Nutrition management guideline for very-long chain acyl-CoA dehydrogenase deficiency (VLCAD): An evidence- and consensus-based approach.

The nutrition management guideline for very-long chain acyl-CoA dehydrogenase deficiency (VLCAD) is the fourth in a series of web-based guidelines focusing on the diet treatment for inherited metabolic disorders and follows previous publication of guidelines for maple syrup urine disease (2014), phenylketonuria (2016) and propionic acidemia (2019). The purpose of this guideline is to establish harmonization in the treatment and monitoring of individuals with VLCAD of all ages in order to improve clinical outcomes. Six research questions were identified to support guideline development on: nutrition recommendations for the healthy individual, illness management, supplementation, monitoring, physical activity and management during pregnancy. This report describes the methodology used in its development including review, critical appraisal and abstraction of peer-reviewed studies and unpublished practice literature; expert input through two Delphi surveys and a nominal group process; and external review from metabolic physicians and dietitians. It includes the summary statements of the nutrition management recommendations for each research question, followed by a standardized rating based on the strength of the evidence. Online, open access of the full published guideline allows utilization by health care providers, researchers and collaborators who advise, advocate and care for individuals with VLCAD and their families and can be accessed from the Genetic Metabolic Dietitians International (https://GMDI.org) and Southeast Regional Genetics Network (https://southeastgenetics.org/ngp) websites.

A randomized, placebo-controlled, double-blind trial of supplemental docosahexaenoic acid on cognitive processing speed and executive function in females of reproductive age with phenylketonuria: A pilot study.

Low blood docosahexaenoic acid (DHA) is reported in patients with phenylketonuria (PKU); however, the functional implications in adolescents and adults are unknown. This pilot study investigated the effect of supplemental DHA on cognitive performance in 33 females with PKU ages 12-47 years. Participants were randomly assigned to receive DHA (10mg/kg/day) or placebo for 4.5 months. Performance on cognitive processing speed and executive functioning tasks was evaluated at baseline and follow up. Intention-to-treat and per protocol analyses were performed. At follow up, biomarkers of DHA status were significantly higher in the DHA-supplemented group. Performance on the cognitive tasks and reported treatment-related adverse events did not differ. While no evidence of cognitive effect was seen, a larger sample size is needed to be conclusive, which may not be feasible in this population. Supplementation was a safe and effective way to increase biomarkers of DHA status (www.clinicaltrials.gov; Identifier: NCT00892554).

Experimental evaluation of antidepressant effect of Vacha (Acorus calamus) in animal models of depression.

Depression is a common diagnosis throughout India. It is one of the major sequelae of modern lifestyle which is full of stress. Several drugs and therapies have been tried but a safe and effective treatment of depressive illness is yet not fully established. The main objective of this experimental study on animal models is to evaluate the antidepressant action of plant drug Vacha (Acorus calamus). The behavioral study was conducted and at the same time 5-HT receptor involvement was evaluated. The experimental study was done in rats to evaluate their Open Field Behavior (OFB), High Plus Maze (HPM) activity and 5-hydroxytryptamine (5-HT) receptor syndrome, before and after feeding Vacha. Concurrent Vacha administration in the depression model prevented the development of behavioral deficit in ambulation and rearing due to stress. Similarly, in High Plus Maze Test (HPMT), exploratory activity of rat was restored with Vacha administration. In adopted model of depression, when the animal was subjected to Vacha administration, the behavioural deficit was prevented very well as compared to stressed group. While eliciting the 5-HT syndrome, only two components out of five were influenced by Vacha, indicating that Vacha does not sensitize postsynaptic 5-HT1A receptors, which explains the behavioral deficit prevention in stressed rat group. Vacha definitely has antidepressant effects in animal model of depression.

Nutritional management of patients with urea cycle disorders.

The nutritional management of patients with urea cycle disorders (UCDs) involves restriction of dietary protein along with provision of adequate protein-free energy, essential amino acid supplements, and vitamins and minerals in combination with nitrogen-scavenging drugs. The present paper discusses nutrition therapy for a range of circumstances: during an acute hyperammonaemic episode and at hospital discharge; before, during, and after surgery; and for lifelong chronic management of UCDs.

