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Troisier-Hanot-Chauffard syndrome

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Diabetes mellitus associated with hypertrophic cirrhosis of the liver and dark brownish skin pigmentation caused by deposition of excess of melanin or iron pigment, or both, in tissues. There is lassitude, weakness, weight loss, upper right abdominal quadrant sharp pain, dyspnoea, loss of libido. Occasionally, specific progressive polyarthropathy.

Greatest frequency in men between 40 and 60 years of age. The hereditary type is transmitted as an autosomal recessive trait.

The condition was first described by Troisier in 1871, in 1882 by Hanot and Chauffard. The term haemochromatosis was introduced by von Recklinghausen in 1889.

Leschke's syndrome, or congenital pigmentary dystrophy, is an early manifestation of haemochromatosis. See under Erich Leschke, German internist, 1887-1933.

We thank Patrick Jucker-Kupper, Switzerland, for information submitted.

Bibliography

  • C. E. Troisier:
    Diabète sucré.
    Bulletin de la Société anatomique de Paris, 1871, 16: 231.
  • V. C. Hanot, A. M. E. Chauffard:
    Cirrhose hypertrophique pigmentaire dans le diabète sucré.
    Revue de médecine, Paris, 1882, 2: 385-403.
  • F. D. Von Recklinghausen:
    Über Hämochromatose.
    Berliner klinische Wochenschrift, 1889, 26: 925.
    Tageblatt der Versammlungen deutscher Naturforscher und Aerzte, Heidelberg, 1889; 62: 324.
  • Leo Appelbaum:
    Über Hämochromatose. Medical thesis. Würzburg 1900.

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