Plasma amino acids in patients with hepatic encephalopathy

Fischer, Josef E.; Yoshimura, N; Aguirre, Alfonso; James, J. Howard; Cummings, Megan; Aybel, R.M.; Deindoerfer, Fred H.

doi:10.1016/0002-9610(74)90009-9

Cited by 374 publications

(201 citation statements)

References 20 publications

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“…The mean value and the standard deviation of the Fischer index in our control collective were very similar to published values for adult controls (8,9,10) and healthy children (12). Decreases in the Val/Tyr ratio and in the Fischer index were measurable in our patients before hepatic failure had become critical.…”

Section: Discussionsupporting

confidence: 87%

“…The absolute and the fractional plasma amino acid profile in our patients were unbalanced, as described in adult cirrhotics (7)(8)(9). Tyrosine was highly increased; there was a trend to higher absolute phenylalanine and methionine values, and branched chain amino acids were decreased.…”

Section: Discussionmentioning

confidence: 90%

“…Methiogressive liver failure (5,6). The only definitive therapy nine can be markedly elevated (7)(8)(9). The Fischer appears to be liver transplantation (3,6).…”

Section: Introduction Donor Liver and Low Body Weight In Recipients Arementioning

confidence: 99%

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The Plasma Amino Acid Profile and its Relationships to Standard Quantities of Liver Function in Infants and Children with Extrahepatic Biliary Atresia and Preterminal Liver Cirrhosis

Byrd

Wiltfang²,

Rodeck³

et al. 1993

Clinical Chemistry and Laboratory Medicine

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Summary:The absolute and relative concentrations of 16 plasma amino acids in 48 mostly dystrophic infants and children (median of age 1 1 A years) with extrahepatic biliary atresia and mainly stable preterminal cirrhosis were compared with those of controls. Patient plasma amino acid data were analysed statistically for diagnostic usefulness and correlated with standard biochemical quantities of liver function and of liver perfusion. In the patients the total amounts of non-essential and essential amino acids were reduced by 19%, and with the same significance (p < 0.0005). Plasma tyrosine was increased (+40%), while taurine (-44%) and branched chain amino acids (+28.8% to -34.7%) were decreased. Methionine values varied widely. In the molar fractional plasma amino acid profile, only alanine, valine, and leucine were decreased, while threonine, methionine, tyrosine, phenylalanine, ornithine, and serine were increased. Discriminate function analysis showed that the plasma amino acid data discriminated 93.8% of the patients from controls. The concentrations of some amino acids in plasma seemed to have been influenced by protein-calorie deficiency in the patients. The valine/tyrosine ratio and the Fischer index (ratio branched chain/aromatic amino acids) were significantly reduced in the patients versus controls (1.54 + 0.55 vs 3.08 + 0.55 and 1.66 + 0.39 vs 3.00 + 0.48). A number of significant correlations (range of r: 0.37 -0.59, p < 0.05, 30-48 data pairs) were calculated between plasma amino acid data and several standard biochemical quantities of liver function. The statistical analyses also showed that the Fischer index began to decrease gradually and linearly early in the progression of liver failure. It is concluded that plasma amino acid data can be useful in the evaluation of the progression of liver failure and possibly of the nutritional status in liver transplant candidates with biliary atresia.

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Section: Discussionsupporting

confidence: 87%

Section: Discussionmentioning

confidence: 90%

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The Plasma Amino Acid Profile and its Relationships to Standard Quantities of Liver Function in Infants and Children with Extrahepatic Biliary Atresia and Preterminal Liver Cirrhosis

Byrd

Wiltfang²,

Rodeck³

et al. 1993

Clinical Chemistry and Laboratory Medicine

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“…Such patients usually drink 50 g Aminoleban ® EN, dissolved in 180 ml water and mixed with 6 g flavour, three times a day, as instructed in the product's package insert. Aminoleban ® EN contains a mixture of the branched-chain amino acids (BCAAs) L-isoleucine (Ile), L-leucine (Leu), and L-valine (Val), which are added in order to improve Fischer's rate, 1,2) but which have an extremely bitter taste. Patients must often take these enteral nutrients for long periods, and their bitterness is not only unpleasant but may also cause decreased compliance and/or intake.…”

Section: Aminolebanmentioning

confidence: 99%

Suppression of the Bitterness of Enteral Nutrients Using Increased Particle Sizes of Branched-Chain Amino Acids (BCAAs) and Various Flavours: a Taste Sensor Study

Miyanaga

Mukai

et al. 2004

Chem. Pharm. Bull.

