Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Evaluation of Amino-Aciduria in Infancy and Childhood

Evaluation of Amino-Aciduria in Infancy and Childhood Abstract Introduction Interest in the detection of free amino acids in the urine has been stimulated by the development of chromatographic techniques.1-5 As a result, new genetically determined conditions have been described, such as glycinuria6 and "Hartnup" disease.7 A number of well-known diseases have been further characterized by the excretion of increased or abnormal amino acids in the urine, as in rickets, scurvy, galactosemia, Wilson's disease, lead poisoning, and burns. The detection of amino-aciduria in certain mentally disturbed children8,9 offers a new biochemical approach in the study of the basic disturbance and may even suggest genetic implications. Reference to the occurrence of amino-aciduria in patients with mental disturbances can be found in the studies on phenylketonuria10,11 and Lowe's syndrome,12 and in the reports by Allan et al.8 and by Sutton and Read.9 In this report we shall discuss the principles of paper chromatography, summarize References 1. Consden, R.; Gordon, A. H., and Martin, A. J. P.: Quantitative Analysis of Protein: A Partition Chromagraphic Method Using Paper , Biochem. J. 38:224, 1944. 2. Smith, I.: Chromatographic Techniques , New York, Interscience Publishers, Inc., 1958, pp. 94-96. 3. Block, R. J.; Durrum, E. L., and Zweig, G.: Paper Chromatography and Paper Electrophoresis , New York, Academic Press, Inc., 1955. 4. Dent, C. E.: A Study of the Behavior of Some 60 Amino Acids and Other Ninhydrin Reacting Substances on Phenol-'Collidine' Filter Paper Chromatograms with Notes as to the Occurrence of Some of Them in Biological Fluids , Biochem. J. 43:168, 1948. 5. Woolf, L. I.: Paper Chromatography , Great Ormond St. J. No. 1-2:61, 1951. 6. deVries, A.; Kochwa, S.; Lazebuik, J.; Frank, M., and Djaldetti, M.: Glycinuria, a Hereditary Disorder Associated with Nephrolithiasis , Am. J. Med. 23:408, 1957.Crossref 7. Baron, D. N.; Dent, C. E.; Harris, A.; Hart, E. W., and Jepson, J. B.: Hereditary Pellagra-like Skin Rash with Temporary Cerebellar Ataxia, Constant Renal Amino-Aciduria, and Other Bizarre Biochemical Features , Lancet 2: 421, 1956.Crossref 8. Allan, J. D.; Cusworth, D. C.; Dent, C. E., and Wilson, V. K.: A Disease, Probably Hereditary, Characterized by Severe Mental Deficiency and a Constant Gross Abnormality of Amino Acid Metabolism , Lancet 1:182, 1958.Crossref 9. Sutton, H. E., and Read, J. H.: Abnormal Amino Acid Metabolism in a Case Suggesting Autism , A.M.A. Am. J. Dis. Child. 96:23, 1958. 10. Jervis, G. J.; Block, R. J.; Bolling, D., and Kanze, E.: Chemical and Metabolic Studies on Phenylalanine , J. Biol. Chem. 134:105, 1940. 11. Knox, W. E., and Hsia, D. Y-Y.: Pathogenetic Problems in Phenylketonuria , Am. J. Med. 22:687, 1957.Crossref 12. Lowe, C. U.; Terrey, M., and MacLachlan, E. A.: Organicaciduria, Decreased Renal Ammonia Production, Hydrophthalmos, and Mental Retardation , A.M.A. Am. J. Dis. Child. 83:164, 1952. 13. Gale, E. F.: The Bacterial Amino Acid Decarboxylases , Advances Enzymol. 6:1, 1946. 14. Van Slyke, D. D.; MacFayden, D., and Hamilton, P. B.: The Gasometric Determination of Amino Acids in Urine by Ninhydrin-Carbon Dioxide Method , J. Biol. Chem. 150:251, 1943. 15. Schweigert, B. S., and Snell, E. E.: Microbiologic Methods for the Estimation of Amino Acids , Nutrition Abstr. & Rev. 16:497, 1947. 16. Shemin, D.: Amino Acid Determination on Crystalline Bovine and Human Serum Albumin by the Isotope Dilution Method , J. Biol. Chem. 159:439, 1945. 17. Lederer, E., and Lederer, M.: Chromatography, A Review of Principles and Applications , New York, Elsevier Press, Inc., 1957. 18. Hanes, C. S.: Personal communication to the authors. 19. Ma, T. S., and Zuazago, G.: Micro-Kjedahl Determination of Nitrogen, a New Indicator and an Improved Rapid Method , J. Indust. & Engin. Chem. (Analyt. Ed.) 14:280, 1942. 20. Lauson, H. D.: Sources of Error in Plasma Creatinine Determination , J. Appl. Physiol. 4:227, 1951. 21. Hare, R. S.: Endogenous Creatinine in Serum and Urine , Proc. Soc. Exper. Biol. & Med. 74:148, 1950. 22. Moore, S., and Stein, W. H.: Photometric Ninhydrin Method for Use in the Chromatography of Amino Acids , J. Biol. Chem. 176:367, 1948. 23. Moore, S., and Stein, W. H.: A Modified Ninhydrin Reagent for the Photometric Determination Amino Acids and Related Compound , J. Biol. Chem. 211:907, 1954. 24. Ghadimi, H.; Stern, M., and Shwachman, H.: A Study of the Free Amino Acids in Sweat from Patients with Cystic Fibrosis , A.M.A. J. Dis. Child. 99:333, 1960. 25. Dent, C. E.: Chromatography in the Study of Amino Acid Metabolism , in Lectures on the Scientific Basis of Medicine , 1951-52 to 1954-55, London, Athlone Press, 1954, Vol. 2, p. 213. 26. Moore, S., and Stein, W. H.: Chromatography of Amino Acids on Sulfonated Polystyrene Resins , J. Biol. Chem. 192:663, 1951. 27. Moore, S., and Stein, W. H.: Procedures for the Chromatographic Determination of Amino Acids in 4% Cross-Linked Sulfonated Polystyrene Resins , J. Biol. Chem. 211:893, 1954. 28. Verghese, N., and Ramakrishnan, P. N.: A Simple Method for Desalting Biological Fluids for Paper Chromatography of Amino-Acids , J. Clin. Path. 10:104, 1957.Crossref 29. Ghadimi, H., and Shwachman, H.: Unpublished data. 30. Huisman, T. H. J.: L'Elimination des acides amines chez des enfants normaux d'ages differents , Arch. franc. pédiat. 14:166, 1957. 31. Westall, R. G.: The Amino Acids and Other Ampholytes of Urine: III. Unidentified Substances Excreted in Normal Human Urine , Biochem. J. 60:247, 1955. 32. Childs, B.: Urinary Excretion of Free Alpha-Amino Acid Nitrogen by Normal Infants and Children , Proc. Soc. Exper. Biol. & Med. 81:225, 1952. 33. Rivier, C., and Jeanneret, M. R.: Observations sur l'aminoacidurie du nourrisson bienportant , Helvet. paediat. acta 11:489, 1956. 34. Fowler, D. I.; Norton, P. M.; Cheung, M. W., and Pratt, E. L.: Observations on the Urinary Amino Acid Excretion in Man: The Influence of Age and Diet , Arch. Biochem. & Biophys. 68:452, 1957. 35. Harper, H. A.; Hutchin, M. F., and Kimmel, J. R.: Concentrations of 19 Amino Acids in Plasma and Urine of Fasting Normal Males , Proc. Soc. Exper. Biol. & Med. 80:768, 1952. 36. Harrison, H. E., and Harrison, H. C.: Aminoaciduria in Relation to Deficiency Diseases and Kidney Function , J.A.M.A. 164:1571, 1957. 37. Doolan, P. D.; Harper, H. A.; Hutchin, M. E., and Shreeve, W. W.: Renal Clearance of 18 Individual Amino Acids in Human Subjects , J. Clin. Invest. 34:1247, 1955. 38. Doolan, P. D.; Harper, H. A.; Hutchin, M. E., and Alpen, E. L.: The Renal Tubular Response to Amino Acid Loading , J. Clin. Invest. 35:888, 1956. 39. Dent, C. E., and Walshe, J. M.: Aminoacid Metabolism , Brit. M. Bull. 10:247, 1954. 40. Dent, C. E.: The Renal Acidurias , in Colloque sur les acides amines , Paris, Masson & Cie., 1954, p. 229. 41. Stein, W. H.: A Chromatographic Investigation of the Amino Acid Constituents of Normal Urine , J. Biol. Chem. 201:45, 1953. 42. Eckhard, R. D., and Davidson, C. S.: Urinary Excretion of Amino Acids by a Normal Adult Receiving Diets of Varied Protein Content , J. Biol. Chem. 177:687, 1949. 43. Evered, D. F.: The Excretion of Amino Acids by the Human: A Quantitative Study with Ion-Exchange Chromatography , Biochem. J. 62: 416, 1956. 