HEMOCHROMATOSIS

excessive accumulation of body iron, mostly in liver & pancreas 

Types

 - primary
 - secondary (hemosiderosis)
 * repeated blood transfusions
 * increased intestinal abs
orption of iron

Primary (hereditary) hemochromatosis

·        Autosomal recessive, due to excessive       intestinal absorption of iron

·        Diagnosed at 5^th decade of life

·        Males predominate by 5:1 to 7:1

·        Fully developed cases exhibit

 - Micronodular cirrhosis>hepatocellular carcinoma

 - Diabetes mellitus

 - Skin pigmentation

Pathogenesis

 - excessive iron is toxic to tissues through:

·        Lipid peroxidation via free radicals.           

·        Stimulation of collagen via activation of stellate cells.

·        Interaction of reactive oxygen species & iron with DNA>lethal cell injury or hepatocellular carcinoma.