Possible Correlates of Free Radicals and Free Radical Mediated Disorders in Ayurveda with Special Referance to Bhutagni Vyapara and Ama at molecular Level.

The description of metabolic processes, operating at various levels insider the body, has been essentially covered in Ayurveda under thirteen types of Agnis and their functions at different levels, which are often compared with the enzymes and biochemical which take part in biological and / or biophysical transformations and reactions. When these Agnis, at different levels, get disordered they lead to the production of certain undesired elements or byproducts in the system, which are called as 'Ama' in ayurveda and are considered as very important morbid factor responsible for causation of a variety of diseases and playing key role in genesis of most of the diseases.The present article attempts to correlate the most recent concept of today's medicine i.e., Free Radical concept with that of the concept of Agni and Ama, described in Ayurveda and thereby opens newer vistas of search for remedies from Ayurvedic research, which may be helpful in the prevention and care of Free Radical Mediated Diseases.

Ayurvedic herbal drugs with possible cytostatic activity.

Ayurveda is considered to be the traditional science of health in India and is based on the principle of subjectivity. All matter is composed of five basic elements, which can be perceived by the five sense organs. All food and drugs are classified according to their pharmacological properties, which are derived from these five elements. To investigate which Ayurvedic plants might have cytostatic activity, an Ayurvedic model for the pathogenesis of cancer was made. Based on this, selection criteria were formed, that were used to select plants from a list of Ayurvedic herbal drugs. Some of the selected species could be collected in India and Nepal. The dried material of 14 species was submitted to ethanol (70% v/v) extraction and the extracts were tested for cytotoxicity on COLO 320 tumour cells, using the microculture tetrazolium (MTT) assay. The IC50-value, the concentration causing 50% growth inhibition of the tumour cells, was used as a parameter for cytotoxicity. Extracts of the flowers of Calotropis procera (Ait.) R. Br. (Asclepiadaceae) and of the nuts of Semecarpus anacardium L.f. (Anacardiaceae) displayed the strongest cytotoxic effect with IC50-values of 1.4 micrograms/ml and 1.6 micrograms/ml, respectively. The extracts of several other plants did not show a cytotoxic effect up to 100 micrograms/ml, the highest concentration tested.

Therapeutic potential of Satvavajaya therapy in the management of Amavata.

Amavata which is popularly correlated with Rheumatoid disease, is characterised by its eternal course, uncertain progression and unpredictable series of exacerbations and remissions. In Ayurveda the impact of the Amavata over psychological setup of the patient is well recognised. Satvavajaya therapy of Ayurveda may be considered as a kind of patient education in rheumatology but with much broader base and much better applicability.

Concept of deha prakriti vis-à-vis human constitution in ayurveda.

The Ayurvedic literature exhibits a systematic information about psychosomatic constitution and its genetic & environment denominators. According to Ayurveda the psychosomatic constitution, as also known as Deha Prakrti, is represented by a vivid description of the Physique, Physiology & psychological make-up of an individual. The Deha Prakrti is essentially genetically determined and is likely to be influenced by a variety of environmental factors to some extent. Dehaprakrti is the foremost factor which determines the pattern of susceptibility of an individual to different diseases, their prognosis, course and complications.

The concept of aging in ayurveda.

The present study reveals that the process of aging was elaborately described in Ayurveda. In order to prology the life span and to promote longevity a separate discipline called Rasayana Tantra was also developed. The sailent feature of this discipline are described in this article.

Ayurvedic concept of hridroga its present relevance.

Hridroga is among those diseases mentioned in Ayurveda where in the description is quite brief and the Ayurvedic view point needs clarification. In view of the increasing evidence of cardiac disorders in the present times this aspect assumes added importance. Among the five types of Hridroga described, in Vatika variety Ayurveda seems to have conceived the disease entity that comes under Ischemic Heart Disease. None of the other Cardiac afflictions seem to have been described under Hridroga. It seems that a few of the presenting features based on this Acute/important/predominant nature have been described Vatika Sotha and Swasa. It looks as though pain dominating heart disease is conceived as Hridroga. When oedema is found to be the main presenting feature it is kept under Vatika sotha and when Dysponea is the main symptom it is described as Swasa. The present article undertakes to discuss this point in detail, based on Ayurvedic concepts as well as current knowledge on medicine.

