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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Hepatic encephalopathy (H.E.) is associated with and perhaps caused by changes in plasma-aminoacid patterns--decreased branched-chain aminoacids (B.C.A.A.) and increased aromatic aminoacids (A.A.A.). The decreased B.C.A.A. may be in part secondary to hyperinsulinaemia, but the B.C.A.A. are catabolised by both fat and muscle. The increase in A.A.A. may reflect a "catabolic stimulus" reflected in hyperglucagonaemia, particularly in severe hepatic failure and H.E., and a decreased insulin/glucagon ratio. Endogenous protein, lean body-mass, or liver then releases large amounts of A.A. and the A.A.A. cannot be catabolised by the failing liver, and thus accumulate in the circulation. With decreased plasma-B.C.A.A., the molar ratio of B.C.A.A. and A.A.A. decreases allowing the toxic A.A.A. to penetrate the blood-brain barrier in increased amounts and encephalopathy develops. Appropriate therapy for H.E. must include reversal of the "catabolic state" by providing sufficient B.C.A.A. and calories to decrease the flux of A.A.A. from muscle and liver, and the restoration of the normal molar ratio of B.C.A.A. and A.A.A.
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PMID:Insulin, glucagon, aminoacid imbalance, and hepatic encephalopathy. 6 15

Sepsis is a major catabolic insult resulting in modifications in carbohydrate and fat energy metabolism, and leading to increased muscle breakdown and nitrogen loss. Insulin resistance, which develops in sepsis, decreases glucose utilization, but plasma insulin levels are sufficiently elevated to prevent lipolysis, resulting in a further energy deficit. The availability of fuels in sepsis is therefore limited, and the body resorts to muscle breakdown, gluconeogenesis, and amino acid oxidation for energy supply. Previous work has not defined, however, the exact alterations in amino acid metabolism. Therefore, the following studies were undertaken. Blood samples were drawn from fifteen patients in whom the diagnosis of sepsis was clinically established; the samples were analyzed for amino acid, beta-hydroxyphenylethanolamines, glucose, insulin and glucagon concentrations. The plasma amino acid pattern observed was characterized by an increase in total amino acid content, due mainly to high levels of the aromatic amino acids (phenylalanine and tyrosine) and the sulfur-containing amino acids (taurine, cystine and methionine). Alanine, aspartic acid, glutamic acid and proline were also elevated, but to a lesser degree. The branched chain amino acids (valine, leucine and isoleucine) were within normal limits, as were glycine, serine, threonine, lysine, histidine and tryptophan. Those patients who did not survive sepsis had higher levels of aromatic and sulfur-containing amino acids as compared to those patients surviving sepsis. On the other hand, those patients surviving sepsis had higher levels of alanine and the branched chain amino acids. In a second group of five patients with overwhelming sepsis accompanied by a state of metabolic encephalopathy, a parenteral nutrition solution consisting of 23% dextrose, and an amino acid formulation enriched with branched chain amino acids was administered. In these five patients, normalization of the plasma amino acid pattern and reversal of encephalopathy was observed. The following sequence of events may be postulated: The septic patient develops insulin resistance in the peripheral tissues, primarily muscle, while the adipose tissue is much less affected. The insulin resistance and the inability to utilize fat leads to increased muscle proteolysis. Muscle breakdown results in release into the blood of enormous amounts of various amino acids; the muscle itself is able to oxidize the branched chain amino acids, supplying the muscles' own energy requirements and alanine for gluconeogenesis. The extensive muscle proteolysis coupled with relative hepatic insufficiency occurring early in sepsis results in the appearance in the plasma of high levels of most of the amino acids present in muscle, particularly the aromatic and the sulfur-containing amino acids. The outcome of patients with sepsis might be positively affected by combined therapy with glucose, insulin and branched chain amino acids.
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PMID:Amino acid derangements in patients with sepsis: treatment with branched chain amino acid rich infusions. 9 98

