Hemochromatosis and Diabetes

  • Bruno W. Volk
  • Klaus F. Wellmann


Hemochromatosis is a rare disorder of metabolism characterized by the deposition throughout the body of abnormally large amounts of hemosiderin and, to a lesser degree, of hemofuscin, particularly in the liver, pancreas, and skin. The three cardinal signs of the disease are hepatomegaly with cirrhosis, diabetes mellitus, and abnormal pigmentation of the skin. In 1865, Trousseau1 described the first case, although no name was coined by him for this disease. The second case was reported in 1871 by Troisier,2 who called the disease “le cirrhose pigmentaire dans diabète sucré.” Hanot and Chauffard,3 11 years later, believed that the diabetes associated with hemochromatosis was probably the primary disease and caused cirrhosis as well as pigmentation of the skin. They called the disease “cirrhose hypertrophique pigmentaire dans le diabète sucré.” Other authors4–7 similarly considered the hyperglycemia as the primary cause of the disorder and thought that the disturbance of blood sugar homeostasis resulted from the changes in the liver and the other organs.


Iron Overload Iron Metabolism Cardinal Sign Portal Cirrhosis Abnormal Pigmentation 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Trousseau, A.: Clinique Mèd. d’Hótel-Dieu de Paris, 2nd Ed., Vol. 2, 1865, p. 663.Google Scholar
  2. 2.
    Troisier, M.:Bull. Soc. Anal, 16:231, 1871.Google Scholar
  3. 3.
    Hanot, V., and Chauffard, A.: Rev. Med., 2:385, 1882.Google Scholar
  4. 4.
    Letulle, M.: Bull Mem. Soc. Med. Hop. Paris, 2:406, 1885.Google Scholar
  5. 5.
    Hanot, V.:Br. Med. J., 1:206, 1896.PubMedGoogle Scholar
  6. 6.
    Hanot, V., and Schachmann, M.: Arch. Physiol. Norm. Pathol, 7: 1886.Google Scholar
  7. Von Recklinghausen, F.: Naturforscher und Ärzte in Heidelberg, 1889, p. 324.Google Scholar
  8. Calmettes, J.: Thèse de Paris, 1896.Google Scholar
  9. 9.
    Berg, H. W.: Med Ree., 56:881, 1899.Google Scholar
  10. 10.
    Labbè, M.: Arch. Appar. Dig. Nutr., 6:403, 1912.Google Scholar
  11. 11.
    McCreery, A. H.: Can. Med Assoc. J., 7:481,1917.PubMedGoogle Scholar
  12. 12.
    Zaleski, S.: Virchows Arch., 104:91, 1886.Google Scholar
  13. 13.
    Kretz, R.: Beitr. Klin. Med Chir., Heft 15, 1896.Google Scholar
  14. 14.
    Sheldon, J. H.: Haemochromatosis. Oxford University Press, London, 1935.Google Scholar
  15. 15.
    Marble, A. and Bailey, C. C: Am. J. Med, 11:590, 1951.PubMedGoogle Scholar
  16. 16.
    Marble, A., and Steinke, J.: Medizinische, 1:19, 1959.PubMedGoogle Scholar
  17. 17.
    Bell, E. T.: Diabetes, 4:435, 1995.Google Scholar
  18. 18.
    Dubin, L. N.: Am. J. Clin. Pathol, 25:514, 1955.PubMedGoogle Scholar
  19. 19.
    Finch, S. C., and Finch, C. A.: Medicine (Baltimore), 34:381, 1955.Google Scholar
  20. 20.
    MacDonald, R. A., and Mallory, G. K.: Arch. Intern. Med, 105:686, 1960.PubMedGoogle Scholar
  21. 21.
    Bothwell, T. H., and Finch, C. A.: Iron Metabolism. Little, Brown, Boston, 1962.Google Scholar
  22. 22.
    Beutler, E., Fairbanks, V. F., and Fahey, J. L: Clinical Disorders of Iron Metabolism. Grune & Stratton, New York, 1963.Google Scholar
