[Idiopathic hemochromatosis, analysis of 19 cases]

We studied 19 patients with idiopathic haemochromatosis between 12,000 patients by the Department of Internal Medicine. By 17 the diagnosis was made very late with severe state of chronic liver disease, only two went to consult because transaminases were increased without exploration in routinary examination. Hepatomegalie was constant exploratory feature (100%) followed by impaired hydrocarbonated metabolism (52%). The biochemical usual pattern was: hipersideremie, decreased transferrin and increased transaminases activities in all the patients. Increased iron urinary excretion (more than 8 g/24 h) with desferroxiamine administration is a useful biochemical feature if because severe liver disease the biopsy punction was not indicated due to impaired hemostasia. Because the serious illness with late diagnosis and the normal survival if the diagnosis is early the iron metabolism would be useful screening as in another countries.