Chromium is required for maintenance of normal glucose tolerance. After complete bowel resection and five months of total parenteral nutrition, severe glucose intolerance, weight loss, and a metabolic encephalopathy-like confusional state developed in a patient. Serum chromium levels were at the lowest normal level. Supplementation of 150 microgram of chromium per day reversed the glucose intolerance, reduced insulin requirements, and resulted in weight gain and the disappearance of encephalopathy. The low levels of chromium and response to chromium supplementation suggest that chromium deficiency can arise in long-term total parenteral nutrition.
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PMID:Chromium deficiency during total parenteral nutrition. 10 57

A case of hyperinsulinism occuring in a newborn, with a birthweight of 4,050 g, is reported. The hypoglycaemia was refractory to the usual therapy (increase of glucose administration per os, and I.V., corticosteroids, glucagon, diazoxide). At surgery, undertaken at 9 days of age, an adenomatous nodule was removed along with the left part of the pancreas. Death occurred at 18 days, after the child had developed a transitory acidoketosic diabetes and an encephalopathy. Measurement of insulin by radio-immunoassay revealed a strong increase in the ratio insulin/glycaemia, characteristic of nesidioblastoma, as well as a high concentration of insulin in the tumor as compared to normal tissue. On the ultrastructural level, the observed features differed from those seen in children and adults and showed an abnormal overload of dense deposits in the cytoplasm of some histiocytes.
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PMID:[Islet cell adenoma with neonatal onset. Clinical, hormonological and ultrastructural study of a case]. 19 46

Alterations in insulin and glucagon levels might account for the plasma amino acid imbalance of cirrhotics. In order to verify this hypothesis we evaluated basal insulin, glucagon, branched-chain amino acids, aromatic amino acids, and free tryptophan in 13 controls and 37 cirrhotics divided on the basis of their mental state; in 4 patients the hormonal and amino acid patterns were sequentially studied during various stages of encephalopathy. Glucagon is high in cirrhotics and progressively increases with the worsening of the mental state. Free tryptophan and aromatic amino acids show a similar behavior and significantly correlate with glucagon levels (r = 0.67 and r = 0.81, respectively). On the other hand insulin levels, which are high in cirrhotics without encephalopathy, fall in the presence of deep coma. Insulin did not correlate with any of the plasma amino acids considered. Our data suggest that the catabolic state associated with increased glucagon levels may account for some of the alterations in the plasma amino acid profiles of cirrhotics. Portal-systemic shunting does not seem to be the common cause of both hyperglucagonemia and hyperaminoacidemia. Decreased branched-chain amino acid levels may be related to factors different from those involved in the alterations of carbohydrate homeostasis.
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PMID:Insulin and glucagon levels in liver cirrhosis. Relationship with plasma amino acid imbalance of chronic hepatic encephalopathy. 46 10

In a series of examinations of more than 1000 psychiatricneurological patients we tried to state the effects of diabetes mellitus on the nervous system. We attempted to diagnose the diabetic metabolic error by means of using the oral glucose tolerance test (100 g glucose), the plasma insulin levels and many other laboratory-technical examinations (altogether about 23 000 individual tests). It was found that 21,6% of the patients had a pathological glucose tolerance, 29.6% of them had a pathological insulin response and 5,5% of them showed a known and manifest diabetes mellitus. The comparison between the number of patients with a metabolic disturbance and the frequency of sick persons to be expected epidemiologically shows that an influence exercised by the diabetic metabolism may be stated in neurological and psychic disturbances of cerebral sclerosis and polyneuropathy. The central syndromes are more frequent than the peripheric ones. Mainly low plasma insulin is present. As a rule, pathological results may be seen more often during pathological glucose tolerance in electroencephalography, pneumencephalography, CSF-examinations, personality and intelligence states. This suggests that a diffuse disturbance upon the nervous system comes from the diabetic metabolism, without any acute metabolic disturbance which we did not find among our patients. This disturbance is evident clinically as so-called diabetic polyneuropathy and also encephalopathy. In opposition to that very often pathological changes can be seen by technical research methods in other neurological and psychiatric diagnoses without influencing essentially the autonomous progress of the psychiatricneurological illness.
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PMID:[Diseases of the central nervous system in diabetes mellitus]. 61 60