  23. 23.
    Bork, K.: Virchows Arch., 269:178, 1928.Google Scholar
  24. 24.
    Althausen, T. L., Doig, R. K., Weiden, S., Motteram, R., Turner, C. N., and Moore, A.: Arch. Intern. Med, 88:553, 1951.Google Scholar
  25. 25.
    Heilmeyer, L.:Acta Haematol, 11:137, 1954.PubMedGoogle Scholar
  26. 26.
    Stachewitsch, A.: Cited by Creutzfeldt, W.: Acta Hepato-Splenol, 5:156, 1959.Google Scholar
  27. 27.
    Creutzfeldt, W.: Acta Hepato-Splenol, 6:156, 1959.Google Scholar
  28. 28.
    Bothwell, T. H., and Finch, C. A.: Iron Metabolism. Little, Brown, Boston, 1962, p. 366.Google Scholar
  29. 29.
    Strohmeyer, G., Gottesbüren, H., Behr, C., and Sauer, H.: Dtsch. Med. Wochenschr., 101:1055,1976PubMedGoogle Scholar
  30. 30.
    Dymock, L W., Cassar, J., Pyke, D. A., Oarley, W. G., and Williams, R.: Am. J. Med, 52:203, 1972.PubMedGoogle Scholar
  31. 31.
    Wöhler, F.: Verh. Dtsch. Ges. Inn. Med, 70:300, 1964.Google Scholar
  32. 32.
    Becker, D., and Miller, M.: N. Engl J. Med, 263:367, 1960.PubMedGoogle Scholar
  33. 33.
    Milder, M. S., Cook, J. D., Stray, S., and Finch, C. A.: Medicine (Baltimore), 59:34, 1980.Google Scholar
  34. 34.
    Balcerzak, S. P., Mintz, D. H., and Westerman, M. P.: Am. J. Med Sei., 155:53, 1968.Google Scholar
  35. 35.
    Saddi, R., Hamon, B., Feingold, J., Eschwege, E., and Fagard, R.: in: Journées Annuelles de Diabetologie. Edited by M. Raterty. Flammarion, Paris, 1978.Google Scholar
  36. 35a.
    Stocks, A. E., and Powell, L. W.: Q. J. Med, 42:733, 1973.PubMedGoogle Scholar
  37. 35b.
    Saddi, R., and Feingold, J.: Clin. Genet., 5:242, 1974.PubMedGoogle Scholar
  38. 36.
    Bourel, M., Simon, M., deNercy, Y. H., Brissot, P., and Hespel, J.-P.: Hemochromatoses, 27:7,1977Google Scholar
  39. 37.
    Root, H. F.: N. Engl J. Med, 201:201, 1929.Google Scholar
  40. 38.
    Engel, R.: Klin. Wochenschr., 13:1682, 1934.Google Scholar
  41. 39.
    Kissel, P., Debry, G., Marchal, C., and Glabeke, B.: Rev. Fr. Endocrinol Clin. Nutr. Metab., 5:309, 1964.Google Scholar
  42. 40.
    Buchanan, J., and Young, E. T.: Postgrad. Med. J., 42:551, 1966.PubMedGoogle Scholar
  43. 41.
    Megyesi, C, Samols, E., and Marks, R.: Lancet, 2:1051, 1967.PubMedGoogle Scholar
  44. 42.
    Collins, J. R., and Crofford, O. B.: Arch. Intern. Med, 124:142, 1969.PubMedGoogle Scholar
  45. 43.
    Saaman, L. N. A., Stone, D. B., and Eckhardt, R. D.: Arch. Intern Med, 124:149, 1969.Google Scholar
  46. 44.
    Walsh, C. H., Malins, J. M., and Bloom, S. R.: Br. Med J., 2:1267, 1978.PubMedGoogle Scholar
  47. 45.
    Gonvers, J.-J., Henchoz, L., Hofstetter, J.-R., and Müller, W. A.: Schweiz. Med. Wochenschr., 107:1841, 1977.PubMedGoogle Scholar
  48. 46.
    Nelson, R. L., Baldi, W. P., Rubenstein, A. H., Go, V. L. W., and Service, F. J.: J. Clin. Endocrinol Metab., 49:412, 1979.PubMedGoogle Scholar
  49. 47.
    Passa, P., Rousseie, F., Gauville, C., and Canivet, J.: Diabetes, 26:113, 1977.PubMedGoogle Scholar
  50. 48.
    Oakley, Y. W., Pyke, D. A., and Taylor, K. W.: Clinical Diabetes and Its Biochemical Basis. Blackwell, Oxford, 1968, pp 383, 675.Google Scholar
