Acid Profile In Patients Research Articles

Chemical diagnosis of inherited defects of fatty acid metabolism and ketogenesis.

Urinary organic acid profiles in patients with inherited defects of fatty acid metabolism and ketogenesis are described. Medium-chain acyl-CoA dehydrogenase, short-chain acyl-CoA dehydrogenase, multiple acyl-CoA dehydrogenase, and 3-hydroxy-3-methyl-glutaryl-CoA lyase deficiencies can be recognized at the metabolite level. Data on long-chain acyl-CoA dehydrogenase and systemic carnitine deficiencies are scarce. In the latter disorders, dicarboxylic aciduria is rather nonspecific and points to a modest omega-oxidation of long chain fatty acids.

Abnormal plasma amino acid profiles in patients undergoing bone marrow transplant

Amino acid concentrations in plasma have been measured during total parenteral nutrition in patients undergoing bone marrow transplantation. Profound hyperaminoacidemia was noted in the immediate para-transplant period. The increase was due mainly to high levels of phenylalanine and methionine. During the radiation treatment period these amino acids plus valine, proline, serine and glycine were exceptionally high. Levels of cystine and asparagine tended to be low. The results suggest that infusion of a mixture of amino acids which is lower in phenylalanine and methionine might result in better nitrogen utilization.

Abnormal plasma amino acid profiles in patients undergoing bone marrow transplant

Amino acid concentrations in plasma have been measured during total parenteral nutrition in patients undergoing bone marrow transplantation. Profound hyperaminoacidemia was noted in the immediate para-transplant period. The increase was due mainly to high levels of phenylalanine and methionine. During the radiation treatment period these amino acids plus valine, proline, serine and glycine were exceptionally high. Levels of cystine and asparagine tended to be low. The results suggest that infusion of a mixture of amino acids which is lower in phenylalanine and methionine might result in better nitrogen utilization.

Faecal bile acid profiles in patients with large bowel cancer in Japan.

To study the possible role of bile acids in carcinogenesis of the large bowel faecal bile acid profiles were analysed in detail in 14 patients with large bowel cancer and compared with 14 matched healthy subjects. The total faecal bile acid excretion was lower in the patients (297.3 +/- 402.8 mumol/day) than in the healthy subjects (470.6 +/- 231.1 mumol/day) (p less than 0.01), although the difference became insignificant when these data were expressed as mumol per wet or dry faecal weight. The faecal profiles of individual bile acids and their conjugates were similar in both groups except that 5 beta-chol-3-en-24-oic acid, a bacterial metabolite of sulpholithocholic acid, was found in nine cancer patients (64%) compared with only two healthy subjects (14%) (p less than 0.01). The significance of 5 beta-chol-3-en-24-oic acid in carcinogenesis needs further prospective and experimental studies.

Analysis of plasma bile acid profiles in patients with liver diseases associated with cholestasis.

Bile acids in plasma of patients with intra- or extra-hepatic cholestasis have been analyzed by gas chromatography-mass spectrometry after fractionation into groups by mode of conjugation. During cholestasis plasma concentrations ranged from 14 to 252 mumol/l. The predominant bile acid was cholic acid, comprising 44% to 89% of total bile acids. Tetrahydroxy- and trihydroxy-cholanoates with a tentative 6-hydroxy structure were identified, whereas 1-hydroxylated bile acids were not found. 3 beta-Hydroxy-5-cholenoic acid was the most important monohydroxycholanoate, comprising up to 13% of total bile acids in serum. The ratio of cholic to chenodeoxycholic acid in patients with primary biliary cirrhosis was in all cases more than 1. With the exception of 1-hydroxylated bile acids the main bile acids in urine an plasma were the same. Renal clearance of individual conjugated and sulfated bile acids could be calculated for some of the patients, assuming insignificant metabolism of bile acids in the kidney. The renal clearance of cholic acid conjugates tended to be higher tna that of chenodeoxycholic acid conjugates, and sulfates of cholic acid conjugates tended to have a higher renal clearance than sulfates of chenodeoxycholic acid conjugates. The clearance of sulfated 3 beta-hydroxy-5-cholenoic acid was still smaller. Chenodeoxycholic acid predominated in two healthy persons. Unconjugated bile acids accounted for 30% and 53%, respectively, of total bile acids. Sulfates of lithocholic, allolithocholic, and 3 beta-hydroxy-5-cholenoic acids were found after stimulation with a test meal.

Plasma amino acid profiles in patients with fulminant hepatic failure treated by repeated polyacrylonitrile membrane hemodialysis

Raised plasma amino acid concentrations have been implicated in the pathogenesis of hepatic encephalopathy. In the present study the effect of repeated 4- to 6-hr polyacrylonitrile membrane hemodialysis on circulating amino acid levels was investigated in 26 patients with fulminant hepatic failure in grade IV coma. Because all patients had grossly abnormal plasma amino acid profiles on admission, the relationship between alterations in plasma amino acid levels and changes in coma grade was also examined. Ten patients fully regained consciousness after two to nine periods of hemodialysis and 9 (34.6%) survived. During comparable periods of hemodialysis, total amino acid removal (range 0.49 to 15.30 mmoles in survivors, 0.45 to 22.85 mmoles in nonsurvivors) and plasma amino acid pool clearances (range 2.54 to 8.47 in survivors, 3.04 to 8.33 in nonsurvivors) in the two groups of patients was similar. Plasma amino acid levels in the nonsurvivors remained elevated throughout their illness despite hemodialysis. In contrast, in the survivors hemodialysis was associated with a gradual reduction in plasma levels of all amino acids, and a trend was observed for plasma tyrosine, phenylalanine, and proline to return to normal levels 24 hr in advance of recovery of consciousness. However, the relationship between coma grade and plasma level was not exact for any individual amino acid. Furthermore, the plasma ratio of branched chain amino acids to aromatic amino acids, also thought to be significant in hepatic encephalopathy, bore no consistent relationship to coma grade. These results, therefore, do not support a unique pathogenic role for plasma amino acids in the encephalopathy of fulminant hepatic failure.