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)

In an attempt to clarify the pathogenesis of the decreased branched-chain amino acid (BCAA) plasma concentrations in cirrhosis, the plasma clearances were measured in 7 patients with cirrhosis and in 7 age- and sex-matched control subjects. BCAA were given as prime-continuous infusions. The plasma clearances of valine, isoleucine, and leucine, calculated as infusion rate divided by steady state concentration, were low normal in cirrhotics despite hyperinsulinaemia, but different BCAA had different clearances (P less than 0.01). The endogenous basal appearance rates of BCAA, estimated by the basal concentrations multiplied by the plasma clearances, were lower in cirrhotics (P less than 0.025). The apparent theoretical volumes of distribution of BCAA, assessed by the ratio between the clearance and the concentration decay constant after infusion stop, were on average 67% of the total body weight, and were neither different among the three BCAA, nor between the two groups. The urea nitrogen synthesis rate did not increase significantly, suggesting that most of the infused BCAA nitrogen was taken up in peripheral tissues. The decreased concentration of BCAA in cirrhotics (394 +/- 81 mumol/l (mean +/- SD) in the present series vs 510 +/- 68 in controls; P less than 0.025) is not attributable to changes in plasma clearance. The most likely explanation is decreased afflux of BCAA into plasma.
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PMID:Plasma clearances of branched-chain amino acids in control subjects and in patients with cirrhosis. 357 28

We measured fasting plasma amino acids in 26 children aged 6 months to 5 years with extrahepatic biliary atresia and cirrhosis and compared them with fasting values in 95 normal control children aged 4 months to 12 years. We found that the cirrhotic children had elevations of total free plasma amino acids implying reduced hepatic metabolism of amino acids and that the molar ratio of the branched chain amino acids (isoleucine, leucine, and valine) to the aromatic amino acids (phenylalanine and tyrosine) was significantly depressed. Methionine was also markedly elevated, and taurine concentrations were significantly decreased. Manipulation of the amino acid distribution in dietary protein to normalize plasma amino acids prior to orthotopic hepatic transplantation may be helpful in improving amino acid utilization.
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PMID:Amino acid abnormalities in infants with extrahepatic biliary atresia and cirrhosis. 368 71

Amino acid levels were measured in the plasma and ascitic fluid of 13 alcoholic patients with liver cirrhosis and of 14 normal controls. The plasma aromatic amino acids of the alcoholic patients were not statistically different from those of the control subjects, whereas the levels of branched-chain amino acids were reduced (P less than 0.05). The values for the ratio of the muscle/liver metabolized amino acids (valine + isoleucine + leucine)/(phenylalanine + tyrosine + methionine) were statistically lower in the cirrhotic patients both in plasma and ascitic fluid (P less than 0.05). The amino acid levels in the ascitic fluid of the cirrhotic patients were slightly lower than those in the plasma of the same patients and the values for the muscle/liver metabolized amino acid ratio were similar in plasma and ascitic fluid. Aspartic acid, glutamic acid and glycine levels were higher among the cirrhotic patients (P less than 0.05). Cirrhotic patients present an alteration in amino acid metabolism which creates a different amino acid pattern in both plasma and ascitic fluid. The significance of the proposed ratio and its possible relation to hepatic failure are discussed.
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PMID:Amino acid patterns in the plasma and ascitic fluid of cirrhotic patients. 383 4