44. Steele, B.; Sayberlich, H. E.; Reynolds, M., and Bayman, C. A.: Amino Acids in the Urine of Human Subjects Fed on Eggs or Soy Beans , J. Nutrition 33:209, 1947. 45. Dunn, M. S.; Camien, N. M.; Shankman, S., and Block, H.: Urinary Excretion of 12 Amino Acids by Normal Male and Female Subjects Measured Microbiologically , Arch. Biochem. 13:207, 1947. 46. Woodson, H. W.; Hier, S. W.; Solomon, J. D., and Bergein, O.: Urinary Excretion of Amino Acids by Human Subjects on Normal Diets , J. Biol. Chem. 172:613, 1958. 47. Woolf, L. I., and Norman, A. P.: The Urinary Excretion of Amino Acids and Sugars in Early Infancy , J. Pediat. 50:271, 1957. 48. Dent, C. E.: Clinical Applications of Aminoacid Chromatography , Scandinav. J. Clin. & Lab. Invest. 10:122, 1957. 49. Eckhardt, R. D.; Cooper, A. M.; Faloon, W. W., and Davidson, C. S.: Urinary Excretion of Amino Acids in Man , Tr. New York Acad. Sc. 10:284, 1948. 50. Snyderman, S. E.: Metabolism of Amino Acids , Pediatrics 21:117, 1958. 51. Dent, C. E., and Bickel, H.: Personal communication to the authors. 52. Lowe, C. U.: Congenital Abnormalities of Amino Acid in Renal Tubules , Pediatrics 21: 1039, 1958. 53. Lowe, C. U., and Bruck, E.: Primary Chronic Metabolic Acidosis with Organic Aciduria , Mod. Prob. Pediat. 3:509, 1958. 54. Kretchmer, N., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 81. 55. Hsia, D. Y-Y., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 92. 56. Dustin, J. P.; Moore, S., and Bigwood, E. J.: Chromatographic Studies on the Excretion of Amino Acid in Early Infancy , Metabolism 4:75, 1955. 57. Sereni, F.; McNamara, H.; Shibuya, M.; Kretchmer, N., and Barnett, H. L.: Concentration in Plasma and Rate of Urinary Excretion of Amino Acid in Premature Infants , Pediatrics 15: 575, 1955. 58. Pratt, E. L., and Hasselmeyer, E.: Urinary Amino Acid Excretion by Premature Infants , A.M.A. Am. J. Dis. Child. 88:659, 1954. 59. Page, E. W.; Glendening, M. B.; Dignam, W., and Harper, H. A.: The Causes of Histidinuria in Normal Pregnancy , Am. J. Obst. & Gynec. 68:110, 1954. 60. Wallfaff, E. B.; Brodie, E. C., and Borden, A. L.: Urinary Excretion of Amino Acids in Pregnancy , J. Clin. Invest. 29:1542, 1950. 61. Cecil, I. B., and Voge, B.: A Simple Test for Pregnancy , Brit. M.J. 2:829, 1929. 62. Crumpler, H. R.; Dent, C. E.; Harris, H., and Westall, R. G.: β-Aminoisobutyric Acid (α-Methyl-β-Alanine): A New Amino Acid Obtained from Human Urine , Nature , London 167: 307, 1951. 63. Gartler, S. M.; Firschein, L. I., and Gidaspow, T.: Some Genetical and Anthropological Consideration of Urinary β-Aminoisobutyric Acid Excretion , Acta genet. basel. 6:435, 1956-57. 64. Mudge, G. H.: Clinical Patterns of Tubular Dysfunction , Am. J. Med. 24:785, 1958. 65. De Grouchy, J., and Sutton, H. E.: A Genetic Study of β-Aminoisobutyric Acid Excretion , Am. J. Human Genet. 9:76, 1957. 66. Harris, H.: Family Studies on the Urinary Excretion of β-Aminoisobutyric Acids , Ann. Eugen. 18:43, 1953. 67. Jervis, G. A.: Excretion of Phenylalanine and Derivatives on Phenylpyruvic Oligophrenia , Proc. Soc. Exper. Biol. & Med. 75:83, 1950. 68. Armstrong, M. D., and Robinson, K. S.: On the Excretion of Indole Derivatives in Phenylketonuria (Letter to Editor) , Arch. Biochem. 52: 287, 1954. 69. Bickel, H.; Gerrard, J., and Hickmans, E. M.: The Influence of Phenylalanine Intake on the Chemistry and Behaviour of a Phenylketonuric Child , Acta paediat. 43:64, 1954. 70. Armstrong, M. D.; Shaw, K. N. F., and Robinson, K. S.: Studies on Phenylketonuria: II. The Excretion of Hydroxyphenylacetic Acid in Phenylketonuria , J. Biol. Chem. 213:797, 1955. 71. Armstrong, M. D., and Binkley, E. L.: Studies on Phenylketonuria: V. Observations on a Newborn Infant with Phenylketonuria , Proc. Soc. Exper. Biol. & Med. 93:418, 1956. 72. Hsia, D., and Richmond, S.: Detection of the Heterozygous Carrier , J. Ment. Def. 1:53, 1957. 73. Smith, A. J., and Strang, L. B.: An Inborn Error of Metabolism with the Urinary Excretion of α-Hydroxy-Butyric Acid and Phenylpyruvic Acid , Arch. Dis. Childhood 33:109, 1958. 74. Harris, H.: in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 106, 109. 75. Walshe, J. M.: Disturbances of Amino Acid Metabolism Following Liver Injury , Quart. J. Med. 22:483, 1953. 76. Hsia, D. Y-Y., and Gellis, S. S.: Amino Acid Metabolism in Infectious Hepatitis , J. Clin. Invest. 33:1603, 1954. 77. Jonxis, J. H. P., and Huisman, T. H. J.: Excretion of Amino Acid in Free and Bound Form During Intravenous Administration of Protein Hydrolysate , Metabolism 6:175, 1957. 78. Eckhardt, R. D., and Davidson, C.: Urinary Excretion of Amino Acids Following the Rapid Injection of a Solution of Amino Acid in Man , J. Clin. Invest. 29:727, 1948. 79. Christensen, H. N.;; Lynch, E. L., and Powers, J. H.: The Conjugated, Non-Protein, Amino Acids of Plasma: III. Peptidemia and Hyperpeptiduria as a Result of the Intravenous Administration of Partially Hydrolyzed Casein (Amigen) , J. Biol. Chem. 166:649, 1946. 80. Bickel, H., et al.: Cystine Storage Disease with Aminoaciduria and Dwarfism (Lignac-Fanconi Disease) , Acta paediat. ( (Supp. 90) ) 42:27, 1952. 81. Clay, R. D.; Darmady, E. M., and Hawkins, M.: The Nature of the Renal Lesion in the Fanconi Syndrome , J. Path. & Bact. 65:551, 1953. 82. Dern, P. L.: Aminoaciduria with Cystinosis: Case Report with Determination of Urinary Amino Acid and Ocular Cystine , Ann. Int. Med. 46:138, 1957. 83. Darmady, E. M.: Renal Lesions in Relation to Aminoaciduria and Water Diuresis , in Ciba Foundation Symposium on the Kidney , Boston, Little, Brown & Company, 1954, p. 27. 84. Maschas, H.; Lamotte, M.; Lamotte-Barillon, S., and Barbier, P.: Un Cas de cystinose revelée par une lithiase urinaire , Bull. et mem. Soc. méd hôp. Paris 64:491, 1948. 85. Debré, R.; Marie, J.; Cléret, F., and Messimy: Rachitisme tardif coexistant avec une néphrite chronique et une glycosurie , Arch. méd. Enf. 37:597, 1934. 86. Dent, C. E., and Harris, H.: Hereditary Forms of Rickets and Osteomalacia , J. Bone & Joint Surg. 38B:204, 1956. 87. Fanconi, G.: Weitere Beiträge zur Cystinkrankheit (der Amindiabetes) , Helvet. paediat. acta 1:183, 1946. 88. Fanconi, G., and Bickel, H.: Die chronische Aminoacidurie (aminosaure Diabetes oder nephrotisch-glukosurischer Zwergwuchs) bei der Glykoserose und der Cystinkrankheit , Helvet. paediat. acta 4:359, 1949. 89. Beumer, H., and Weppler, W.: Über die Cystinkrankheit der ersten Lebenszeit , Klin. Wchnschr. 16:8, 1937. 90. Hottinger, A.: Über Cystin-Diathese , Ann. paediat. 156:257, 1941. 91. Bürki, E.: Über Augenveränderungen bei der Cystinkrankheit , Ann. paediat. 156:324, 1941. 92. Freudenberg, E.: Weitere Beobachtungen zur Frage der Cystinosis , Ann. paediat. 182:85, 1954. 93. Bernstein, J.; Meyer, R., and Cohen, F.: The DeToni-Fanconi Syndrome in Childhood, The Society for Pediatric Research Program and Abstracts, 29th Annual Meeting, Buck Hill Falls, Pa., May 8-9, 1959. 94. Freudenberg, E.: Cystinosis: Cystine Disease (Lignac's Disease) in Children , Advances Pediat. 4:265, 1949. 95. Fanconi, G.: Der frühinfantile nephrotischglykosurische Zwergwuchs mit hypophosphatä mischer Rachitis , Jahrb. Kinderh. 147:299, 1936. 