ARGININOSUCCINICACIDURIA: CLINICAL, METABOLIC AND DIETARY STUDY
D. P. BRENTON
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
Search for more papers by this authorD. C. CUSWORTH
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
Search for more papers by this authorSANDRA HARTLEY
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
Search for more papers by this authorSUSAN LUMLEY
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
Search for more papers by this authorCorresponding Author
J. A. KUZEMKO
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
J. A. Kuzemko, District Hospital, Peterborough.Search for more papers by this authorD. P. BRENTON
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
Search for more papers by this authorD. C. CUSWORTH
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
Search for more papers by this authorSANDRA HARTLEY
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
Search for more papers by this authorSUSAN LUMLEY
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
Search for more papers by this authorCorresponding Author
J. A. KUZEMKO
Patdiatric Department, Peterborough District Hospital, Peterborough, and University College Hospital Medical School, London
J. A. Kuzemko, District Hospital, Peterborough.Search for more papers by this author
REFERENCES
- Allan, J. D., Cusworth, D. C., Dent, C. E. and Wilson, V. K. (1958) A disease, probably hereditary, characterised by severe mental deficiency and a constant gross abnormality of amino acid metabolism. Lancet 1, 182.
- Cusworth, D. C. and Dent, C. E. (1960) Renal clearances of amino acids in normal adults and in patients with aminoaciduria. Biochem. J. 74, 550.
- Cusworth, D. C. and Westall, R. G. (1961) Determination of argininosuccinic acid by ion-exchange chromatography. Nature 192, 555.
- Fenton, J. G. and Williams, A. H. (1968) Improved method for the estimation of plasma ammonia by ion exchange, J. Clin. Path. 21, 14.
- Levin, B., Mackay, M.M., and Oberholzer, V. G. (1961) Argininosuccinic aciduria: an inborn error of amino acid metabolism. Arch. Dis. Childh. 36, 622.
- Levin, B., Oberiiolzer, V. G. and Sinclair, L. (1969) Biochemical investigations of hypo-ammonaemia. Lancet 2, 170.
- Miller, A.L., and McLEan, P. (1967) Urea cycle enzymes in the liver of a patient with argininosuccinic aciduria. Clin. Sci. 32, 385.
- Moser, H. W., Efron, M. L., Brown, H., Diamond, R. and Neumann, C. G. (1967) Artgininosuccinic aciduria: report of two new cases and demonstration of intermittent elevation of blood ammonia. Clinical Studies 42, 9.
- Spackman, D. H., Stein, W. H. and Moore, S. (1958) Automatic recording apparatus for use in chromatography of amino acids. Analyt. Chem. 30, 1190.
- Stein, W. H. and Moore, S. (1954) The free aminoacids of human blood plasma. J. biol. Chem. 211, 915.
- Tanner, J. M., Whitehouse, R. H. and Takaishi (1966) Standards from birth to maturity for height, weight, height velocity and weight velocity: British Children 1965, Part 1. Arch. Dis. Child. 41, 219.
- Tomlinson, S. and Wesstall, R. G. (1960) Argininosuccinase activity in brain tissue. Nature 188, 235.
- Westall, R. G. (1960) Argininosuccinic aciduria: identification and reactions of the abnormal metabolite in a newly described form of mental disease, with some preliminary metabolic studies. Biochem. J. 77, 135.
