Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020505 (hyperphagia)
 6,116 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Marked weight loss is the major nutritional defect in chronic pancreatitis. Inadequate food intake owing to recurrent or near continuous pain usually accounts for the initial 10 to 20 per cent of loss of body weight, which decreases again with the onset of diabetes and is often precipitous with the development of steatorrhea. Treatment of pain, control of diabetes, and intensive pancreatic replacement therapy for steatorrhea usually causes weight gain, but seldom to ideal weight. It appears that the patient's body weight gets set at a new "weight-stat." Although isolated abnormalities of small bowel function tests can be elicited and deficiencies of fat-soluble vitamins, calcium, zinc, selenium, and so forth may be demonstrated, these rarely lead to clinical syndromes, as with demonstrable low B12 uptake in some 10 to 15 per cent of patients. In the late stage of the disease and particularly in NATP, extreme protein-calorie malnutrition may occur, which may not be correctable even by hyperalimentation. Although the mortality of the disease was reportedly higher in areas of socioeconomic deprivation, it appears from recent studies in Switzerland and other developed countries that mortality during a 12-year period may be in the region of 50 per cent worldwide.
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PMID:Nutritional deficiencies in chronic pancreatitis. 268 Sep 66

Plasma selenium levels were determined at various intervals during hospitalization of 71 patients with upper gastrointestinal and other malignancies. These patients often require frequent nutritional as well as surgical or medical intervention. Attempts were made to identify, evaluate, and compensate for numerous confounding variables at each of the 374 plasma selenium determinations. Selenium levels in stable patients who were neither receiving aggressive antineoplastic therapy, nor septic, nor taking corticosteroids and who had no clinically significant metabolic imbalance were then separately analyzed. In 55 stable patients selenium levels were 28% lower than those found in 20 normal controls (mean 61.8 micrograms/L, P less than 0.0005). An analysis of all the readings showed that selenium levels were substantially decreased by recent radiotherapy or sepsis, by regional tumor spread and increased tumor burden, and by intravenous and/or enteral hyperalimentation and intravenous lipids. In contrast to these findings, levels were relatively higher in patients with an adequate oral diet or with a lesser tumor burden. The comparison between selenium levels in stable and in aggressively treated or septic patients supports the importance of the relationship of nutrition to selenium levels in cancer patients.
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PMID:Plasma selenium levels in patients with advanced upper gastrointestinal cancer. 344 Feb 35

We studied three children with chronic gastrointestinal disease who had been on intravenous hyperalimentation for periods of time ranging from 4 to 23 months. Each child was found to have low plasma and red blood cell glutathione peroxidase activity. This was associated, in the two children tested, with a marked deficiency of serum selenium. Their plasma glutathione peroxidase levels ranged between 4 and 24% of normal and their red blood cell levels ranged between 4 and 14% of normal. The intravenous alimentation was then supplemented with sodium selenite (240 micrograms Se/d). Within 4-5 weeks, the plasma glutathione peroxidase activity returned to normal. Red cell glutathione peroxidase activity remained essentially unchanged for 4-6 weeks, after which it increased over the following 3-4 months. Red cells were separated by density on a continuous Percoll-diatrizoate gradient. In normal individuals, the specific activity of glutathione peroxidase did not differ across the gradient despite a 2.5-fold difference in the specific activity of pyruvate kinase. When studied initially, glutathione peroxidase activity from the deficient patients did not change across the gradient. As the red cell enzyme activity increased with selenium repletion, the highest specific activity was initially found at the top of the gradient (youngest cells). After 3-4 months of supplementation, the specific activity became equal across the gradient. Thus, with selenium repletion, there is a rapid increase in plasma glutathione peroxidase activity, a 4-6 week lag prior to an increase in red cell enzyme activity, and the increase in red cell activity is due to newly synthesized red cells made in the presence of selenium.
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PMID:Selenium repletion and glutathione peroxidase--differential effects on plasma and red blood cell enzyme activity. 392 Aug 95