96. McCune, D. J.; Mason, H. H., and Clarke, H. T.: Intractable Hypophosphatemic Rickets with Renal Glycosuria and Acidosis (The Fanconi Syndrome) , Am. J. Dis. Child. 65:81, 1943.Crossref 97. Dent, C. E.: Amino Aciduria in the Fanconi Syndrome: A Study Making Extensive Use of Techniques Based on Paper Partition Chromatography , Biochem. J. 41:240, 1947. 98. Williamson, D. A. J.: Cystinosis , Arch. Dis. Childhood 27:356, 1952.Crossref 99. Eberlein, W. R.: Aminoaciduria in Childhood: Cystinuria and Cystinosis , Am. J.M. Sc. 225:677, 1953.Crossref 100. Parker, W. S.; Prader, A., and Fanconi, G.: Further Observations on Cystine Storage Disease , Pediatrics 16:228, 1955. 101. Salassa, R. M.; Power, M. H.; Ulrich, J. A., and Hayles, A. B.: Observations on the Metabolic Effects of Vitamin D in Fanconi's Syndrome , Proc. Staff Meet. Mayo Clin. 29:214, 1954. 102. McCune, D. J.; Mason, H. H., and Clarke, H. T. L.: Late Rickets with Glycosuria and Organic Acid Acidosis (Fanconi Type) , Am. J. Dis. Child. 58:673, 1939. 103. Jackson, H. R., and Clarke, B. E.: Cystinosis: Report of 2 Cases with Postmortem Examination , A.M.A. Am. J. Dis. Child. 85:513, 1953. 104. Lathem, W.; Baker, K., and Bradley, S. E.: Urinary Amino Acid Excretion in Renal Disease, with Observations on Fanconi Syndrome , Am. J. Med. 18:249, 1955.Crossref 105. Milne, M. D.; Stanbury, W. S., and Thomson, A. E.: Observations on the Fanconi Syndrome and Renal Hyperchloraemic Acidosis in the Adult , Quart. J. Med. 21:61, 1952. 106. Dragsted, P. J., and Hjorth, N.: Fanconi's Syndrome , Acta med. scandinav. 146:317, 1953.Crossref 107. Drablos, A.: The de Toni-Fanconi Syndrome with Cystinosis , Acta paediat. 40:438, 1951.Crossref 108. King, F. P., and Lochridge, E. P.: Cystinosis (Cystine-Storage Disease) , A.M.A.J. Dis. Child. 82:446, 1951. 109. Myerson, R. M., and Pastor, B. H.: Fanconi Syndrome and Its Clinical Variants , Am. J. M. Sc. 228:378, 1954.Crossref 110. Russell, D. S., and Barrie, H. J.: Storage of Cystine in Reticulo-Endothelial System and Its Association with Chronic Nephritis and Renal Rickets , Lancet 2:889, 1936. 111. Worthen, G., and Good, A.: The de Toni-Fanconi Syndrome with Cystinosis Clinical and Metabolic Study of 2 Cases in a Family and a Critical Review on the Nature of the Syndrome , A.M.A.J. Dis. Child. 95: 653, 1958.Crossref 112. Skinner, W.; Dortis, C., and Paul, J. R., Jr.: Amino-Aciduria in Childhood, Cystinuria or Cystinosis , J. South Carolina M.A. 53:203, 1957. 113. de Toni, G.: Remarks on the Relations Between Renal Rickets (Renal Dwarfism) and Renal Diabetes , Acta paediat. 16:479, 1933.Crossref 114. de Toni, G.: Renal Rickets with Phospho-Gluco-Amino Renal Diabetes (de Toni-Debré-Fanconi Syndrome) , Ann. paediat. 187:42, 1956. 115. Bickel, H., and Thursby-Pelham, D. C.: Hyperamino-Aciduria in Lignac-Fanconi Disease, in Galactosaemia and in an Obscure Syndrome , Arch. Dis. Childhood 29:224, 1954.Crossref 116. Robson, E., and Rose, G. A.: The Effect of Intravenous Lysine on the Renal Clearances of Cystine, Arginine and Ornithine in Normal Subjects, in Patients with Cystinuria and Fanconi Syndrome, and in Their Relatives , Clin. Sc. 16:75, 1957. 117. Hottinger, A.: Zur Cystindiathese , Ann. paediat. 169:272, 1947. 118. Luder, J., and Sheldon, W.: A Familial Tubular Absorption Defect of Glucose and Amino-Acids , Arch. Dis. Childhood 30:160, 1955.Crossref 119. Harris, H: Renal Aminoaciduria , Brit. M. Bull. 13:26, 1957. 120. Dent, C. E., and Rose, G. A.: Aminoacid Metabolism in Cystinuria , Quart. J. Med. 20:205, 1951. 121. Brooks, W. D. W.; Heasman, M. A., and Lovell, R. R. H.: Retinitis Pigmentosa Associated with Cystinuria , Lancet 1:1096, 1949.Crossref 122. Lewis, H. B.: Occurrence of Cystinuria in Healthy Young Men and Women , Ann. Int. Med. 6:183. 1932.Crossref 123. Prakken, H.: Case of Congenital Cystinuria , J. Pediat. 40:186, 1952.Crossref 124. Yeh, H. L.: Frankl, W.; Dunn, M. S.; Parker, P.; Hughes, B., and Gyorgi, P.: Urinary Excretion of Amino Acids by a Cystinuric Subject , Am. J. M. Sc. 214:507, 1947.Crossref 125. Dent, C. E.; Senior, B., and Walshe, J. M.: Pathogenesis of Cystinuria: II. Polarographic Studies of the Metabolism of Sulphur-Containing Amino-Acids , J. Clin. Invest. 33:1216, 1954.Crossref 126. Lewis, H. B.; Brown, B. H., and White, G. R.: Metabolism of Sulphur: XXIII. The Influence of the Ingestion of Cystine, Cysteine and Methionine on the Excretion of Cystine in Cystinuria , J. Biol. Chem. 114:171, 1936. 127. Brown, B. H., and Lewis, H. B.: Cystine in Normal and Cystinuric Human Blood , Proc. Soc. Exper. Biol. & Med. 36:487, 1937. 128. Cleland, J. B.: Cystine, Methionine and Sulphate Excretion in Cystinuria , Australian J. Exper. Biol. & M. Sc. 24:210, 1946. 129. Cleland, J. B.: Cystine, Methionine and Sulphate Excretion in Cystinuria , Australian J. Exper. Biol. & M. Sc. 29:85, 1951. 130. Cleland, J. B.: Cystine, Methionine and Sulphate Excretion in Cystinuria , Australian J. Exper. Biol. & M. Sc. 25:33, 1947. 131. Harris, H., and Robson, E.: Variation in Homozygous Cystinuria , Acta genet. 5:381, 1955. 132. Dent, C. E.; Heathcote, J. G., and Joron, G. E.: The Pathogenesis of Cystinuria: I. Chromatographic and Microbiological Studies of the Metabolism of Sulphur-Containing Amino-Acids , J. Clin. Invest. 33:1210, 1954. 133. Stein, W. H.: Excretion of Amino Acids in Cystinuria , Proc. Soc. Exper. Biol. & Med. 78:705, 1951. 134. Fowler, D., and Harris, H.: Plasma Cystine Levels in Cystinuria , Lancet 1:544, 1952.Crossref 135. Dent, C. E., and Harris, H.: The Genetics of "Cystinuria," Ann. Eugen. 16:60, 1951.Crossref 136. Harris, H.; Mittwoch, U.; Robson, E. B., and Warren, F. L.: The Pattern of Amino-Acid Excretion in Cystinuria , Ann. Human Genet. 19:196, 1955.Crossref 137. Harris, H., and Warren, F. L.: Quantitative Studies on the Urinary Cystine in Patients with Cystine Stone Formation and in Their Relatives , Ann. Eugen. 18:125, 1953.Crossref 138. Bearn, A.: An Inborn Error of Metabolism with Multiple Manifestations , Am. J. Med. 22:747, 1957.Crossref 139. Iber, F. L.; Chalmers, T. C., and Uzman, L.: Studies of Protein Metabolism in Hepatolenticular Degeneration , Metabolism 6:388, 1957. 140. Stein, W. H.; Bearn, A. A., and Moore, S.: The Amino Acid Content of the Blood and Urine in Wilson's Disease , J. Clin. Invest. 33:410, 1954.Crossref 141. Uzman, L. L., and Denny-Brown, D.: Amino-Aciduria of Wilson's Disease (Hepatolenticular Degeneration) , Am. J. M. Sc. 223:392, 1952.Crossref 142. DeVerdier, C. H.: Paper Chromatographic Analysis of Amino Acid Excretion in Wilson's Disease , Acta med. scandinav. 138:344, 1950.Crossref 143. Gilsanz, V.; Segovia, J. M., and Castro Mendoza, H.: Contribution to Study of Wilson's Disease , A.M.A. Arch. Int. Med. 95:727, 1955.Crossref 144. Uzman, L. L., and Hood, B.: The Familial Nature of the Amino-Aciduria of Wilson's Disease (Hepatolenticular Degeneration) , Am. J. M. Sc. 223:392, 1952.Crossref 145. Bearn, A. G., and Kunkel, H. G.: Abnormalities of Copper Metabolism in Wilson's Disease and Their Relationship to Aminoaciduria , J. Clin. Invest. 33:400, 1954.Crossref 146. Cooper, A. M.; Eckhardt, D.; Faloon, W. W., and Davidson, C. S.: Investigation of the Aminoaciduria in Wilson's Disease (Hepatolenticular Degeneration): Demonstration of a Defect in Renal Function , J. Clin. Invest. 