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Contribution to Study of Wilson's Disease

Contribution to Study of Wilson's Disease Abstract We regard it of interest to report our study of three brothers affected with Wilson's disease, in two of whom the process appeared with its most characteristic clinical picture and in one of whom the only finding in relation to this disease was the presence of amino-aciduria. The nature of the hepatolenticular degeneration that appears in Wilson's disease is yet unknown, but in the last few years, by evaluation of the disturbances of the metabolism of copper and the amino acids eliminated in the urine, new concepts have been brought forth which are worthy of further research. Baker,1 in 1948, summarized the theories which up to that time had tried to explain the nature of Wilson's disease as follows: 1. It is a degenerative process affecting the liver and brain. 2. It is an acquired disease, and both organs are damaged simultaneously by an endogenous toxin. 3. The primary References 1. References 16 and 17. 2. Baker, A. B.: Arch. Path. 46:268, 1948. 3. Lipmann, F.: Biochem. Ztschr. 268:314, 1934. 4. Denny-Brown, D., and Porter, H.: New England J. Med. 245:917, 1951.Crossref 5. Uzman, L., and Denny-Brown, D.: Am. J. M. Sc. 215:599, 1948.Crossref 6. Cooper, A. M.; Eckart, R. D.; Faloon, W. W., and Davidson, C. S.: J. Clin. Invest. 29:265, 1950.Crossref 7. Uzman, L. L., and Hood, B.: Am. J. M. Sc. 223:392, 1952.Crossref 8. Dent, C. E., and Harris, H.: Ann. Eugenics 16:60, 1951.Crossref 9. De Verdier, C. H.: Acta med. scandinav. 138:344, 1950.Crossref 10. Hood, B., and Fagerberg, S. E.: Acta med. scandinav. 140:374, 1951.Crossref 11. Himsworth, H. P.: Lectures on the Liver and Its Diseases , Ed. 2, Cambridge, Mass., Harvard University Press, 1950. 12. Dent, C. E.: Clin. Proc. 8:21, 1949. 13. Clay, R. D.; Darmady, E. A., and Hawkins, M.: J. Path. & Bact. 65:551, 1953. 14. Bickel, H.: Lancet 2:403, 1953. 15. Hottinger, A.: Ann. pædiat. 169:272, 1947. 16. Drabløs, A.: Acta pædiat. 40:438, 1951. 17. Jiménez Díaz, C.: Lecciones de Patología Medica , Vol. 6, Madrid, Ed. Científico-Médica, 1948. 18. Jiménez Díaz, C.: Rev. clín. españ. 39:92, 1950. 19. Stowers, J. M., and Dent, C. E.: Quart. J. Med. 16:275, 1947. 20. Cooke, W. T.; Barclay, J. A.; Govan, A. D. T., and Nagley, L.: Arch. Int. Med. 80:147, 1947. 21. Luthy, F.: Deutsche Ztschr. Nervenh. 123: 101, 1931. 22. Jonxis, J. H. P., and Huisman, T. H. J.: Lancet 2:428, 1953. 23. Anderson, I. A.; Miller, A., and Kenny, A. P.: Quart. J. Med. 21:33, 1952. 24. Cumings, J. N.: Brain 71:410, 1948.Crossref 25. Matthews, W. B.; Milne, M. D., and Bell, M.: Quart. J. Med. 21:425, 1952. 26. Brick, I. B.: New England J. Med. 247:635, 1952.Crossref 27. Uzman, L.: Am. J. M. Sc. 226:645, 1953.Crossref 28. Eden, A., and Green, H. H.: Biochem. J. 34:1202, 1940. 29. Pope, G. G., and Stevens, M. F.: J. Biochem. 33:1070, 1939. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Archives of Internal Medicine American Medical Association

Contribution to Study of Wilson's Disease

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References (15)