Changes in amino acid concentrations in plasma during a 100 g oral glucose tolerance test were investigated in patients with liver cirrhosis and in healthy controls. In the controls, almost all amino acid concentrations reached a nadir about 3 hours after glucose loading, then returned to initial levels after 6 hours. Immunoreactive insulin levels reached a peak about 30 minutes after loading, then decreased gradually, reaching initial levels after 6 hours. In the controls, the decrease ratios, defined as maximum decrease during the 3 hours after loading/initial concentration in plasma, were 0.607 and 0.554 for isoleucine (Ile) and leucine (Leu) respectively and 0.382 for valine (Val) which is significantly lower than for Ile or Leu. A similar tendency was recognized in patients with liver cirrhosis. The initial concentration of tyrosine (Tyr) and phenylalanine (Phe) in liver cirrhosis was significantly higher and their decrease ratios were significantly lower than in controls. Though no difference was observed between initial concentrations of tryptophan (Trp) in controls and liver cirrhosis patients, the decrease ratio of Trp in liver cirrhosis was lower (0.061) than that of controls (0.279) (p less than 0.001). The value, t-Trp/BCAA + AAA, i.e. total Trp concentration (mmol/l)/concentration (mmol/l) of branched chain amino acids (BCAA, Ile + Leu + Val) plus aromatic amino acids (AAA, Tyr + Phe), which is known to correlate with the brain Trp concentration of rats (Fernstrom, J. D. & Wurtman, R. J. (1972) Science 178, 414-416), changed significantly from 9.6 +/- 2.4 (mean +/- 1 SD) at the initiation to 12.9 +/- 3.3 at 3 hours after loading in controls (p less than 0.001), and in liver cirrhosis it changed from 10.3 +/- 1.9 to 15.8 +/- 3.1 (p less than 0.001).
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PMID:Changes in plasma amino acids during the oral glucose tolerance test and the effect of these changes on hepatic encephalopathy. 389 65

The metabolic effects of selected and branched-chain amino acid (BCAA)-enriched parenteral solutions were studied in liver cirrhosis. After 3 days of an oral protein-free diet with balanced amino acid (AA) infusion, 36 cirrhotic patients without encephalopathy were randomly divided into four groups. Groups A and B were infused for 5 days with BCAA (valine, leucine, isoleucine) at doses of 0.5 and 1.0 g/kg/day, respectively, as the only nitrogen source. Group C received 0.8 g/kg of essential and nonessential AA solution with a prevalence of BCAA; the last group (D) continued the basic standard diet, as control. Routine chemistry, urinary nitrogen losses, nitrogen balance, and the whole plasma AA pattern were detected before and after the treatment period. BCAA alone led to an impressive and significant improvement in the basic AA pattern in both the A and B groups. The same results were obtained in group C for plasma AA. In particular, the ratio of BCAA to aromatic amino acids in groups A, B, and C was significantly increased (p less than 0.01, less than 0.02, less than 0.02, respectively). In group D the AA pattern and the BCAA/aromatic amino acid ratio remained unchanged. The negative nitrogen balance of the base state remained unchanged after 0.5 g of BCAA (A); it improved significantly and became positive during and after the infusions of a double dose of BCAA (B), as it did in the case of selective solutions (C), although to a lesser extent; the negative nitrogen balance of the control group showed only a slight improvement.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Standard or branched-chain amino acid infusions as short-term nutritional support in liver cirrhosis? 392 19

Evidence from several sources suggest that blood-brain transport of the large neutral amino acids (NAA) is abnormal in animals with a portacaval anastomosis (PCA) and in patients with liver cirrhosis and portal-systemic shunting and encephalopathy, but the underlying mechanisms are unknown. After PCA, the concentration of glutamine (Gln) in brain is markedly increased as a by-product of cerebral ammonia detoxification, and the rate of efflux of Gln from brain is also increased. The following studies were undertaken to clarify the relationships among plasma and brain concentrations of NAA after PCA in rats and to examine the relationship of brain Gln concentration to plasma and brain NAA concentrations. After PCA plasma phenylalanine, tyrosine and histidine were elevated and leucine, isoleucine and valine were lowered. In brain, phenylalanine, tyrosine, histidine and methionine were markedly elevated after PCA and their concentrations in brain far exceeded the concentrations in plasma. Analyses of single, partial and multiple correlations of plasma NAA ratios expressed as plasma competitor function (PCF), brain NAA and brain Gln showed significant correlations between PCF nd brain NAA in shunted rats. A better correlation was found between brain NAA and brain Gln. Correlation coefficients obtained from multiple correlation analysis equalled or exceeded those obtained in the partial correlation or in the single correlation, suggesting that the effects of PCF and brain Gln on brain NAA were separate and additive. Gln was shown to compete with other NAA for blood brain transport by inhibiting brain 14C phenylalanine uptake.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Relationship of brain glutamine and brain neutral amino acid concentrations after portacaval anastomosis in rats. 393 Feb 56