29:265, 1950.Crossref 147. Chalmers, T. C.; Iber, F., and Uzman, L. L.: Hepatolenticular Degeneration (Wilson's Disease) as a Form of Idiopathic Cirrhosis . New England J. Med. 256:235, 1957.Crossref 148. Uzman, L. L.: Amino Aciduria in Hepatolenticular Degeneration (Wilson's Disease) , Am. J. M. Sc. 215:33, 1948.Crossref 149. Uzman, L. L.: On the Relationship of Urinary Copper Excretion to the Amino Aciduria in Wilson's Disease , Am. J. M. Sc. 226:45, 1953.Crossref 150. Hsia, D. Y-Y.; Hsia, H. H.; Green, S.; Kay, M., and Gellis, S. S.: Amino-Aciduria in Galactosemia , A.M.A. Am. J. Dis. Child. 88:458, 1954. 151. Holzel, A.; Komrower, G. M., and Wilson, V. K.: Amino-Aciduria in Galactosaemia , Brit. M. J. 1:194, 1952.Crossref 152. Holzel, A., and Komrower, G. M.: A Study of the Genetics of Galactosaemia , Arch. Dis. Childhood 30:155, 1955.Crossref 153. Komrower, G. M.: Schwarz, V.; Holzel, A., and Goldberg, L.: A Clinical and Biochemical Study of Galactosaemia , Arch. Dis. Childhood 31:254, 1956.Crossref 154. Komrower, G. M.: L'Amino-Acidurie dans la galactosémie , Arch. franc pédiat. , 10:185, 1953. 155. Bickel, H., and Hickmans, E. M.: Paper Chromatographic Investigations on the Urine of Patients R. T. and R. R. , Arch. Dis. Childhood 27:348, 1952. 156. Cusworth, D. C.; Dent, C. E., and Flynn, F. V.: The Amino-Aciduria in Galactosaemia , Arch. Dis. Childhood 30:150, 1955.Crossref 157. Henderson, W.: A Case of Hartnup's Disease , Arch. Dis. Childhood 33:114, 1958.Crossref 158. Westall, R. G.; Dancis, J., and Miller, S.: Maple Syrup Urine Disease , A.M.A.J. Dis. Child. 94:571, 1957. 159. Thelander, H. E., and Imagawa, R.: Amino Aciduria, Congenital Defects, and Mental Retardation: a Preliminary Report , J. Pediat. 49:123, 1956.Crossref 160. Schönenberg, H.: Papierchromatographische Untersuchungen bei der Pfaundler-Hurlerschen Krankheit , Monatsschr. Kinderh. 102:404, 1954. 161. Ames, S. R., and Risley, H. A.: Aminoaciduria in Progressive Muscular Dystrophy , Proc. Soc. Exper. Biol. & Med. 68:131, 1948. 162. Nestel, P. J., and Nestel, L.: Investigation of Aminoaciduria in Erythro-Blastosis Foetalis and Its Possible Relationship to Kernicterus , M.J. Australia 1:909, 1957. 163. Steinberg, H.; Szeinberg, A., and Cohen, B. E.: Aminoaciduria and Hypermetabolism in Progeria , Arch. Dis. Childhood 32:401, 1957. 164. Gray, C. H., and Illing, E. K. B.: Plasma and Urinary Amino-Acids in Diabetes , J. Endocrinol. 8:44, 1952. 165. Gross, J.; Comfort, M., and Ulrich, J.: Abnormalities of Serum and Urinary Amino Acids in Hereditary and Nonhereditary Pancreatitis , Tr. A. Am. Physicians 70:127, 1957. 166. van Creveld, S., and Aarons, P.: Transitory Renal Osteoporosis with Aminoaciduria and Development of Hypersensitivity to Vitamin D , Ann. paediat. 173:299, 1949. 167. Bickel, H.: Zum Mechanismus der Aminoacidurie, presented at the Eighth International Congress of Paediatrics, Copenhagen, 1956. 168. Altman, K. L., and Miller, G.: A Disturbance of Tryptophan Metabolism in Congenital Hypoplastic Anaemia , Nature , London 172:868, 1953. 169. Huisman, T. H. J.: The Concentration of Different Amino Acids in the Blood Plasma in Children Suffering from Rickets and Scurvy , Pediatrics 14:245, 1954. 170. Jonxis, J. H. P.: Amino-Aciduria and Rickets , Helvet. paediat. acta 10:245, 1955. 171. Jonxis, J. H. P., and Huisman, T. H. J.: Amino-Aciduria in Rachitic Children , Lancet 2:428, 1953. 172. Jonxis, J. H. P., and Huisman, T. H. J.: Amino Aciduria and Ascorbic Acid Deficiency , Pediatrics 14:238, 1954. 173. Eades, C. H., Jr.; Pollack, R. L., and Hardy, J. D.: Thermal Burns in Man: IX. Urinary Amino Acid Patterns , J. Clin. Invest. 34:1756, 1955. 174. Nardi, G. L.: "Essential" and "Non-Essential" Amino Acids in Urine of Severely Burned Patients , J. Clin. Invest. 33:847, 1954. 175. Woolf, L. I., and Giles, H. M. C.: Urinary Excretion of Amino-Acids and Sugar in the Nephrotic Syndrome , Acta paediat. 45:489, 1956.Crossref 176. Shreeve, W. W.; Hutchin, M. E.; Harper, H. A.; Miller, C. D., and Doolan, P. D.: Excretion of Amino Acids in Nephrosis , Proc. Soc. Exper. Biol. & Med. 88:510, 1955. 177. Wilson, V. K.; Thomson, M. L., and Dent, C. E.: Amino-Aciduria in Lead Poisoning , Lancet 2:66, 1953. 178. Chisholm, J. J., Jr.; Harrison, H. C.; Eberlein, W. R., and Harrison, H. E.: Amino Aciduria, Hypophosphatemia and Rickets in Lead Poisoning , A.M.A.J. Dis. Child. 89:159, 1955. 179. Rothstein, A., and Berke, H.: Amino-Aciduria in Uranium Poisoning , J. Pharmacol. & Exper. Therap. 96:179, 1949. 180. Voegtlin, C., and Hodge, H. C., Editors: Pharmacology and Toxicology of Uranium Compounds , National Nuclear Energy Series, Manhattan Project Technical Section, Division 6, No. 2, New York, McGraw-Hill Book Company, Inc., 1949, p. 972. 181. Spencer, A. G., and Franglen, G. T.: Gross Amino-Aciduria Following Lysol Burn , Lancet 1:190, 1952. 182. Knox, W. E.: Personal communication to the authors. 183. Sarrouy, C.; Cabannes, R., and Clausse, J.: Étude des acides amines du sang et des urines au cours des syndrome de denutrition du nourrissoh , Presse méd. 65:1221, 1957. 184. Schendel, H. E.; Antonis, A., and Hansen, J.: Increased Amino-Aciduria in Infants with Kwashiorkor Fed Natural and Synthetic Diets , Pediatrics 23:662, 1959. 185. Cheung, M. W.; Fowler, D. I; Norton, P. M.; Synderman, S. E., and Holt, J. E., Jr.: Observations on Amino Acid Metabolism in Kwashiorkor , J. Trop. Pediat. 1:141, 1955. 186. Kretchmer, N.: Disorders Associated with the Metabolism of Phenylalanine and Tyrosine , Pediatrics 21:445, 1958. 187. Levine, S. Q.; Marples, E., and Gordon, H. H.: A Defect in the Metabolism of Tyrosine and Phenylalanine in Premature Infants: I. Identification and Assay of Intermediary Products; II. Spontaneous Occurrence and Eradication by Vitamin C , J. Clin. Invest. 20:209, 1941. 188. Medes, G.: A New Error of Tyrosine Metabolism: Tyrosinosis; The Intermediary Metabolism of Tyrosine and Phenylalanine , Biochem. J. 26:917, 1932. 189. Levine, S. Z.; Dann, M., and Marples, E.: A Defect in the Metabolism of Tyrosine and Phenylalanine in Premature Infants: III. Demonstration of the Irreversible Conversion of Phenylalanine to Tyrosine in the Human Organism , J. Clin. Chem. 22:551, 1943. 190. Levine, S. Z.; Marples, E., and Gordon, H. H.: A Defect in the Metabolism of Aromatic Amino Acids in Premature Infants: The Role of Vitamin C , Science 90:620, 1939. 191. Nitowsky, H. M.; Govan, C. D., Jr., and Gordon, H. H.: Effect of Hemopoietic and Other Agents on the Hydroxyphenyluria of Premature Infants , A.M.A. Am. J. Dis. Child. 85:462, 1953. 192. Katz, E. J., and Hasterlik, R. J.: Amino-Aciduria Following Total Body Irradiation in Human Beings , J. Lab. & Clin. Med. 44:816, 1954. 193. Ghadimi, H., and Shwachman, H.: Evaluation of Aminoaciduria in Infancy and Childhood: Pt 2, unpublished data. 194. Sutton, H. E., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 99. 195. Stearns, G.: Creatin and Creatinine Metabolism in Infancy: Infant Metabolism , in Proceeding of W.H.O., Oct.-Nov., 1950, edited by I. H. Scheinberg, New York, The Macmillan Company, 1956. 196. DeMaeyer, E. M., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 32. 197. Holt, E. L., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 103. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

Evaluation of Amino-Aciduria in Infancy and Childhood

Loading next page...