  51. 49.
    Lawrence, R. D.: Lancet, 2:401, 1949.PubMedGoogle Scholar
  52. 50.
    Griffiths, J. D., Dymock, I. W., Davies, E. W. G., Hill, D. W., and Williams, R.: Diabetes, 20:766, 1971.PubMedGoogle Scholar
  53. 51.
    Simon, M., Vongsavanthong, J. P., Roussey, M., and Bourel, M.: Sem. Hop., 49:2133, 1973.PubMedGoogle Scholar
  54. 52.
    John, H. J.: J. Am. Med. Assoc., 112:2272, 1939.Google Scholar
  55. 53.
    Butt, H. R., and Wilder, R. M.: Arch. Pathol., 26:262, 1938.Google Scholar
  56. 54.
    Jensen, W. K.: In: Joslin’s Diabetes Mellitus. Edited by A. Marble, P. White, R. F. Bradley, and L. P. Krall. Lea & Febiger, Philadelphia, 1971, p. 712.Google Scholar
  57. 55.
    Boulin, R.:Presse Méd., 53:326, 1945.Google Scholar
  58. 56.
    Warren, S., LeCompte, P. M., and Legg, M. A.: The Pathology of Diabetes Mellitus. Lea & Febiger, Philadelphia, 1966, p. 365.Google Scholar
  59. 57.
    Potter, N. B., and Milne, L. S.: Am. J. Med. Sei., 143:46, 1911.Google Scholar
  60. 58.
    Labbè, M., and Stevenin, H.: Presse Med., 30:424, 1922.Google Scholar
  61. 59.
    Rosenthal, S. R.: Arch. Pathol, 13:83, 1932.Google Scholar
  62. 60.
    Elmer, W. P.: Intern. Med J. (St. Louis), 18:912, 1911.Google Scholar
  63. 61.
    Hensel, O.:Med J. Ree., 127:14, 1928.Google Scholar
  64. 62.
    Sprunt, T. P.: Arch. Intern. Med, 8:75, 1911.Google Scholar
  65. 63.
    Blumer, G.: N. Y. Med J., 94:922, 1911.Google Scholar
  66. 64.
    Hess, O., and Zurhelle, E.: Z. Klin. Med, 57:344, 1905.Google Scholar
  67. 65.
    Cecil, R. L.: J. Exp. Med., 14:500, 1911.PubMedGoogle Scholar
  68. 66.
    Barber, H.:Guy’s Hosp. Rep., 79:45, 1929.Google Scholar
  69. 67.
    Barber, H., and Bith, H.: Bull. Mem. Soc. Med. Hop. Paris, 33:119, 1912.Google Scholar
  70. 68.
    Manning, D. F.: J. Mo. State Med. Assoc., 22:390, 1925.Google Scholar
  71. Ollivier, J.: Thèse de Paris, 1925, p. 47.Google Scholar
  72. 70.
    Seibert, P.: Beitr. Pathol. Anat. Allg. Pathol., 84:111, 1930.Google Scholar
  73. 71.
    Hartroft, W. S.: Diabetes, 5:98, 1956.PubMedGoogle Scholar
  74. 72.
    McGavran, M. H., and Hartroft, W. S.: Am. J. Pathol., 32:631, 1956.Google Scholar
  75. 72a.
    Warson, F., and Gepts, W.: Unpublished observation in: Gepts, W.: In: The Islets of Langerhans. Edited by S. J. Cooperstein and D. Watkins. Academic Press, New York, 1981, p. 321.Google Scholar
  76. 73.
    Warren, S., LeCompte, P. M., and Legg, M. A.: The Pathology of Diabetes Mellitus. Lea & Febiger, Philadelphia, 1966, p. 367.Google Scholar
  77. 74.
    Mallory, F. B.: Am. J. Pathol, 1:117, 1925.PubMedGoogle Scholar
  78. 75.
    Herbut, P. A., and Tamaka, H. T.: Am. J. Clin. Pathol, 16:640, 1946.PubMedGoogle Scholar
  79. 76.
    MacDonald, R. A.: Arch. Intern. Med, 107:606, 1961.PubMedGoogle Scholar
  80. 77.
    Brown, E. B., Jr., Dubach, R., Smith, D. E., Reynafaije, C., and Moore, C. V.: J. Lab. Clin. Med, 50:862, 1957.PubMedGoogle Scholar
  81. 78.
    Brown, E. B., Jr., Dubach, R., and Moore, C. V.:J. Lab Clin. Med, 53:591, 1959.PubMedGoogle Scholar