Administration of total parenteral nutrition (TPN) solutions high in branched chain amino acids (BCAA) is thought to improve metabolic support during stress. This prospective, randomized, double blind study compared 45 per cent BCAA with 25 per cent BCAA in 12 patients. Seven patients had multiple trauma; two, gastrointestinal surgery; one, pancreatitis; and two, cirrhosis. The TPN regimen was 1.0-1.5 gm/kg/day amino acids and 30-45 glucose kcal/kg/day. The BCAA formula used was high in isoleucine and valine, but not leucine. Amino acid plasma levels, blood chemistries, 3-methylhistidine excretion, and nitrogen balance were studied. Control studies showed negative nitrogen balance (-7.1 +/- 2.9 gm) (mean +/- SEM), elevated insulin (61 +/- 21 microunit/ml), and elevated 3-methylhistidine (3MH) excretion (688 +/- 309 micromol); plasma leucine (93 +/- 11 nmol/ml) and isoleucine (37 +/- 23) were low, and valine (155 +/- 20) was elevated. Plasma methionine (40 +/- 9) and tyrosine (70 +/- 12) were high normal. Phenylalanine (85 +/- 5) was elevated. Both groups showed increased nitrogen excretion and positive nitrogen balance during the study (25 per cent, 2.0 +/- 1.4 gm/day; 45 per cent, 1.2 +/- 2.6 gm/day). Three-methylhistidine excretion changed little in either group (557 +/- 149, 414 +/- 91), insulin rose (135 +/- 27, 65 +/- 19), and plasma leucine (82 +/- 4, 71 +/- 9) changed little. Plasma isoleucine (51 +/- 3, 155 +/- 16) and valine (173 +/- 11, 691 +/- 23) both rose, more in the 45 per cent group. Methionine (67 +/- 12, 37 +/- 4) and tyrosine (51 +/- 6, 50 +/- 10) changed little.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Comparison of total parenteral nutrition with 25 per cent and 45 per cent branched chain amino acids in stressed patients. 393 93

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

In order to investigate disturbances in glycoregulation and plasma amino acids and their possible relationship in alcoholic liver diseases, plasma concentrations of insulin, C-peptide, glucagon and branched-chain (valine, leucine, isoleucine) as well as aromatic (phenylalanine, tyrosine) amino acids were measured during an arginine test (i.v infusion of arginine chloride 0.5 g/kg over 30 min) in 21 alcoholic patients: 11 with cirrhosis (group C) and 10 with steatosis (group S). Insulin responses to arginine was reduced in both groups, whereas glucagon response was increased in group C and reduced in group S. Plasma concentrations of branched-chain amino acids were reduced in both groups, irrespective of the degree of hyperinsulinism. Plasma concentrations of aromatic amino acids were increased only in cirrhotic patients; the increase was independent of the degree of hyperglucagonism and of the plasma insulin/glucagon molar ratio. These results suggest that disturbances of glycoregulation in plasma amino acids imbalance do not play a major role in alcoholic cirrhosis and steatosis.
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PMID:[Disturbances in glycoregulation and plasma amino acids in alcoholic hepatopathies. Study using the arginine test]. 623 16

Repeated plasmaphereses were done in 7 patients with acute hepatic failure (5 of them with coma stage IV and two with stage III). Acute liver insufficiency was induced by fulminant viral hepatitis in 4 cases, by drugs in one case, and was caused by a dystrophic exacerbation of liver cirrhosis in two further cases. Four of the 7 treated patients have survived. Beginning improvement of hepatic function was evidenced by an increase of factor VII and prothrombin in plasma. Significant lowering of bilirubin could be observed in all cases. While ammonia values decreased continuously in the four successfully treated patients while on plasmapheresis, the opposite behaviour was observed in the decreased patients. The influence of plasmapheresis on the pathologically altered aminoacid pattern in hepatic coma was investigated in two patients: Before treatment clearly to excessively increased values of methionine and aromatic aminoacids (phenylalanine and tyrosine) were seen. Branched-chain aminoacids leucine, isoleucine and valine were normal to moderately decreased. After termination of plasmapheresis methionine and aromatic aminoacids were significantly lower, branched-chain aminoacids were slightly below the initial values. Improvement of consciousness correlated with increase of the quotient (val+leu+ile)/(phe+tyr).
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PMID:[Treatment of acute liver failure by plasmapheresis]. 640 57


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