Very rare syndrome combining diabetes mellitus and hemochromatosis.
Troisier-Hanot-Chauffard Syndrome; Leschke Syndrome; Recklinghausen Applebaum Syndrome; Troisier Syndrome.
Incidence and genetic inheritance
Greatest frequency in men between 40 and 60 years old. A medical condition transmitted as autosomal recessive trait. It was first described by Troisier in 1871 and subsequently expanded by Hanot and Chauffard in 1882.
Diabetes mellitus associated with hypertrophic cirrhosis of the liver and dark brownish skin pigmentation caused by deposition of excess of melanin, iron pigment, or both in tissues. Lassitude, weakness, weight loss, upper right abdominal quadrant sharp pain, dyspnea, and loss of libido occur. Occasionally, specific progressive polyarthropathy appears.
Assess diabetes and adapt intraoperative fluid regimen. The insulin management should be based on perioperative glucose level monitoring. Assess liver function (clinical, echography, CT, laboratory, including coagulation) and adapt anesthetic technique and choice of agents in consideration for the potential presence of hepatic toxicity.
AME: Cirrhose hypertrophique pigmentaire dans le diabète sucré. Rev Med (Paris) 2:385, 1882.
CE: Diabète sucré. Bull Soc Anat (Paris) 16:231, 1871.