 
/lp/american-medical-association/evaluation-of-amino-aciduria-in-infancy-and-childhood-MgSiQXfrej

References (217)

Publisher
American Medical Association
Copyright
Copyright © 1960 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1960.02070030459009
Publisher site
See Article on Publisher Site

Abstract

Abstract Introduction Interest in the detection of free amino acids in the urine has been stimulated by the development of chromatographic techniques.1-5 As a result, new genetically determined conditions have been described, such as glycinuria6 and "Hartnup" disease.7 A number of well-known diseases have been further characterized by the excretion of increased or abnormal amino acids in the urine, as in rickets, scurvy, galactosemia, Wilson's disease, lead poisoning, and burns. The detection of amino-aciduria in certain mentally disturbed children8,9 offers a new biochemical approach in the study of the basic disturbance and may even suggest genetic implications. Reference to the occurrence of amino-aciduria in patients with mental disturbances can be found in the studies on phenylketonuria10,11 and Lowe's syndrome,12 and in the reports by Allan et al.8 and by Sutton and Read.9 In this report we shall discuss the principles of paper chromatography, summarize References 1. Consden, R.; Gordon, A. H., and Martin, A. J. P.: Quantitative Analysis of Protein: A Partition Chromagraphic Method Using Paper , Biochem. J. 38:224, 1944. 2. Smith, I.: Chromatographic Techniques , New York, Interscience Publishers, Inc., 1958, pp. 94-96. 3. Block, R. J.; Durrum, E. L., and Zweig, G.: Paper Chromatography and Paper Electrophoresis , New York, Academic Press, Inc., 1955. 4. Dent, C. E.: A Study of the Behavior of Some 60 Amino Acids and Other Ninhydrin Reacting Substances on Phenol-'Collidine' Filter Paper Chromatograms with Notes as to the Occurrence of Some of Them in Biological Fluids , Biochem. J. 43:168, 1948. 5. Woolf, L. I.: Paper Chromatography , Great Ormond St. J. No. 1-2:61, 1951. 6. deVries, A.; Kochwa, S.; Lazebuik, J.; Frank, M., and Djaldetti, M.: Glycinuria, a Hereditary Disorder Associated with Nephrolithiasis , Am. J. Med. 23:408, 1957.Crossref 7. Baron, D. N.; Dent, C. E.; Harris, A.; Hart, E. W., and Jepson, J. B.: Hereditary Pellagra-like Skin Rash with Temporary Cerebellar Ataxia, Constant Renal Amino-Aciduria, and Other Bizarre Biochemical Features , Lancet 2: 421, 1956.Crossref 8. Allan, J. D.; Cusworth, D. C.; Dent, C. E., and Wilson, V. K.: A Disease, Probably Hereditary, Characterized by Severe Mental Deficiency and a Constant Gross Abnormality of Amino Acid Metabolism , Lancet 1:182, 1958.Crossref 9. Sutton, H. E., and Read, J. H.: Abnormal Amino Acid Metabolism in a Case Suggesting Autism , A.M.A. Am. J. Dis. Child. 96:23, 1958. 10. Jervis, G. J.; Block, R. J.; Bolling, D., and Kanze, E.: Chemical and Metabolic Studies on Phenylalanine , J. Biol. Chem. 134:105, 1940. 11. Knox, W. E., and Hsia, D. Y-Y.: Pathogenetic Problems in Phenylketonuria , Am. J. Med. 22:687, 1957.Crossref 12. Lowe, C. U.; Terrey, M., and MacLachlan, E. A.: Organicaciduria, Decreased Renal Ammonia Production, Hydrophthalmos, and Mental Retardation , A.M.A. Am. J. Dis. Child. 83:164, 1952. 13. Gale, E. F.: The Bacterial Amino Acid Decarboxylases , Advances Enzymol. 6:1, 1946. 14. Van Slyke, D. D.; MacFayden, D., and Hamilton, P. B.: The Gasometric Determination of Amino Acids in Urine by Ninhydrin-Carbon Dioxide Method , J. Biol. Chem. 150:251, 1943. 15. Schweigert, B. S., and Snell, E. E.: Microbiologic Methods for the Estimation of Amino Acids , Nutrition Abstr. & Rev. 16:497, 1947. 16. Shemin, D.: Amino Acid Determination on Crystalline Bovine and Human Serum Albumin by the Isotope Dilution Method , J. Biol. Chem. 159:439, 1945. 17. Lederer, E., and Lederer, M.: Chromatography, A Review of Principles and Applications , New York, Elsevier Press, Inc., 1957. 18. Hanes, C. S.: Personal communication to the authors. 19. Ma, T. S., and Zuazago, G.: Micro-Kjedahl Determination of Nitrogen, a New Indicator and an Improved Rapid Method , J. Indust. & Engin. Chem. (Analyt. Ed.) 14:280, 1942. 20. Lauson, H. D.: Sources of Error in Plasma Creatinine Determination , J. Appl. Physiol. 4:227, 1951. 21. Hare, R. S.: Endogenous Creatinine in Serum and Urine , Proc. Soc. Exper. Biol. & Med. 74:148, 1950. 22. Moore, S., and Stein, W. H.: Photometric Ninhydrin Method for Use in the Chromatography of Amino Acids , J. Biol. Chem. 176:367, 1948. 23. Moore, S., and Stein, W. H.: A Modified Ninhydrin Reagent for the Photometric Determination Amino Acids and Related Compound , J. Biol. Chem. 211:907, 1954. 24. Ghadimi, H.; Stern, M., and Shwachman, H.: A Study of the Free Amino Acids in Sweat from Patients with Cystic Fibrosis , A.M.A. J. Dis. Child. 99:333, 1960. 25. Dent, C. E.: Chromatography in the Study of Amino Acid Metabolism , in Lectures on the Scientific Basis of Medicine , 1951-52 to 1954-55, London, Athlone Press, 1954, Vol. 2, p. 213. 26. Moore, S., and Stein, W. H.: Chromatography of Amino Acids on Sulfonated Polystyrene Resins , J. Biol. Chem. 192:663, 1951. 27. Moore, S., and Stein, W. H.: Procedures for the Chromatographic Determination of Amino Acids in 4% Cross-Linked Sulfonated Polystyrene Resins , J. Biol. Chem. 211:893, 1954. 28. Verghese, N., and Ramakrishnan, P. N.: A Simple Method for Desalting Biological Fluids for Paper Chromatography of Amino-Acids , J. Clin. Path. 10:104, 1957.Crossref 29. Ghadimi, H., and Shwachman, H.: Unpublished data. 30. Huisman, T. H. J.: L'Elimination des acides amines chez des enfants normaux d'ages differents , Arch. franc. pédiat. 14:166, 1957. 31. Westall, R. G.: The Amino Acids and Other Ampholytes of Urine: III. Unidentified Substances Excreted in Normal Human Urine , Biochem. J. 60:247, 1955. 32. Childs, B.: Urinary Excretion of Free Alpha-Amino Acid Nitrogen by Normal Infants and Children , Proc. Soc. Exper. Biol. & Med. 81:225, 1952. 33. Rivier, C., and Jeanneret, M. R.: Observations sur l'aminoacidurie du nourrisson bienportant , Helvet. paediat. acta 11:489, 1956. 34. Fowler, D. I.; Norton, P. M.; Cheung, M. W., and Pratt, E. L.: Observations on the Urinary Amino Acid Excretion in Man: The Influence of Age and Diet , Arch. Biochem. & Biophys. 68:452, 1957. 35. Harper, H. A.; Hutchin, M. F., and Kimmel, J. R.: Concentrations of 19 Amino Acids in Plasma and Urine of Fasting Normal Males , Proc. Soc. Exper. Biol. & Med. 80:768, 1952. 36. Harrison, H. E., and Harrison, H. C.: Aminoaciduria in Relation to Deficiency Diseases and Kidney Function , J.A.M.A. 164:1571, 1957. 37. Doolan, P. D.; Harper, H. A.; Hutchin, M. E., and Shreeve, W. W.: Renal Clearance of 18 Individual Amino Acids in Human Subjects , J. Clin. Invest. 34:1247, 1955. 38. Doolan, P. D.; Harper, H. A.; Hutchin, M. E., and Alpen, E. L.: The Renal Tubular Response to Amino Acid Loading , J. Clin. Invest. 35:888, 1956. 39. Dent, C. E., and Walshe, J. M.: Aminoacid Metabolism , Brit. M. Bull. 10:247, 1954. 40. Dent, C. E.: The Renal Acidurias , in Colloque sur les acides amines , Paris, Masson & Cie., 1954, p. 229. 41. Stein, W. H.: A Chromatographic Investigation of the Amino Acid Constituents of Normal Urine , J. Biol. Chem. 201:45, 1953. 42. Eckhard, R. D., and Davidson, C. S.: Urinary Excretion of Amino Acids by a Normal Adult Receiving Diets of Varied Protein Content , J. Biol. Chem. 177:687, 1949. 