Skip to main content
Log in

Urinary aminoacics in mental retardation

  • Original Articles
  • Published:
The Indian Journal of Pediatrics Aims and scope Submit manuscript

“Mental retardation is perhaps the greatest single source of human suffering. The child born mentally retarded is not only a tragic human figure in himself, living yet not alive, since he is never to be what he could have been, but he is the innocent agent of profound and endless suffering to his family and a perpetual burden to society” (Lyman 1963).

Summary

40 cases of idiopathic mental retardation, 12 cases of post-traumatic brain damage, 3 gargoyles, 3 cretins and 5 mongols were studied for their urinary aminoacids. In the first two groups, there was no significant abnormality detected. A moderate hyperamino-aciduria was seen in 2 gargoyles and hypoaminoaciduria in cretins. The urinary aminoacidgram of mongols was normal. The pathogenesis of aminoacid excretion and its relation to mental retardation is discussed.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  • Baron, D. N., Dent, C. E., Harris, H., Hart, F. W. and Jepson, J. B. (1956). H. Disease.Lancet,2, 421.

    Article  Google Scholar 

  • Benda, C. E. (1947). Mongolism and Cretinism.Heinemann, London.

    Google Scholar 

  • Berry, H. K. (1960). Aminoacid excretion in 700 children.Metabolism,9, 363.

    CAS  Google Scholar 

  • Bessman, S. P. and Baldwin, R. (1962). Imidazole aminoaciduria in cerebromacular degeneration.Science,135, 789.

    Article  PubMed  CAS  Google Scholar 

  • Carson, N. A. J. and Neil, D. W. (1962). Metabolic surveys in mentally backward individuals in Northern Ireland.Arch. Dis. Childh. 37, 505.

    PubMed  CAS  Google Scholar 

  • Carver, M. J., Wiltre, H. and Wittson, C. L. (1959). Basic studies in mongolism.Dis. nerv. Syst. 20, 162.

    PubMed  CAS  Google Scholar 

  • Carver, M. J., Stephen, J., Paska, D. R. and Witson, C. (1962). Aminoaciduria in selected metabolic errors.Metabolism,11, 357.

    PubMed  CAS  Google Scholar 

  • Childs, B., Nyhan, W. L., Borden, M., Bard, L. and Cooke, R. E. (1961). Idiopathic hyperglycenemia and hyperglycinuria, a new disorder of aminoacid metabolism.Pediatrics,27, 522.

    PubMed  CAS  Google Scholar 

  • Crispel, K. A., William, G., Hollified, G. and Parson, W. (1961). Alteration of protein metabolism in myxodema.J. Chron. Dis. 14, 507.

    Article  Google Scholar 

  • Dhatt, P. S., Gupta, H. L., Saini, A. S. (1967). Significance of aminoaciduria in Indian childhood cirrhosis.Indian J. Pediat. 34, 195.

    PubMed  CAS  Google Scholar 

  • Eayrs, J. T. (1960). Influence of thyroid on central nervous system.Brit. med. Bull. 16, 122.

    PubMed  CAS  Google Scholar 

  • Ghadimi, H. and Schwachman, H. (1960). Evaluation of aminoaciduria in infancy and childhood.Amer. J. Dis. Child. 99, 457.

    CAS  Google Scholar 

  • Ghadimi, H. (1961). A familial disturbance of histidine metabolism.New Engl. J. Med. 265, 221.

    Article  PubMed  CAS  Google Scholar 

  • Handerson, J. L., Mogregor, A. R., Thannhauser, S. J. and Helden, R. (1952). Pathology and biochemistry of gargoylism. Report of three cases with review of literature.Arch. Dis. Childh 27, 230.

    Google Scholar 

  • Harris, H., Penrose, L. S. and Thomas, D. H. (1959). Cystathioninuria.Ann. human Genet. 23, 442.

    Article  CAS  Google Scholar 

  • Lavitola, G. and Periante, F. (1957). Studies of aminoacid metabolism in mongoloid parasthenia.Acta. Neurol. 12, 751.

    CAS  Google Scholar 

  • Lvman, F. L. (1963). Phenylketonuria.Charles C. Thomas, Springfield, Ill.

    Google Scholar 

  • Mackenzie, D. Y. and Woolf, L. I. (1959). Maple syrup urine disease. Inborn error of valine. leucine and isoleucine associated with gross mental deficiency.Brit. med. J. 1, 90.

    Article  PubMed  CAS  Google Scholar 

  • McMurray, W. C. (1962). Citrullinuria, a new aminoaciduria associated with mental retardation.Lancet,1, 138.

    Article  Google Scholar 

  • Perry, T. L., Shaw, K. N. F. and Walkin, D. (1959). Urinary excretion of aminobutyric acid in mongolism.Nature,189, 1970.

    Article  Google Scholar 

  • Poser, C. M. (1963). Aminoaciduria in familial mental retardation.Arch. Neurol. 9, 35.

    PubMed  CAS  Google Scholar 

  • Saini, A. S., Singh, I. D. and Singh, A. J. (1963). Patterns of urinary aminoacids in normal Punjabis.Indian J. med. Res. 51, 941.

    PubMed  CAS  Google Scholar 

  • Schonberg, H. (1954). Quoted by Synderman, S. E. (1958). Metabolism of aminoacids, a review.Pediatrics,21, 117.

    Google Scholar 

  • Shafer, I. A., Scriver, C. R., and Efron, M. L. (1962). Familial hyperprolinemia, cerebral dysfunction and renal anomalies occurring in a family with hereditary nephropathy and deafness.New Engl. J. Med. 267, 51.

    Article  Google Scholar 

  • Thelander, H. E. and Imagawa, R. (1952). Aminoaciduria and mentally defective children.Acta Pediat. 52, 41.

    Google Scholar 

  • Wada, Y.et al. (1963). Quoted by Lowe in Textbook of Pediatrics, Ed. Waldo E. Nelson,W. B. Saunders, Philadelphia, 1964.

    Google Scholar 

  • Werner, S. C. (1962). Thyroid.Harper and Row, New York.

    Google Scholar 

  • Wright, S. W. and Fink, K. (1957). Excretion of β- aminoisobutyric acid in normal and mongoloid children.Amer. J. ment. Def. 61, 530.

    CAS  Google Scholar 

  • Wright, S. W. (1960). Investigations of mentally defective children. Studies on urinary aminoacids.J. ment. Def Res. 4, 32.

    CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

From the Departments of Pediatrics and Physiology, Medical College, Rohtak.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Dhatt, P.S., Gupta, H.L. & Saini, A.S. Urinary aminoacics in mental retardation. Indian J Pediatr 35, 469–475 (1968). https://doi.org/10.1007/BF02753118

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02753118

Keywords

Navigation