Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Low plasma levels of branched-chain amino acids, leucine, isoleucine, and valine are postulated to play an etiologic role in hepatic encephalopathy. Supplementation is advocated to reverse encephalopathy and improve nutritional status and survival. We measured in vivo leucine metabolism in normal individuals (n = 5) and in two groups of patients with cirrhosis (n = 8) with a primed continuous infusion of L-[15N, 1-13C] leucine to quantitate the following parameters of leucine metabolism: nitrogen and carbon fluxes, oxidation, contribution to protein synthesis, breakdown of endogenous protein to leucine, deamination and reamination to/from ketoisocaproate. Studies were performed in the fasting and fed states with a conventional enteral diet (Propac) and a branched chain-enriched diet (one third Propac plus two thirds Hepatic-Aid). In vivo leucine metabolism was similar in the fasting and fed states in normal individuals in patients with cirrhosis and with both diets when studied at a protein intake of 0.6 gm/kg ideal body weight/day. When fed these diets, oxidation increased (p less than 0.05) and breakdown decreased (p less than 0.05). The Hepatic-Aid diet increased (p less than 0.05) nitrogen and carbon fluxes significantly more than did the standard diet. Four additional patients with cirrhosis on a diet with more protein were studied (0.75 gm/kg ideal body weight/day). Carbon and nitrogen fluxes, oxidation, synthesis, and deamination were increased (p less than 0.05) when patients with cirrhosis were fed the Propac diet compared with those who fasted. The Hepatic-Aid diet further increased (p less than 0.05) all parameters except synthesis and did not decrease protein breakdown. These data show that patients with cirrhosis metabolize leucine in vivo in a manner identical to that of normal subjects and that leucine-enriched formulas increase oxidation to CO2 without improving protein synthesis.
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PMID:In vivo measurement of leucine metabolism with stable isotopes in normal subjects and in those with cirrhosis fed conventional and branched-chain amino acid-enriched diets. 403 63

The effects of branched-chain amino acid (BCAA)-enriched nutrient mixture (nutrient-mixture) on the nitrogen metabolism and nutritional state were clinically investigated in 10 patients with liver cirrhosis. Nutrient-mixture-supplemented diet was prepared by adding 150 g nutrient-mixture daily to low-protein diet, and comparisons were made with a regular diet (control diet). Each diet supplied 2,100 kcal energy and 80 g protein per day. Patients were given control diet for 2 weeks and thereafter treated successively with nutrient-mixture-supplemented and control diet each for 2 weeks. Nitrogen balance improvement and positive balance were observed during the feeding of nutrient-mixture-supplemented diet. The composition of nitrogen compounds in urine and the fecal nitrogen excretion did not alter during the test period. Plasma aromatic amino acid (AAA) concentrations decreased and BCAA/AAA molar ratios increased significantly during the 1st and 2nd week of nutrient-mixture-supplemented diet administration. Plasma methionine concentration also decreased in the 1st week. Plasma pre-albumin levels rose significantly during the 1st and 2nd week of nutrient-mixture-supplemented diet administration, and the number connection test improved significantly following the supplemented diet. These results suggest that the use of nutrient-mixture in the nutritional treatment of liver cirrhosis had no deleterious effects on nitrogen metabolism and is useful for the improvement of plasma amino acid imbalance and protein-energy malnutrition.
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PMID:Nutritional treatment of liver cirrhosis by branched-chain amino acid-enriched nutrient mixture. 406 64

The combination of arterial hypoxemia and low pulmonary vascular resistance in patients with liver cirrhosis is unexplained. Pulmonary microcirculatory dilation, but not gross arterio-venous shunts, has been the usual postmortem finding in patients with liver cirrhosis. When 10 patients with alcoholic liver cirrhosis breathed 10% oxygen in nitrogen, they failed to increase their pulmonary vascular resistance. However, four patients with functional murmurs, three patients with hyperkinetic heart syndrome, six patients with normal pulmonary artery pressures and intracardiac left to right shunts, and five patients with renal failure and anemia all increased their pulmonary vascular resistances when they breathed 10% oxygen in nitrogen. These findings suggested that in liver cirrhosis the normal regulating mechanism (hypoxic vasoconstriction) of the pulmonary circulation may be impaired, resulting in failure of the lung to match perfusion with ventilation.
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PMID:Failure of hypoxic pulmonary vasoconstriction in patients with liver cirrhosis. 505 27