43. Evered, D. F.: The Excretion of Amino Acids by the Human: A Quantitative Study with Ion-Exchange Chromatography , Biochem. J. 62: 416, 1956. 44. Steele, B.; Sayberlich, H. E.; Reynolds, M., and Bayman, C. A.: Amino Acids in the Urine of Human Subjects Fed on Eggs or Soy Beans , J. Nutrition 33:209, 1947. 45. Dunn, M. S.; Camien, N. M.; Shankman, S., and Block, H.: Urinary Excretion of 12 Amino Acids by Normal Male and Female Subjects Measured Microbiologically , Arch. Biochem. 13:207, 1947. 46. Woodson, H. W.; Hier, S. W.; Solomon, J. D., and Bergein, O.: Urinary Excretion of Amino Acids by Human Subjects on Normal Diets , J. Biol. Chem. 172:613, 1958. 47. Woolf, L. I., and Norman, A. P.: The Urinary Excretion of Amino Acids and Sugars in Early Infancy , J. Pediat. 50:271, 1957. 48. Dent, C. E.: Clinical Applications of Aminoacid Chromatography , Scandinav. J. Clin. & Lab. Invest. 10:122, 1957. 49. Eckhardt, R. D.; Cooper, A. M.; Faloon, W. W., and Davidson, C. S.: Urinary Excretion of Amino Acids in Man , Tr. New York Acad. Sc. 10:284, 1948. 50. Snyderman, S. E.: Metabolism of Amino Acids , Pediatrics 21:117, 1958. 51. Dent, C. E., and Bickel, H.: Personal communication to the authors. 52. Lowe, C. U.: Congenital Abnormalities of Amino Acid in Renal Tubules , Pediatrics 21: 1039, 1958. 53. Lowe, C. U., and Bruck, E.: Primary Chronic Metabolic Acidosis with Organic Aciduria , Mod. Prob. Pediat. 3:509, 1958. 54. Kretchmer, N., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 81. 55. Hsia, D. Y-Y., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 92. 56. Dustin, J. P.; Moore, S., and Bigwood, E. J.: Chromatographic Studies on the Excretion of Amino Acid in Early Infancy , Metabolism 4:75, 1955. 57. Sereni, F.; McNamara, H.; Shibuya, M.; Kretchmer, N., and Barnett, H. L.: Concentration in Plasma and Rate of Urinary Excretion of Amino Acid in Premature Infants , Pediatrics 15: 575, 1955. 58. Pratt, E. L., and Hasselmeyer, E.: Urinary Amino Acid Excretion by Premature Infants , A.M.A. Am. J. Dis. Child. 88:659, 1954. 59. Page, E. W.; Glendening, M. B.; Dignam, W., and Harper, H. A.: The Causes of Histidinuria in Normal Pregnancy , Am. J. Obst. & Gynec. 68:110, 1954. 60. Wallfaff, E. B.; Brodie, E. C., and Borden, A. L.: Urinary Excretion of Amino Acids in Pregnancy , J. Clin. Invest. 29:1542, 1950. 61. Cecil, I. B., and Voge, B.: A Simple Test for Pregnancy , Brit. M.J. 2:829, 1929. 62. Crumpler, H. R.; Dent, C. E.; Harris, H., and Westall, R. G.: β-Aminoisobutyric Acid (α-Methyl-β-Alanine): A New Amino Acid Obtained from Human Urine , Nature , London 167: 307, 1951. 63. Gartler, S. M.; Firschein, L. I., and Gidaspow, T.: Some Genetical and Anthropological Consideration of Urinary β-Aminoisobutyric Acid Excretion , Acta genet. basel. 6:435, 1956-57. 64. Mudge, G. H.: Clinical Patterns of Tubular Dysfunction , Am. J. Med. 24:785, 1958. 65. De Grouchy, J., and Sutton, H. E.: A Genetic Study of β-Aminoisobutyric Acid Excretion , Am. J. Human Genet. 9:76, 1957. 66. Harris, H.: Family Studies on the Urinary Excretion of β-Aminoisobutyric Acids , Ann. Eugen. 18:43, 1953. 67. Jervis, G. A.: Excretion of Phenylalanine and Derivatives on Phenylpyruvic Oligophrenia , Proc. Soc. Exper. Biol. & Med. 75:83, 1950. 68. Armstrong, M. D., and Robinson, K. S.: On the Excretion of Indole Derivatives in Phenylketonuria (Letter to Editor) , Arch. Biochem. 52: 287, 1954. 69. Bickel, H.; Gerrard, J., and Hickmans, E. M.: The Influence of Phenylalanine Intake on the Chemistry and Behaviour of a Phenylketonuric Child , Acta paediat. 43:64, 1954. 70. Armstrong, M. D.; Shaw, K. N. F., and Robinson, K. S.: Studies on Phenylketonuria: II. The Excretion of Hydroxyphenylacetic Acid in Phenylketonuria , J. Biol. Chem. 213:797, 1955. 71. Armstrong, M. D., and Binkley, E. L.: Studies on Phenylketonuria: V. Observations on a Newborn Infant with Phenylketonuria , Proc. Soc. Exper. Biol. & Med. 93:418, 1956. 72. Hsia, D., and Richmond, S.: Detection of the Heterozygous Carrier , J. Ment. Def. 1:53, 1957. 73. Smith, A. J., and Strang, L. B.: An Inborn Error of Metabolism with the Urinary Excretion of α-Hydroxy-Butyric Acid and Phenylpyruvic Acid , Arch. Dis. Childhood 33:109, 1958. 74. Harris, H.: in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 106, 109. 75. Walshe, J. M.: Disturbances of Amino Acid Metabolism Following Liver Injury , Quart. J. Med. 22:483, 1953. 76. Hsia, D. Y-Y., and Gellis, S. S.: Amino Acid Metabolism in Infectious Hepatitis , J. Clin. Invest. 33:1603, 1954. 77. Jonxis, J. H. P., and Huisman, T. H. J.: Excretion of Amino Acid in Free and Bound Form During Intravenous Administration of Protein Hydrolysate , Metabolism 6:175, 1957. 78. Eckhardt, R. D., and Davidson, C.: Urinary Excretion of Amino Acids Following the Rapid Injection of a Solution of Amino Acid in Man , J. Clin. Invest. 29:727, 1948. 79. Christensen, H. N.;; Lynch, E. L., and Powers, J. H.: The Conjugated, Non-Protein, Amino Acids of Plasma: III. Peptidemia and Hyperpeptiduria as a Result of the Intravenous Administration of Partially Hydrolyzed Casein (Amigen) , J. Biol. Chem. 166:649, 1946. 80. Bickel, H., et al.: Cystine Storage Disease with Aminoaciduria and Dwarfism (Lignac-Fanconi Disease) , Acta paediat. ( (Supp. 90) ) 42:27, 1952. 81. Clay, R. D.; Darmady, E. M., and Hawkins, M.: The Nature of the Renal Lesion in the Fanconi Syndrome , J. Path. & Bact. 65:551, 1953. 82. Dern, P. L.: Aminoaciduria with Cystinosis: Case Report with Determination of Urinary Amino Acid and Ocular Cystine , Ann. Int. Med. 46:138, 1957. 83. Darmady, E. M.: Renal Lesions in Relation to Aminoaciduria and Water Diuresis , in Ciba Foundation Symposium on the Kidney , Boston, Little, Brown & Company, 1954, p. 27. 84. Maschas, H.; Lamotte, M.; Lamotte-Barillon, S., and Barbier, P.: Un Cas de cystinose revelée par une lithiase urinaire , Bull. et mem. Soc. méd hôp. Paris 64:491, 1948. 85. Debré, R.; Marie, J.; Cléret, F., and Messimy: Rachitisme tardif coexistant avec une néphrite chronique et une glycosurie , Arch. méd. Enf. 37:597, 1934. 86. Dent, C. E., and Harris, H.: Hereditary Forms of Rickets and Osteomalacia , J. Bone & Joint Surg. 38B:204, 1956. 87. Fanconi, G.: Weitere Beiträge zur Cystinkrankheit (der Amindiabetes) , Helvet. paediat. acta 1:183, 1946. 88. Fanconi, G., and Bickel, H.: Die chronische Aminoacidurie (aminosaure Diabetes oder nephrotisch-glukosurischer Zwergwuchs) bei der Glykoserose und der Cystinkrankheit , Helvet. paediat. acta 4:359, 1949. 89. Beumer, H., and Weppler, W.: Über die Cystinkrankheit der ersten Lebenszeit , Klin. Wchnschr. 16:8, 1937. 90. Hottinger, A.: Über Cystin-Diathese , Ann. paediat. 156:257, 1941. 91. Bürki, E.: Über Augenveränderungen bei der Cystinkrankheit , Ann. paediat. 156:324, 1941. 92. Freudenberg, E.: Weitere Beobachtungen zur Frage der Cystinosis , Ann. paediat. 182:85, 1954. 93. Bernstein, J.; Meyer, R., and Cohen, F.: The DeToni-Fanconi Syndrome in Childhood, The Society for Pediatric Research Program and Abstracts, 29th Annual Meeting, Buck Hill Falls, Pa., May 8-9, 1959. 94. Freudenberg, E.: Cystinosis: Cystine Disease (Lignac's Disease) in Children , Advances Pediat. 4:265, 1949. 95. Fanconi, G.: Der frühinfantile nephrotischglykosurische Zwergwuchs mit hypophosphatä mischer Rachitis , Jahrb. Kinderh. 147:299, 1936. 