A critical study of the concept of iaschaemic heart disease in ayurveda.

In this era of Antibiotics decline in infectious diseases is seen but degenerative diseases have established themselves all over world, among which Atherosclerosis and Ischemic Heart Diseases are considered as greatest killer. In view of the increasing morbidity and mortality by these diseases and in absence of safety and adequate efficacy of modern drugs, it has become a timely necessity to understand disease process properly and to explore traditional indigenous system of medicine. So that drugs stated useful in ancient literature for these diseases can be used for I.H.D. Vatika Hrcchula described in Ayurveda simulates with Ischemic Heart Diseases.

A randomized, controlled trial of treatment of alcoholic hepatitis with parenteral nutrition and oxandrolone. II. Short-term effects on nitrogen metabolism, metabolic balance, and nutrition.

Patients with moderately severe or severe alcoholic hepatitis, described in a companion paper in this issue, had serial studies of energy and protein metabolism and elemental balances before and during treatment for 21 days with one of four randomly assigned regimens: 1) standard therapy, consisting of abstinence, a balanced, nutritionally adequate diet, and multivitamins; 2) oxandrolone (20 mg orally four times a day) plus standard therapy; 3) nutritional supplementation, consisting of 2 liters of 3.5% crystalline amino acids in 5% dextrose given by peripheral vein (PPN) plus standard therapy; and 4) a combination of the other three treatments. Dietary and intravenous intakes and weights were recorded daily, and weekly averages were calculated. Anthropometric measurements and blood studies were done weekly; blood studies included white blood cell counts and differentials, serum prealbumin, transferrin, and total protein and plasma aminograms. Four-days complete balance studies and measures of 15N,1-13C-leucine metabolism also were performed at baseline and after the treatment period. Major findings were as follows: a) Intakes of total calories and protein were significantly higher in PPN-treated than in other groups. b) All patients had positive elemental balances, both at baseline and at the end of the treatment period. However, those treated with PPN (with or without oxandrolone) had higher positive balances of nitrogen, potassium, phosphorus, and magnesium, indicating improvement in lean body mass. c) Anthropometric measurements showed no significant changes, but measures of the visceral protein compartment (serum prealbumin, transferrin, total protein, total lymphocyte count) improved significantly with time. For most of these variables, increases were significantly greater in those treated with PPN with or without oxandrolone than in the other groups. However, for prealbumin, the increase was greatest in the oxandrolone-treated group d) PPN treatment produced dramatic increases in levels of branched-chain amino acids and improvement in the ratio of plasma branched chain to aromatic amino acids. Other treatments had no effect on plasma aminograms. e) Metabolism of 15N,1-13C-leucine was normal and was not affected significantly by treatment. Therapy with PPN and/or oxandrolone was tolerated well. We conclude that PPN has favorable effects on energy and protein metabolism in florid alcoholic hepatitis; oxandrolone has lesser effects, although it may exert some additional action and particularly increases serum prealbumin levels. The results support the use of nutritional supplementation in therapy of moderately severe or severe alcoholic hepatitis.

An experimental evaluation of possible teratogenic potential in Boerhaavia diffusa in Albino rats.

The present study was undertaken to evaluate any possibility of teratogenic effects in Boerhaavia diffusa (Punarnava), a widely used herbal medicine for renal and urinary tract diseases by Ayurvedic physicians in India. The ethanolic extract of Boerhaavia diffusa (BDE) was administered daily in a dose of 250 mg/kg, body weight p.o., to pregnant albino female rats during the entire period of gestation. BDE was found to be devoid of any teratogenic effect as litter size and survival rate of foetuses were the same as for the normal control group and no foetal anomaly could be detected.

Perspective in plant drug research.

Plant medicine becomes more popular thought the world in these days probably of its latest toxicity. Ayurveda that leads in plant medicine preserves the natural form of herbs maximum, in various pharmaceutical preparations. Isolation of active principles has not proved much fruitful. However, the need of hour is cultivation of more medicinal plants along with popularization of their use traditional simpler forms.