Blood substrate and hormone concentration were determined in 16 children with Reye syndrome prior to and following administration of hypertonic glucose. Baseline concentrations of lactate, pyruvate, alanine, glutamine, glutamate, proline, hydroxyproline, lysine, and aspartate were elevated (p less than 0.01), whereas citrulline and arginine were low. All substrate concentrations were below or within the normal range following 36 hours of therapy except those of lactate, pyruvate, and aspartate. Urea nitrogen excretion was reduced (p less than 0.05) on the second day of therapy. Plasma concentrations of insulin and growth hormone increased and glucagon decreased during the first day. Cortisol remained elevated throughout the study period. We conclude that the high circulating concentrations of substrates are the result of both increased mobilization and decreased clearance and that hypertonic glucose infusion suppresses substrate mobilization. A primary abnormality of the mitochondria could explain the metabolic perturbations that occurred. A possible relationship between the encephalopathy in this disorder and an insult to both brain and brain capillary mitochondria is discussed.
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PMID:Metabolic response to hypertonic glucose administration in Reye syndrome. 66 61

This investigation assesses the effect of two-hour activated charcoal hemoperfusion using a column containing 300 g of acrylic hydrogel-coated activated charcoal either alone or combined with hemodialysis on small and "middle molecule" removal in uremic patients. Comparison was made with standard five-hour hemodialysis. Two patients with dialysis encephalopathy were treated with four-hour combined hemoperfusion/hemodialysis without beneficial clinical effects. Hemoperfusion increased the clearance rates of creatinine and urate when combined with dialysis. Hemoperfusion alone removed 1.3 +/- 0.6 g (mean +/- SD) of creatinine and 0.6 +/- 0.2 g of urate, while combined hemoperfusion/hemodialysis removed 1.7 +/- 0.6 g of creatinine and 1.0 +/- 0.5 g of urate in a two-hour period. Both treatment schedules removed less solute than standard five-hour hemodialysis but were associated with comparable "middle molecule" removal. Hemoperfusion accounted for additional amino acid removal when combined with hemodialysis, while hemoperfusion alone produced significant reduction only in the amino acid cystine. Changes in the hormones thyroxine, triiodothyronine, human growth hormone, and insulin were noted during the procedures. Acceptable falls in platelet counts and fibrinogen occurred with hemoperfusion. Coated charcoal hemoperfusion may prove to have a role in the management of uremic patients, although the acrylic hydrogel-coated charcoal hemoperfusion device requires combination with techniques allowing fluid and electrolyte removal.
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PMID:Solute, amino acid, and hormone changes with coated charcoal hemoperfusion in uremia. 68 27

Hypothermia may occur in association with hypoglycemia, and indeed may be the only sign. Two cases are presented. In one, the patient presented with hypoglycemic encephalopathy. In the insulin dependent diabetic, the condition is life-threatening. Subnormal temperature is a clue to hypoglycemia in the alcoholic. The mechanism of hypothermia has been extensively studied, but remains unclear.
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PMID:Hypothermia--a sign of hypoglycemia. 101 86

Reye's syndrome is difined as fatty degeneration of the viscera with encephalopathy. The signs are change in consciousness after recovery of a viral illness, vomiting, high fever, and progresseve deterioration of the level of consciousness. Signs of brain stem involvement quickly follow. The clinical findings are: mild elevations of serum transaminases (SGOT and SGPT), normal to slightly elevated bilirubin, abnormal blood-clotting functions, normal to low blood glucose, high blood ammonia, and normal cerebrospinal fluid which may be under increased pressure. Death is due to increased intracranial pressure. Therapy is used to stabilize these signs. It includes: infusion of hypertonic glucose followed by insulin, peritoneal dialysis, and exchange transfusion. Intracranial pressure was monitored continually to determine when therapy should be administered.
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PMID:Intracranial pressure monitoring in Reye's syndrome. 127 55


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