No abstract

ExtractThe changes in plasma aminogram prcduced by variations in level of protein intake provided either as whole protein or a mixture of amino acids were studied. Controls were 29 normal infants, one to three months of age, fed a standard evaporated milk formula providing 3 to 3.5 g/kg/day of protein (fig. 1 and table II). Results obtained with the lowest protein intake (1.1 g/kg/day) are illustrated in figure 2 and presented in table III. Alterations in the plasma aminogram are noted as early as two days after the reduction in intake and become more pronounced as the feeding is prolonged. The most striking changes are depression of the branched chain amino acids (leucine, isoleucine and valine) and of lysine and tyrosine. Glycine and serine are elevated with glycine showing the greater elevation. Less striking changes are decreased concentrations of threonine, phenylalanine, ornithine, butyrine, cystine and proline.The aminograms obtained with feedings of 1.3 and 1.5 g/kg/day are very similar to those obtained with the more extreme degree of protein reduction ( fig. 3 and table IV). The aminogram after feeding 1.7 g/kg/day remains within the range of average ±1 standard deviation except for the elevation of glycine.A high intake of milk protein, 9 g/kg/day, results in an increase in the levels of a majority of the amino acids ( fig. 5 and table V). The sole exception is glycine, which tends to be low. Marked increases occur in the branched chain amino acids, valine being the most affected; there is also a large increase in the level of proline. The greatest elevations occur in the levels of methionine, which also show the most marked individual variation, elevations ranging from 2 to 35 times normal average value. An apparent tendency of the methionine level to return towards normal with prolonged feeding of the high protein diet is evident on the graph. The significance of this finding must be questioned, however, since the subjects who were maintained the longest on this diet were those who originally did not have the very high levels of methionine.There are some differences in the aminogram when infants are fed the equivalent of 3.5 g/kg/day in the form of amino acids instead of intact protein ( fig. 6 and table VI). On the amino acid diets, lower average plasma values are seen for the branched chain amino acids, alanine, proline, asparagine and citrulline; higher values are observed for threonine and serine. The markedly elevated levels of plasma amino acid observed after feeding 9 g/kg/day of whole protein are not seen after feeding the equivalent amount of nitrogen as an amino acid mixture ( fig. 7 and table VII). There is elevation only of valine and serine. When individual subjects were shifted from the high protein to the high amino acid diet, the pattern shifted within 24 hours. SNYDERMAN, HOLT, NORTON, ROITMAN, PHANSALKAR SpeculationA more precise and sensitive indicator of the state of protein nutrition is needed. While the level of the plasma amino acids changes rapidly with alterations in protein...

The clinical features of argininemia in two cousins included hyperactivity, spasticity, ataxia, retardation, and repeated attacks of hyperammonemia. Study of a large kindred suggests that arginase-deficiency is transmitted as a Mendelian recessive. Treatment with an essential amino acid mixture with the total nitrogen intake limited to the requirement, controlled the hyperammonemia, reduced the plasma arginine level, and permitted a marked clinical improvement. There has been a significant increase in intelligence levels; the previously retarded children are now approaching the normal range of function.

SummaryPlasma branched chain amino acid levels and their respective ketoacid analogues were determined in seven maple syrup urine disease patients ranging in age from 12 h to 12 years. One hundred one pairs were analyzed. There was a high degree of correlation between the amino acid and its ketoacid analogue at every amino acid level. The coefficient of correlation (0.84) was highest for leucine-a-ketoisocaproic acid. The ratio of ketoacid analogue to amino acid, (0.87), was also the greatest for leucine. The close correlation implies that adequate monitoring for therapy can be accomplished by the use of the technically simpler and more rapid determination of the plasma branched chain amino acids.