Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Nutritional and metabolic support in patients with amyotrophic lateral sclerosis. 2267 56

Amyotrophic lateral sclerosis (ALS) is a progressive neurological disease with high risk of malnutrition. Symptoms of dysphagia, depression, cognitive impairment, difficulty with self-feeding and meal preparation, hypermetabolism, anxiety, respiratory insufficiency, and fatigue with meals increase the risk of malnutrition. Malnutrition negatively affects prognosis and quality of life, making early and frequent nutrition assessment and intervention essential. Implementation of an adequate calorie diet, dietary texture modification, use of adaptive eating utensils, and placement of a feeding tube aid in preventing malnutrition. When nutrition status is compromised by dysphagia and weight loss (5%-10% of usual body weight) or body mass index <20 kg/m(2) without weight loss and when forced vital capacity is >50%, a percutaneous endoscopic gastrostomy placement is indicated. When forced vital capacity is <50%, a radiologically inserted gastrostomy is the preferred means of enteral placement due to lessened aspiration and respiratory risk. Parenteral nutrition (PN) is indicated only when enteral nutrition (EN) is contraindicated or impossible. This article reviews the background of ALS, nutrition implications and risk of malnutrition, treatment strategies to prevent malnutrition, the role of EN and PN, and feeding tube placement methods according to disease stage.
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PMID:Nutrition management of amyotrophic lateral sclerosis. 2346 70

Parenteral nutrition is indicated in amyotrophic lateral sclerosis (ALS) when dysphagia, loss of appetite, and difficulty protecting the airways cause malnutrition, severe weight loss, dehydration, and increased risk of aspiration pneumonia. The aim of this review is to compare percutaneous endoscopic gastrostomy (PEG), radiologically inserted G-tube (RIG), and percutaneous radiologic gastrostomy (PRG) in patients with ALS, performed with or without noninvasive ventilation (NIV). We searched PubMed, MEDLINE, EMBASE, the Cochrane Central Register of Controlled Trials (CENTRAL), the EBSCO Online Research Database, and Scopus up to December 2015. A priori selection included all randomized controlled trials (RCTs), quasi-randomized trials, and prospective and retrospective studies. The primary outcome was 30-d survival. We found no RCTs or quasi-RCTs. Seven studies about the implementation of the PEG/RIG procedure during the use of NIV and 5 studies without NIV were included. In another study of 59 subjects undergoing open gastrostomy, all with vital capacity < 30% of normal, 18 of whom were dependent on continuous NIV at full ventilatory support settings, there were no respiratory complications. Thus, the use of NIV during the implementation of these procedures, especially when used at full ventilatory support settings of pressure preset 18-25 cm H2O, can support alveolar ventilation before, during, and after the procedures and prevent respiratory complications. The procedures investigated appear equivalent, but the methodological quality of the studies could be improved. Possible benefits with regard to nutrition parameters, quality of life, and psychological features need to be further investigated.
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PMID:Use of Noninvasive Ventilation During Feeding Tube Placement. 2880 87