Publisher
American Medical Association
Copyright
Copyright © 1955 American Medical Association. All Rights Reserved.
ISSN
0888-2479
DOI
10.1001/archinte.1955.00250110097012
Publisher site
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Abstract

Abstract We regard it of interest to report our study of three brothers affected with Wilson's disease, in two of whom the process appeared with its most characteristic clinical picture and in one of whom the only finding in relation to this disease was the presence of amino-aciduria. The nature of the hepatolenticular degeneration that appears in Wilson's disease is yet unknown, but in the last few years, by evaluation of the disturbances of the metabolism of copper and the amino acids eliminated in the urine, new concepts have been brought forth which are worthy of further research. Baker,1 in 1948, summarized the theories which up to that time had tried to explain the nature of Wilson's disease as follows: 1. It is a degenerative process affecting the liver and brain. 2. It is an acquired disease, and both organs are damaged simultaneously by an endogenous toxin. 3. The primary References 1. References 16 and 17. 2. Baker, A. B.: Arch. Path. 46:268, 1948. 3. Lipmann, F.: Biochem. Ztschr. 268:314, 1934. 4. Denny-Brown, D., and Porter, H.: New England J. Med. 245:917, 1951.Crossref 5. Uzman, L., and Denny-Brown, D.: Am. J. M. Sc. 215:599, 1948.Crossref 6. Cooper, A. M.; Eckart, R. D.; Faloon, W. W., and Davidson, C. S.: J. Clin. Invest. 29:265, 1950.Crossref 7. Uzman, L. L., and Hood, B.: Am. J. M. Sc. 223:392, 1952.Crossref 8. Dent, C. E., and Harris, H.: Ann. Eugenics 16:60, 1951.Crossref 9. De Verdier, C. H.: Acta med. scandinav. 138:344, 1950.Crossref 10. Hood, B., and Fagerberg, S. E.: Acta med. scandinav. 140:374, 1951.Crossref 11. Himsworth, H. P.: Lectures on the Liver and Its Diseases , Ed. 2, Cambridge, Mass., Harvard University Press, 1950. 12. Dent, C. E.: Clin. Proc. 8:21, 1949. 13. Clay, R. D.; Darmady, E. A., and Hawkins, M.: J. Path. & Bact. 65:551, 1953. 14. Bickel, H.: Lancet 2:403, 1953. 15. Hottinger, A.: Ann. pædiat. 169:272, 1947. 16. Drabløs, A.: Acta pædiat. 40:438, 1951. 17. Jiménez Díaz, C.: Lecciones de Patología Medica , Vol. 6, Madrid, Ed. Científico-Médica, 1948. 18. Jiménez Díaz, C.: Rev. clín. españ. 39:92, 1950. 19. Stowers, J. M., and Dent, C. E.: Quart. J. Med. 16:275, 1947. 20. Cooke, W. T.; Barclay, J. A.; Govan, A. D. T., and Nagley, L.: Arch. Int. Med. 80:147, 1947. 21. Luthy, F.: Deutsche Ztschr. Nervenh. 123: 101, 1931. 22. Jonxis, J. H. P., and Huisman, T. H. J.: Lancet 2:428, 1953. 23. Anderson, I. A.; Miller, A., and Kenny, A. P.: Quart. J. Med. 21:33, 1952. 24. Cumings, J. N.: Brain 71:410, 1948.Crossref 25. Matthews, W. B.; Milne, M. D., and Bell, M.: Quart. J. Med. 21:425, 1952. 26. Brick, I. B.: New England J. Med. 247:635, 1952.Crossref 27. Uzman, L.: Am. J. M. Sc. 226:645, 1953.Crossref 28. Eden, A., and Green, H. H.: Biochem. J. 34:1202, 1940. 29. Pope, G. G., and Stevens, M. F.: J. Biochem. 33:1070, 1939.

Journal

A.M.A. Archives of Internal Medicine – American Medical Association

Published: May 1, 1955

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