96. McCune, D. J.; Mason, H. H., and Clarke, H. T.: Intractable Hypophosphatemic Rickets with Renal Glycosuria and Acidosis (The Fanconi Syndrome) , Am. J. Dis. Child. 65:81, 1943.Crossref 97. Dent, C. E.: Amino Aciduria in the Fanconi Syndrome: A Study Making Extensive Use of Techniques Based on Paper Partition Chromatography , Biochem. J. 41:240, 1947. 98. Williamson, D. A. J.: Cystinosis , Arch. Dis. Childhood 27:356, 1952.Crossref 99. Eberlein, W. R.: Aminoaciduria in Childhood: Cystinuria and Cystinosis , Am. J.M. Sc. 225:677, 1953.Crossref 100. Parker, W. S.; Prader, A., and Fanconi, G.: Further Observations on Cystine Storage Disease , Pediatrics 16:228, 1955. 101. Salassa, R. M.; Power, M. H.; Ulrich, J. A., and Hayles, A. B.: Observations on the Metabolic Effects of Vitamin D in Fanconi's Syndrome , Proc. Staff Meet. Mayo Clin. 29:214, 1954. 102. McCune, D. J.; Mason, H. H., and Clarke, H. T. L.: Late Rickets with Glycosuria and Organic Acid Acidosis (Fanconi Type) , Am. J. Dis. Child. 58:673, 1939. 103. Jackson, H. R., and Clarke, B. E.: Cystinosis: Report of 2 Cases with Postmortem Examination , A.M.A. Am. J. Dis. Child. 85:513, 1953. 104. Lathem, W.; Baker, K., and Bradley, S. E.: Urinary Amino Acid Excretion in Renal Disease, with Observations on Fanconi Syndrome , Am. J. Med. 18:249, 1955.Crossref 105. Milne, M. D.; Stanbury, W. S., and Thomson, A. E.: Observations on the Fanconi Syndrome and Renal Hyperchloraemic Acidosis in the Adult , Quart. J. Med. 21:61, 1952. 106. Dragsted, P. J., and Hjorth, N.: Fanconi's Syndrome , Acta med. scandinav. 146:317, 1953.Crossref 107. Drablos, A.: The de Toni-Fanconi Syndrome with Cystinosis , Acta paediat. 40:438, 1951.Crossref 108. King, F. P., and Lochridge, E. P.: Cystinosis (Cystine-Storage Disease) , A.M.A.J. Dis. Child. 82:446, 1951. 109. Myerson, R. M., and Pastor, B. H.: Fanconi Syndrome and Its Clinical Variants , Am. J. M. Sc. 228:378, 1954.Crossref 110. Russell, D. S., and Barrie, H. J.: Storage of Cystine in Reticulo-Endothelial System and Its Association with Chronic Nephritis and Renal Rickets , Lancet 2:889, 1936. 111. Worthen, G., and Good, A.: The de Toni-Fanconi Syndrome with Cystinosis Clinical and Metabolic Study of 2 Cases in a Family and a Critical Review on the Nature of the Syndrome , A.M.A.J. Dis. Child. 95: 653, 1958.Crossref 112. Skinner, W.; Dortis, C., and Paul, J. R., Jr.: Amino-Aciduria in Childhood, Cystinuria or Cystinosis , J. South Carolina M.A. 53:203, 1957. 113. de Toni, G.: Remarks on the Relations Between Renal Rickets (Renal Dwarfism) and Renal Diabetes , Acta paediat. 16:479, 1933.Crossref 114. de Toni, G.: Renal Rickets with Phospho-Gluco-Amino Renal Diabetes (de Toni-Debré-Fanconi Syndrome) , Ann. paediat. 187:42, 1956. 115. Bickel, H., and Thursby-Pelham, D. C.: Hyperamino-Aciduria in Lignac-Fanconi Disease, in Galactosaemia and in an Obscure Syndrome , Arch. Dis. Childhood 29:224, 1954.Crossref 116. Robson, E., and Rose, G. A.: The Effect of Intravenous Lysine on the Renal Clearances of Cystine, Arginine and Ornithine in Normal Subjects, in Patients with Cystinuria and Fanconi Syndrome, and in Their Relatives , Clin. Sc. 16:75, 1957. 117. Hottinger, A.: Zur Cystindiathese , Ann. paediat. 169:272, 1947. 118. Luder, J., and Sheldon, W.: A Familial Tubular Absorption Defect of Glucose and Amino-Acids , Arch. Dis. Childhood 30:160, 1955.Crossref 119. Harris, H: Renal Aminoaciduria , Brit. M. Bull. 13:26, 1957. 120. Dent, C. E., and Rose, G. A.: Aminoacid Metabolism in Cystinuria , Quart. J. Med. 20:205, 1951. 121. Brooks, W. D. W.; Heasman, M. A., and Lovell, R. R. H.: Retinitis Pigmentosa Associated with Cystinuria , Lancet 1:1096, 1949.Crossref 122. Lewis, H. B.: Occurrence of Cystinuria in Healthy Young Men and Women , Ann. Int. Med. 6:183. 1932.Crossref 123. Prakken, H.: Case of Congenital Cystinuria , J. Pediat. 40:186, 1952.Crossref 124. Yeh, H. L.: Frankl, W.; Dunn, M. S.; Parker, P.; Hughes, B., and Gyorgi, P.: Urinary Excretion of Amino Acids by a Cystinuric Subject , Am. J. M. Sc. 214:507, 1947.Crossref 125. Dent, C. E.; Senior, B., and Walshe, J. M.: Pathogenesis of Cystinuria: II. Polarographic Studies of the Metabolism of Sulphur-Containing Amino-Acids , J. Clin. Invest. 33:1216, 1954.Crossref 126. Lewis, H. B.; Brown, B. H., and White, G. R.: Metabolism of Sulphur: XXIII. The Influence of the Ingestion of Cystine, Cysteine and Methionine on the Excretion of Cystine in Cystinuria , J. Biol. Chem. 114:171, 1936. 127. Brown, B. H., and Lewis, H. B.: Cystine in Normal and Cystinuric Human Blood , Proc. Soc. Exper. Biol. & Med. 36:487, 1937. 128. Cleland, J. B.: Cystine, Methionine and Sulphate Excretion in Cystinuria , Australian J. Exper. Biol. & M. Sc. 24:210, 1946. 129. Cleland, J. B.: Cystine, Methionine and Sulphate Excretion in Cystinuria , Australian J. Exper. Biol. & M. Sc. 29:85, 1951. 130. Cleland, J. B.: Cystine, Methionine and Sulphate Excretion in Cystinuria , Australian J. Exper. Biol. & M. Sc. 25:33, 1947. 131. Harris, H., and Robson, E.: Variation in Homozygous Cystinuria , Acta genet. 5:381, 1955. 132. Dent, C. E.; Heathcote, J. G., and Joron, G. E.: The Pathogenesis of Cystinuria: I. Chromatographic and Microbiological Studies of the Metabolism of Sulphur-Containing Amino-Acids , J. Clin. Invest. 33:1210, 1954. 133. Stein, W. H.: Excretion of Amino Acids in Cystinuria , Proc. Soc. Exper. Biol. & Med. 78:705, 1951. 134. Fowler, D., and Harris, H.: Plasma Cystine Levels in Cystinuria , Lancet 1:544, 1952.Crossref 135. Dent, C. E., and Harris, H.: The Genetics of "Cystinuria," Ann. Eugen. 16:60, 1951.Crossref 136. Harris, H.; Mittwoch, U.; Robson, E. B., and Warren, F. L.: The Pattern of Amino-Acid Excretion in Cystinuria , Ann. Human Genet. 19:196, 1955.Crossref 137. Harris, H., and Warren, F. L.: Quantitative Studies on the Urinary Cystine in Patients with Cystine Stone Formation and in Their Relatives , Ann. Eugen. 18:125, 1953.Crossref 138. Bearn, A.: An Inborn Error of Metabolism with Multiple Manifestations , Am. J. Med. 22:747, 1957.Crossref 139. Iber, F. L.; Chalmers, T. C., and Uzman, L.: Studies of Protein Metabolism in Hepatolenticular Degeneration , Metabolism 6:388, 1957. 140. Stein, W. H.; Bearn, A. A., and Moore, S.: The Amino Acid Content of the Blood and Urine in Wilson's Disease , J. Clin. Invest. 33:410, 1954.Crossref 141. Uzman, L. L., and Denny-Brown, D.: Amino-Aciduria of Wilson's Disease (Hepatolenticular Degeneration) , Am. J. M. Sc. 223:392, 1952.Crossref 142. DeVerdier, C. H.: Paper Chromatographic Analysis of Amino Acid Excretion in Wilson's Disease , Acta med. scandinav. 138:344, 1950.Crossref 143. Gilsanz, V.; Segovia, J. M., and Castro Mendoza, H.: Contribution to Study of Wilson's Disease , A.M.A. Arch. Int. Med. 95:727, 1955.Crossref 144. Uzman, L. L., and Hood, B.: The Familial Nature of the Amino-Aciduria of Wilson's Disease (Hepatolenticular Degeneration) , Am. J. M. Sc. 223:392, 1952.Crossref 145. Bearn, A. G., and Kunkel, H. G.: Abnormalities of Copper Metabolism in Wilson's Disease and Their Relationship to Aminoaciduria , J. Clin. Invest. 33:400, 1954.Crossref 146. Cooper, A. M.; Eckhardt, D.; Faloon, W. W., and Davidson, C. S.: Investigation of the Aminoaciduria in Wilson's Disease (Hepatolenticular Degeneration): Demonstration of a Defect in Renal Function , J. Clin. Invest. 