During a period of two years pericarditis was observed in five patients with decompensated cirrhosis who had only slightly raised blood-urea-nitrogen and serum-creatinine. Although this association has not been previously described, some patients with hepatic failure seem to have pericarditis in the absence of severe uraemia.
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PMID:Fibrinous pericarditis in hepatorenal failure. 610 54

Hospitalized patients with hepatic insufficiency often suffer from severe catabolic states and are in urgent need of nutritional support during their acute illness. Protein intolerence, however, remains a significant problem with respect to the provision of adequate nutrition, either enterally or parenterally. The following report is an anecdotal series of 63 consecutive patients in a large urban hospital treated prospectively with nutritional support using a prototype high branched-chain amino acid solution (FO80) given by technique of total parenteral nutrition by the subclavian or internal jugular route with hypertonic dextrose. Sixty-three patients, of which 42 had chronic liver disease (cirrhosis) with acute decompensation and 17 with acute hepatic injury as well as four with hepatorenal syndrome, are the subject of this report. All required intravenous nutritional support and were either intolerant to commercially available parenteral nutrition solutions or were in hepatic encephalopathy at the time they were initially seen. The cirrhotic patients had been hospitalized for a mean of 14.5 +/- 1.9 days before therapy, had a mean bilirubin of 13 mg/100 ml, and had been in coma for 4.8 +/- 0.7 days despite standard therapy. Patients with acute hepatitis had been in the hospital for 16.2 +/- 4.1 days before therapy, had a mean bilirubin of 25 mg/100 ml, and had been in coma 5.2 +/- 1.6 days before therapy. Routine tests of liver function, blood chemistries, amino acids, EEGs, and complex neurological testing including Reitan trailmaking tests were used in the evaluation of these patients. Up to 120 grams of synthetic amino acid solution with hypertonic dextrose was tolerated in these patients with improvement noted in encephalopathy of at least one grade in 87% of the patients with cirrhosis and 75% of the patients with hepatitis. Nitrogen balance was achieved when 75 to 80 grams of synthetic amino acids were administered. Survival was 45% in the cirrhotic group and 47% in the acute hepatitis group. Encephalopathy appeared to correlate with individual amino acids differentially in the various groups and with the ratio between the aromatic and the branched-chain amino acids. Ammonia did not correlate with either the degree of encephalopathy or improvement therefrom. In 24 Patients therapy for hepatic encephalopathy was limited to infusion of the branched-chain enriched amino acid solution only, with wake-up in 66% of this group. The results strongly suggest that in protein intolerant patients requiring nutritional support, infusion with branchedchain enriched amino acid solutions is well tolerated with either no worsening of or improvement in hepatic encephalopathy coincident with the achievement of nitrogen equilibrium and adequate nutritional support.
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PMID:Infusion of branched-chain enriched amino acid solution in patients with hepatic encephalopathy. 628 73

In 14 patients with cirrhosis of the liver and portal-systemic shunts the effect of a branched-chain amino acid-enriched elemental diet on portal systemic encephalopathy, routine laboratory parameters and plasma amino acids was investigated. In addition to the standard therapy including protein restriction (40 g/day) the patients received 44 g of an amino acid-protein mixture containing 30% of branched-chain amino acids and placebo over 3 months in a crossover regimen. Plasma valine and leucine increased significantly, whereas all other amino acids, including the ratio (formula: see text), remained unchanged. The electroencephalogram, number connection test, clinical state and laboratory parameters were not influenced by therapy with branched-chain amino acids. Thus, orally administered branched-chain amino acids probably have no influence on hepatic encephalopathy but are an adequate source of nitrogen in patients with cirrhosis of the liver.
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PMID:Branched-chain amino acid-enriched elemental diet in patients with cirrhosis of the liver. A double blind crossover trial. 636 43