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An improved formulation of the enteral nutrient Aminoleban® EN (Otsuka Pharmaceutical Co., Ltd., Tokyo, Japan), has been commercially available since Spring 2004. Like the previous formulation, the improved product contains branched-chain amino acids (BCAAs) L-isoleucine (L-Ile), Lleucine (L-Leu), and L-valine (L-Val), but the average particle size of these amino acids has been increased to 180 to 250 m mm in the improved formulation, compared with 40 to 90 m mm in the old product. The improved formulation has a significantly lower bitterness intensity score than the older formulation, as evaluated both in human gustatory tests and using the artificial taste sensor. We propose that this improved taste masking is due to the larger particle size of the BCAA crystals, due to which their release rates are reduced. The addition of improved flavours has also helped to reduce the bitterness of the improved Aminoleban ® EN formulation significantly. Analysis of the taste sensor data suggests that the sourness and sweetness of the added flavours were critical in diminishing the bitterness of Aminoleban ® EN.

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“…12,14,15 Furthermore, an important clinical parameter in FHF is the BCAA/AAA or Fisher's ratio, which decreases with increasing severity of hepatic dysfunction. 41 Plasma amino acid levels in D-galactosamine-treated rats fol- ‡Gluconeogenic amino acids include aspartate, asparagine, serine, glycine, glutamate, glutamine, histidine, threonine, alanine, proline, methionine, lysine, cysteine, tyrosine, phenylalanine, arginine, valine, and isoleucine. lowed similar changes.…”

Section: Discussionmentioning

confidence: 99%

Intrahepatic amino acid and glucose metabolism in a ?-galactosamine–induced rat liver failure model

et al. 2001

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A better understanding of the hepatic metabolic pathways affected by fulminant hepatic failure (FHF) would help develop nutritional support and other nonsurgical medical therapies for FHF. We used an isolated perfused liver system in combination with a mass-balance model of hepatic intermediary metabolism to generate a comprehensive map of metabolic alterations in the liver in FHF. To induce FHF, rats were fasted for 36 hours, during which they received 2 D-galactosamine injections. The livers were then perfused for 60 minutes via the portal vein with amino acid-supplemented Eagle minimal essential medium containing 3% wt/ vol bovine serum albumin and oxygenated with 95% O 2 /5% CO 2 . Control rats were fasted for 36 hours with no other treatment before perfusion. FHF rat livers exhibited reduced amino acid uptake, a switch from gluconeogenesis to glycolysis, and a decrease in urea synthesis, but no change in ammonia consumption compared with normal fasted rat livers. Mass-balance analysis showed that hepatic glucose synthesis was inhibited as a result of a reduction in amino acid entry into the tricarboxylic acid cycle by anaplerosis. Furthermore, FHF inhibited intrahepatic aspartate synthesis, which resulted in a 50% reduction in urea cycle flux. Urea synthesis by conversion of exogenous arginine to ornithine was unchanged. Ammonia removal was quantitatively maintained by glutamine synthesis from glutamate and a decrease in the conversion of glutamate to ␣-ketoglutarate. Mass-balance analysis of hepatic metabolism will be useful in characterizing changes during FHF, and in elucidating the effects of nutritional supplements and other treatments on hepatic function. (HEPATOLOGY 2001;34:360-371.)Since the late 1980s, orthotopic liver transplantation has become the only widely accepted treatment for fulminant hepatic failure (FHF). [1][2][3][4] Because of the severe shortage in donor organs, various alternatives aimed at providing a "substitute" liver, such as xenogeneic whole liver perfusion 5 and extracorporeal bioartificial liver devices, 6,7 are being developed. Such systems, which can be used as a bridge to transplantation or provide temporary relief during the most acute phase of hepatic failure, are promising, although technically complex and expensive. It has been hypothesized that hepatic encephalopathy may result from the elevation of circulating levels of putative toxins such as ammonia, mercaptans, short-chain fatty acids, [8][9][10] and an abnormal amino acid profile. [11][12][13] In FHF patients, alterations in amino acid concentrations in systemic plasma have been characterized by several investigators, 14,15 which led to the use of solutions rich in branchedchain amino acids (BCAA) for the treatment of hepatic encephalopathy. 16,17 These approaches, which were largely unsuccessful, aimed at counteracting the observed shifts in metabolite levels in the systemic circulation; however, systemic changes in metabolite levels do not solely reflect alterations in liver function, but also changes in whole-b...

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Plasma amino acids in patients with hepatic encephalopathy

Cited by 374 publications

References 20 publications

The Plasma Amino Acid Profile and its Relationships to Standard Quantities of Liver Function in Infants and Children with Extrahepatic Biliary Atresia and Preterminal Liver Cirrhosis

The Plasma Amino Acid Profile and its Relationships to Standard Quantities of Liver Function in Infants and Children with Extrahepatic Biliary Atresia and Preterminal Liver Cirrhosis

Suppression of the Bitterness of Enteral Nutrients Using Increased Particle Sizes of Branched-Chain Amino Acids (BCAAs) and Various Flavours: a Taste Sensor Study

Intrahepatic amino acid and glucose metabolism in a ?-galactosamine–induced rat liver failure model

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