Zusammenfassung
1. Die Phenylalanin-Toleranz wurde vergleichend bei Frühgeborenen, reifen Neugeborenen, Säuglingen und Erwachsenen bestimmt. Die Untersuchung erfolgte nach intravenöser Injektion von L-Phenylalanin. Die Elimination von Phenylalanin aus dem Blut erfolgte entsprechend einer Exponentialfunktion. Zur Beurteilung des Toleranzgrades wurde die Eliminationshalbwertszeit gewählt.
2. Die Phenylalanin-Toleranz war bei den meisten Frühgeborenen herabgesetzt. Bei reifen Neugeborenen kamen Toleranzverminderungen vor; sie waren jedoch wesentlich seltener. Die meisten Neugeborenen und alle Säuglinge zeigten eine Phenylalanin-Toleranz, die der beim normalen Erwachsenen entspricht.
3. Es werden die Beziehungen diskutiert, die zwischen der Phenylalanin-Toleranz und der Aktivität der Phenylalaninhydroxylase bestehen, und es wird auf die bei neonatalen Tieren beobachteten Aktivitätsverminderung des Phenylalaninhydroxylase-Systems hingewiesen.
Summary
1. The phenylalanine tolerance was estimated after intravenous injection of L-phenylalanine in premature newborns, mature newborns, infants and normal adults. The elimination of phenylalanine from the blood followed the pattern of a monomolecular reaction. The rate of elimination was, therefore, chosen for the comparison of the degree of tolerance.
2. The tolerance of phenylalanine was markedly reduced in most of the prematures; the same was found in some of the mature newborn infants. Compared with the tolerance in adults there was no decrease of phenylalanine tolerance in most of the mature newborns and in all infants.
3. The relation of phenylalanine tolerance to the activity of phenylalanine hydroxylase and to the development of the phenylalanine hydroxylase system was discussed.
Literatur
Levine, S. Z., E. Marples, andH. H. Gordon: A defect in the metabolism of tyrosine and phenylalanine in premature infants. I. Identification and assay of intermediary products. J. clin. Invest.20, 199 (1941).
Menkes, J. H., andM. E. Avery: The metabolism of phenylalanine and tyrosine in the premature infant. Bull. Johns Hopk. Hosp.113, 301 (1963).
Mathews, J., andM. W. Partington: The plasma tyrosine levels of premature babies. Arch. Dis. Childh.39, 371 (1964).
Bremer, H. J., P. Tosberg u.U. Hönscher: Untersuchungen über die Tyrosinstoffwechselstörung Frühgeborener. I. Veränderungen des Tyrosin-und Phenylalaninspiegels im Blut Frühgeborener während der ersten Lebenswochen. Ann. paediat. (Basel)206, 12 (1966).
Hsia, D. Y., M. Litwack, M. O'Flynn, andS. Jakovcic: Serum phenylalanine and tyrosine levels in the newborn infant. New Engl. J. Med.267, 1067 (1962).
Reem, G. H., andN. Kretchmer: Development of phenylalanine hydroxylase in liver of the rat. Proc. Soc. exp. Biol (N. Y.)96, 458 (1957).
Kenney, F. T., andN. Kretchmer: Hepatic metabolism of phenylalanine during development. J. clin. Invest.38, 2189 (1959).
Freedland, R. A., M. C. Krakowski, andH. A. Waisman: Effect of age, sex, and nutrition on liver phenylalanine hydroxylase activity in rats. Amer. J. Physiol.202 (I), 145 (1962).
Brenneman, A. R., andS. Kaufman: Characteristics of the hepatic phenylalanine hydroxylating system in newborn rats. J. biol Chem.240, 3617 (1965).
Allen, R. J., J. C. Heffelfinger, R. E. Masotti, andM. U Tsou: Phenylalanine hydroxylase activity in newborn infants. I. Relation to the appearance of metabolites in blood and urine in normal and enzyme deficient states. Pediatrics33, 512 (1964).
Hsia, D. Y. Y., R. S. Paine, andK. W. Driscoll: Phenylketonuria: Detection of the heterozygous carrier. J. ment. Defic. Res.1, 53 (1957).
Berry, H., B. Sutherland, andG. M. Guest: Phenylalanine tolerance tests on relatives of phenylketonuric children. Amer. J. hum. Genet.9, 310 (1957).
Bremer, H. J., andW. Neumann: The tolerance of phenylalanine after intravenous administration in phenylketonurics, heterozygous carriers, and normal adults. Nature (Lond.)209, 1148 (1966).
La Du, B. N., R. R. Howell, P. J. Michael, andE. K. Sober: A quantitative micromethod for the determination of phenylalanine and tyrosine in blood and its application in the diagnosis of phenylketonuria in infants. Pediatrics31, 39 (1963).
Amatuzio, D. S., F. L. Stutzman, M. J. Vanderbild, andS. Nesbitt: Interpretation of the rapid intravenous glucose tolerance test in normal individual and in mild diabetes mellitus. J. clin. Invest.32, 428 (1953).
Kaufman, S.: Studies on the structure of the primary oxidation product formed from tetrahydropteridines during phenylalanine hydroxylation. J. biol. Chem.239, 332 (1964).
Kaufman, S.: The structure of the phenylalanine-hydroxylation cofactor. Proc. nat. Acad. Sci. (Wash.)50, 1085 (1963).
Mitoma, C.: Studies on partially purified phenylalanine hydroxylase. Arch. Biochem.60, 476 (1956).
Wallace, H. W., K. Moldave, andA. Meister: Studies on conversion of phenylalanine to tyrosine in phenylpyruvic oligophrenia. Proc. Soc. exp. Biol. (N. Y.)94, 632 (1957).
Mitoma, C., R. M. Auld, andS. Udenfriend: On the nature of enzymatic defect in phenylpyruvic oligophrenia. Proc. Soc. exp. Biol. (N. Y.)94, 634 (1957).
Christensen, H. N., andJ. B. Clifford: Early postnatal intensification of hepatic accumulation of amino acids. J. biol. Chem.238, 1743 (1963).
Cornblath, M., S. H. Wybregt, andG. S. Baens: Studies of carbohydrate metabolism in the newborn infant. Pediatrics32, 1007 (1963).
Author information
Authors and Affiliations
Additional information
Mit Unterstützung der Deutschen Forschungsgemeinschaft.
Rights and permissions
About this article
Cite this article
Bremer, H.J., Neumann, W. Phenylalanin-Toleranz bei Frühgeborenen, reifen Neugeborenen, Säuglingen und Erwachsenen. Klin Wochenschr 44, 1076–1081 (1966). https://doi.org/10.1007/BF01716309
Issue Date:
DOI: https://doi.org/10.1007/BF01716309