in modifying clinical HIV disease and may also be associated with enhanced mother-to-child transmission of virus, particularly in developing countries. Intervention trials in this setting are currently under way. In conclusion, the interaction of HIV infection and nutrition is of great importance not just because of the major impact that HIV infection has on nutritional state, but also because strategies to improve nutritional status, both quantitatively and qualitatively, may have a beneficial effect on the clinical and immunological course of the disease.     

The Role of Vitamin Deficiency in Liver Disease: To Supplement or Not Supplement?

Over the past few years, growing interest has been shown for the impact of dietary requirements and nutritional factors on chronic diseases. As a result, nutritional programs have been reinforced by public health policies. The precise role of micronutrients in chronic liver disease is currently receiving particular attention since abnormalities in vitamin levels are often detected. At present, treatment programs are focused on correcting vitamin deficiencies, which are frequently correlated to higher rates of comorbidities with poor outcomes. The literature reviewed here indicates that liver diseases are often related to vitamin disorders, due to both liver impairment and abnormal intake. More specific knowledge about the role of vitamins in liver disease is currently emerging from various results and recent evidence. The most significant benefits in this area may be observed when improved vitamin intake is combined with a pharmacological treatment that may also affect the progression of the liver disease, especially in the case of liver tumors. However, further studies are needed.