29:265, 1950.Crossref 147. Chalmers, T. C.; Iber, F., and Uzman, L. L.: Hepatolenticular Degeneration (Wilson's Disease) as a Form of Idiopathic Cirrhosis . New England J. Med. 256:235, 1957.Crossref 148. Uzman, L. L.: Amino Aciduria in Hepatolenticular Degeneration (Wilson's Disease) , Am. J. M. Sc. 215:33, 1948.Crossref 149. Uzman, L. L.: On the Relationship of Urinary Copper Excretion to the Amino Aciduria in Wilson's Disease , Am. J. M. Sc. 226:45, 1953.Crossref 150. Hsia, D. Y-Y.; Hsia, H. H.; Green, S.; Kay, M., and Gellis, S. S.: Amino-Aciduria in Galactosemia , A.M.A. Am. J. Dis. Child. 88:458, 1954. 151. Holzel, A.; Komrower, G. M., and Wilson, V. K.: Amino-Aciduria in Galactosaemia , Brit. M. J. 1:194, 1952.Crossref 152. Holzel, A., and Komrower, G. M.: A Study of the Genetics of Galactosaemia , Arch. Dis. Childhood 30:155, 1955.Crossref 153. Komrower, G. M.: Schwarz, V.; Holzel, A., and Goldberg, L.: A Clinical and Biochemical Study of Galactosaemia , Arch. Dis. Childhood 31:254, 1956.Crossref 154. Komrower, G. M.: L'Amino-Acidurie dans la galactosémie , Arch. franc pédiat. , 10:185, 1953. 155. Bickel, H., and Hickmans, E. M.: Paper Chromatographic Investigations on the Urine of Patients R. T. and R. R. , Arch. Dis. Childhood 27:348, 1952. 156. Cusworth, D. C.; Dent, C. E., and Flynn, F. V.: The Amino-Aciduria in Galactosaemia , Arch. Dis. Childhood 30:150, 1955.Crossref 157. Henderson, W.: A Case of Hartnup's Disease , Arch. Dis. Childhood 33:114, 1958.Crossref 158. Westall, R. G.; Dancis, J., and Miller, S.: Maple Syrup Urine Disease , A.M.A.J. Dis. Child. 94:571, 1957. 159. Thelander, H. E., and Imagawa, R.: Amino Aciduria, Congenital Defects, and Mental Retardation: a Preliminary Report , J. Pediat. 49:123, 1956.Crossref 160. Schönenberg, H.: Papierchromatographische Untersuchungen bei der Pfaundler-Hurlerschen Krankheit , Monatsschr. Kinderh. 102:404, 1954. 161. Ames, S. R., and Risley, H. A.: Aminoaciduria in Progressive Muscular Dystrophy , Proc. Soc. Exper. Biol. & Med. 68:131, 1948. 162. Nestel, P. J., and Nestel, L.: Investigation of Aminoaciduria in Erythro-Blastosis Foetalis and Its Possible Relationship to Kernicterus , M.J. Australia 1:909, 1957. 163. Steinberg, H.; Szeinberg, A., and Cohen, B. E.: Aminoaciduria and Hypermetabolism in Progeria , Arch. Dis. Childhood 32:401, 1957. 164. Gray, C. H., and Illing, E. K. B.: Plasma and Urinary Amino-Acids in Diabetes , J. Endocrinol. 8:44, 1952. 165. Gross, J.; Comfort, M., and Ulrich, J.: Abnormalities of Serum and Urinary Amino Acids in Hereditary and Nonhereditary Pancreatitis , Tr. A. Am. Physicians 70:127, 1957. 166. van Creveld, S., and Aarons, P.: Transitory Renal Osteoporosis with Aminoaciduria and Development of Hypersensitivity to Vitamin D , Ann. paediat. 173:299, 1949. 167. Bickel, H.: Zum Mechanismus der Aminoacidurie, presented at the Eighth International Congress of Paediatrics, Copenhagen, 1956. 168. Altman, K. L., and Miller, G.: A Disturbance of Tryptophan Metabolism in Congenital Hypoplastic Anaemia , Nature , London 172:868, 1953. 169. Huisman, T. H. J.: The Concentration of Different Amino Acids in the Blood Plasma in Children Suffering from Rickets and Scurvy , Pediatrics 14:245, 1954. 170. Jonxis, J. H. P.: Amino-Aciduria and Rickets , Helvet. paediat. acta 10:245, 1955. 171. Jonxis, J. H. P., and Huisman, T. H. J.: Amino-Aciduria in Rachitic Children , Lancet 2:428, 1953. 172. Jonxis, J. H. P., and Huisman, T. H. J.: Amino Aciduria and Ascorbic Acid Deficiency , Pediatrics 14:238, 1954. 173. Eades, C. H., Jr.; Pollack, R. L., and Hardy, J. D.: Thermal Burns in Man: IX. Urinary Amino Acid Patterns , J. Clin. Invest. 34:1756, 1955. 174. Nardi, G. L.: "Essential" and "Non-Essential" Amino Acids in Urine of Severely Burned Patients , J. Clin. Invest. 33:847, 1954. 175. Woolf, L. I., and Giles, H. M. C.: Urinary Excretion of Amino-Acids and Sugar in the Nephrotic Syndrome , Acta paediat. 45:489, 1956.Crossref 176. Shreeve, W. W.; Hutchin, M. E.; Harper, H. A.; Miller, C. D., and Doolan, P. D.: Excretion of Amino Acids in Nephrosis , Proc. Soc. Exper. Biol. & Med. 88:510, 1955. 177. Wilson, V. K.; Thomson, M. L., and Dent, C. E.: Amino-Aciduria in Lead Poisoning , Lancet 2:66, 1953. 178. Chisholm, J. J., Jr.; Harrison, H. C.; Eberlein, W. R., and Harrison, H. E.: Amino Aciduria, Hypophosphatemia and Rickets in Lead Poisoning , A.M.A.J. Dis. Child. 89:159, 1955. 179. Rothstein, A., and Berke, H.: Amino-Aciduria in Uranium Poisoning , J. Pharmacol. & Exper. Therap. 96:179, 1949. 180. Voegtlin, C., and Hodge, H. C., Editors: Pharmacology and Toxicology of Uranium Compounds , National Nuclear Energy Series, Manhattan Project Technical Section, Division 6, No. 2, New York, McGraw-Hill Book Company, Inc., 1949, p. 972. 181. Spencer, A. G., and Franglen, G. T.: Gross Amino-Aciduria Following Lysol Burn , Lancet 1:190, 1952. 182. Knox, W. E.: Personal communication to the authors. 183. Sarrouy, C.; Cabannes, R., and Clausse, J.: Étude des acides amines du sang et des urines au cours des syndrome de denutrition du nourrissoh , Presse méd. 65:1221, 1957. 184. Schendel, H. E.; Antonis, A., and Hansen, J.: Increased Amino-Aciduria in Infants with Kwashiorkor Fed Natural and Synthetic Diets , Pediatrics 23:662, 1959. 185. Cheung, M. W.; Fowler, D. I; Norton, P. M.; Synderman, S. E., and Holt, J. E., Jr.: Observations on Amino Acid Metabolism in Kwashiorkor , J. Trop. Pediat. 1:141, 1955. 186. Kretchmer, N.: Disorders Associated with the Metabolism of Phenylalanine and Tyrosine , Pediatrics 21:445, 1958. 187. Levine, S. Q.; Marples, E., and Gordon, H. H.: A Defect in the Metabolism of Tyrosine and Phenylalanine in Premature Infants: I. Identification and Assay of Intermediary Products; II. Spontaneous Occurrence and Eradication by Vitamin C , J. Clin. Invest. 20:209, 1941. 188. Medes, G.: A New Error of Tyrosine Metabolism: Tyrosinosis; The Intermediary Metabolism of Tyrosine and Phenylalanine , Biochem. J. 26:917, 1932. 189. Levine, S. Z.; Dann, M., and Marples, E.: A Defect in the Metabolism of Tyrosine and Phenylalanine in Premature Infants: III. Demonstration of the Irreversible Conversion of Phenylalanine to Tyrosine in the Human Organism , J. Clin. Chem. 22:551, 1943. 190. Levine, S. Z.; Marples, E., and Gordon, H. H.: A Defect in the Metabolism of Aromatic Amino Acids in Premature Infants: The Role of Vitamin C , Science 90:620, 1939. 191. Nitowsky, H. M.; Govan, C. D., Jr., and Gordon, H. H.: Effect of Hemopoietic and Other Agents on the Hydroxyphenyluria of Premature Infants , A.M.A. Am. J. Dis. Child. 85:462, 1953. 192. Katz, E. J., and Hasterlik, R. J.: Amino-Aciduria Following Total Body Irradiation in Human Beings , J. Lab. & Clin. Med. 44:816, 1954. 193. Ghadimi, H., and Shwachman, H.: Evaluation of Aminoaciduria in Infancy and Childhood: Pt 2, unpublished data. 194. Sutton, H. E., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 99. 195. Stearns, G.: Creatin and Creatinine Metabolism in Infancy: Infant Metabolism , in Proceeding of W.H.O., Oct.-Nov., 1950, edited by I. H. Scheinberg, New York, The Macmillan Company, 1956. 196. DeMaeyer, E. M., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 32. 197. Holt, E. L., in Amino Acid and Protein Metabolism , Report of the 30th Ross Conference on Pediatric Research, edited by S. J. Foman, Columbus, Ohio, Ross Laboratories, 1959, p. 103.

Journal

A.M.A. Journal of Diseases of ChildrenAmerican Medical Association

Published: Apr 1, 1960

There are no references for this article.