Selenium status was determined on seven patients receiving long-term intravenous hyperalimentation (IVH) and they were followed monthly for 4 to 17 months. Analysis of the IVH solutions for selenium revealed no detectable amounts. The selenium indices measures were (1) erythrocyte selenium levels, (2) erythrocyte glutathione peroxidase activities (GSH-Px), and (3) plasma selenium levels. In general, the IVH population had significantly lower values (p less than 0.001) for these selenium indices than a healthy population (n = 275). The plasma selenium levels decreased to below the normal range within one to two months after initiation of IVH. The below normal ranges varied by patient: three patients by three months, one patient by nine months, and three patients had low and normal values throughout the study. No consistent correlation of individual patient's erythrocyte GSH-Px activities and erythrocyte selenium levels existed, but a positive correlation (r = 0.51, p less than 0.01) occurred between plasma selenium levels and erythrocyte GSH-Px activities. Thus, erythrocyte GSH-Px activities decrease parallel to decreases in plasma selenium levels but not with changes in erythrocyte selenium levels. These data suggest that some of these IVH patients may be at risk for selenium deficiency and that selenium supplementation of IVH solutions may be required. Furthermore, these data suggest that the measurement of erythrocyte GSH-Px activity or selenium levels in some IVH patients may not be related to available body selenium.
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PMID:Selenium status of seven chronic intravenous hyperalimentation patients. 681 71

Branched-chain ketoacid dehydrogenase deficiency results in complex and volatile metabolic derangements that threaten brain development. Treatment for classical maple syrup urine disease (MSUD) should address this underlying physiology while also protecting children from nutrient deficiencies. Based on a 20-year experience managing 79 patients, we designed a study formula to (1) optimize transport of seven amino acids (Tyr, Trp, His, Met, Thr, Gln, Phe) that compete with branched-chain amino acids (BCAAs) for entry into the brain via a common transporter (LAT1), (2) compensate for episodic depletions of glutamine, glutamate, and alanine caused by reverse transamination, and (3) correct deficiencies of omega-3 essential fatty acids, zinc, and selenium widespread among MSUD patients. The formula was enriched with LAT1 amino acid substrates, glutamine, alanine, zinc, selenium, and alpha-linolenic acid (18:3n-3). Fifteen Old Order Mennonite children were started on study formula between birth and 34 months of age and seen at least monthly in the office. Amino acid levels were checked once weekly and more often during illnesses. All children grew and developed normally over a period of 14-33 months. Energy demand, leucine tolerance, and protein accretion were tightly linked during periods of normal growth. Rapid shifts to net protein degradation occurred during illnesses. At baseline, most LAT1 substrates varied inversely with plasma leucine, and their calculated rates of brain uptake were 20-68% below normal. Treatment with study formula increased plasma concentrations of LAT1 substrates and normalized their calculated uptakes into the nervous system. Red cell membrane omega-3 polyunsaturated fatty acids and serum zinc and selenium levels increased on study formula. However, selenium and docosahexaenoic acid (22:6n-3) levels remained below normal. During the study period, hospitalizations decreased from 0.35 to 0.14 per patient per year. There were 28 hospitalizations managed with MSUD hyperalimentation solution; 86% were precipitated by common infections, especially vomiting and gastroenteritis. The large majority of catabolic illnesses were managed successfully at home using 'sick-day' formula and frequent amino acid monitoring. We conclude that the study formula is safe and effective for the treatment of classical MSUD. In principle, dietary enrichment protects the brain against deficiency of amino acids used for protein accretion, neurotransmitter synthesis, and methyl group transfer. Although the pathophysiology of MSUD can be addressed through rational formula design, this does not replace the need for vigilant clinical monitoring, frequent measurement of the complete amino acid profile, and ongoing dietary adjustments that match nutritional intake to the metabolic demands of growth and illness.
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PMID:Classical maple syrup urine disease and brain development: principles of management and formula design. 2006 Nov 71