  82. 79.
    Abbott, M. E.: J. Pathol Bacteriol, 7:55, 1901.Google Scholar
  83. 80.
    Donaldson, R.:Guy’s Hosp. Rep., 79:28, 1929.Google Scholar
  84. 81.
    Warren, S., LeCompte, P. M., and Legg, M. A.: The Pathology of Diabetes Mellitus. Lea & Febiger, Philadelphia, 1966, p. 370.Google Scholar
  85. 82.
    Warren, S., and Drake, W. L., Jr.: Am. J. Pathol, 27:573, 1951.PubMedGoogle Scholar
  86. 83.
    Steinke, J.: Quoted in: Warren, S., LeCompte, P. M., and Legg, M. A.: The Pathology of Diabetes Mellitus. Lea & Febiger, Philadelphia, 1966, p. 371.Google Scholar
  87. 84.
    Rogers, W. F., Jr.: Am. J. Med Sei., 220:530, 1950.Google Scholar
  88. 85.
    Gitlow, S. E., and Beyers, M. R.: J. Lab. Clin. Med, 39:337, 1952.PubMedGoogle Scholar
  89. 86.
    Rössle, R.: In: Handb. Spez. Pathol Anat. Histol Edited by F. Henke and O. Lubarsch. Springer-Verlag, Berlin, 1930, p. 243.Google Scholar
  90. 87.
    Granick, S.:Bull N. Y. Acad. Med, 25:403, 1949.PubMedGoogle Scholar
  91. 88.
    Drabkin, D. L: Physiol Rev., 31:345, 1951.PubMedGoogle Scholar
  92. 89.
    Eppinger, H.:Die Leberkrankheiten. Springer-Verlag, Berlin, 1937, p. 423.Google Scholar
  93. 90.
    Schmidt, M. B.: Ergeb. Allg Pathol Pathol Anat., 35:105, 1940.Google Scholar
  94. 91.
    Vannotti, A., and Delachaux, A.: Metabolism and Its Clinical Significance. Grune & Stratton, New York, 1949, p. 15.Google Scholar
  95. 92.
    Regelsberger, H.:Klin. Wochenschr., 21:1122, 1942.Google Scholar
  96. 93.
    Brick, I. B.: Arch. Intern. Med, 96:26, 1955.Google Scholar
  97. 94.
    Crosby, W. H.: In: Controversy in Internal Mediane. Edited by F. J. Ingelfinger, A. S. Relman, and M. Finland. Saunders, Philadelphia, 1966, p. 261.Google Scholar
  98. 95.
    MacDonald, R. A.: Hemochromatosis and Hemosiderosis. Charles C. Thomas, Springfield,1964 p256Google Scholar
  99. 96.
    Gillman, J., and Gillman, T.: Arch. Pathol, 40:239, 1945.Google Scholar
  100. 97.
    Gillman, J., and Gillman, T.: Gastroenterology, 8:19, 1947.PubMedGoogle Scholar
  101. 98.
    Eddy, W. H., and Dalldorf, G.: The Avitaminosis. Williams & Wilkins, Baltimore, 1944, p. 129.Google Scholar
  102. 99.
    Spies, T. D.: In: Clinical Nutrition. Edited by N. JollifFee, F. F. Tisdall, and P. R. Cannon. Hoeber, New York, 1950, p. 531.Google Scholar
  103. 100.
    Bothwell, T. H., Abrahams, C., Bradlow, B. A., and Charlton, R. W.: Arch. Pathol., 79:163,1965PubMedGoogle Scholar
  104. 101.
    MacDonald, R. A., and Pechet, G. S.: Am. J. Pathol, 46:85, 1965.PubMedGoogle Scholar
  105. 102.
    Even, R.: Etude Clinique et Experimentale. Le Francois, Paris, 1932, p. 133.Google Scholar
  106. 103.
    Lazarus, S. S., and Volk, B. W.: Arch. Pathol, 71:44, 1961.PubMedGoogle Scholar

Copyright information

© Plenum Publishing Corporation 1985

Authors and Affiliations

  • Bruno W. Volk
    • 1
  • Klaus F. Wellmann
    • 2
  1. 1.Department of PathologyUniversity of California Irvine Medical CenterOrangeUSA
  2. 2.Department of PathologyState University of New York, Downstate Medical CenterBrooklynUSA

Personalised recommendations