It has been proposed that hepatic encephalopathy and malnutrition in cirrhosis can be reversed by infusion of a protein formula (F080) enriched with branched-chain amino acids (valine, leucine, isoleucine) and containing decreased amounts of aromatic amino acids (phenylalanine, tyrosine, tryptophan). This hypothesis was tested by measuring changes in encephalopathy status, plasma ammonia, amino acid profile, and liver function during seven metabolic balance studies in three patients with cirrhosis and subclinical encephalopathy given increasing amounts (20-100 g/d) of F080. The results showed the following: 1) positive nitrogen balance was achieved only with 80 and 100 g F080/day; 2) plasma ammonia fell during negative, but increased during positive nitrogen balance; 3) plasma tyrosine and cystine fell significantly (p less than 0.05) with all intakes of F080; 4) the abnormal branched-chain to aromatic amino acid ratio was reversed; 5) extracellular volume was expanded in all patients; 6) albumin, bilirubin, prothrombin time became abnormal; and 7) encephalopathy did not significantly change from baseline. It is concluded that, in this population, F080 is an inadequate nutritional formula when given as the sole protein source because it produces hypotyrosinemia and hypocystinemia. The marked changes in the ratio of branched-chain to aromatic amino acids are not accompanied by improvement in encephalopathy.
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PMID:Total parenteral nutrition with F080 in cirrhotics with subclinical encephalopathy. 640 94

Ten patients with histologically proven cirrhosis, admitted in grade I to II acute hepatic coma, received in addition to a standard dietary protein free "anticoma" regime, a continuous nasogastric infusion of a branched-chain amino acid enriched chemically defined enteral diet (Hepaticaid) containing an equivalent of 70 g protein/day for a mean of 7.3 days (range 3-23). No complications of therapy were observed and, specifically, the use of fine bore tubes did not provoke variceal hemorrhage. Overall positive nitrogen balance (4.3 +/- 1.7 g N/day) was observed in patients fed 7 or more days. Improvement to coma grade 0 was seen in all patients save one, who died from hepatorenal failure. While serum ammonia levels remained elevated during the study period, there was a significant increase in the branched-chain/aromatic ratio (p less than 0.01) as plasma branched-chain amino acids rose (p less than 0.05) and tyrosine levels fell (p less than 0.05).
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PMID:Enteral nutrition in malnourished patients with hepatic cirrhosis and acute encephalopathy. 641 10

Resistant ascites was studied in 34 patients with liver cirrhosis and ascites. The patients were initially divided into 3 groups on the basis of the weekly cumulative ascites retention curve: patients relieved of ascites within 3 weeks of admission, patients relieved between 4 and 12 weeks and patients with ascites persisting beyond 13 weeks. "Resistant ascites" was defined as "ascites persisting for more than 13 weeks after admission to the hospital". The patients were then reclassified into 3 groups : Group A being those patients relieved of ascites within 12 weeks, Group B being those with resistant ascites and group C being those who died within 12 weeks of admission. There were no differences in age and sex distribution, etiology of liver cirrhosis, past medical history or physical findings among the 3 groups. However, Group B had higher levels of serum creatinine and blood urea nitrogen than Group A on admission. Serum bilirubin was higher and serum albumin was lower in Group C than in Group B, which indicates that Group C had greater liver cell failure.
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PMID:Clinical studies of resistant ascites in liver cirrhosis. 646 4

The 15N-tracer technique gives new opportunities to medical research for investigation of normal and pathological nitrogen metabolism. Analysis of 15N kinetic data derived from total nitrogen excretion in the urine allows to calculate quantitatively certain parameters of protein synthesis and protein metabolism. At the same time analysis of serum samples will allow measurement of 15N-nitrogen incorporation into particular protein or non-protein fractions of the plasma. In the study reported here patients with different liver parenchyme lesions (acute hepatitis, chronic active hepatitis, biliary obstruction, cirrhosis of the liver, hepatic coma) were investigated with a standardized 15N-tracer method developed by us. It could be shown, that protein turnover in patients with liver insufficiency is significantly decreased as compared to healthy persons. Decrease of protein synthesis is associated with a still more pronounced decrease of protein catabolism. There are significant differences in dynamics of 15N in patients with hepatic coma as compared to other patients with liver parenchyme disease without portal encephalopathy. Nevertheless, even in hepatic coma 15N incorporation into plasma proteins can be shown. Differences in the 15N elimination kinetics of the non-protein pool between patients with chronic active hepatitis, biliary obstruction and hepatic coma could be found.
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PMID:[Protein turnover in liver failure--determination using the stable isotope 15N]. 650 88


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