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What is True about hemochromatosis is - and explain in detail?
(C) (DM and slate gray discoloration of the skin) (42, 627 Harsh Mohan 6th) (629 Robbins Basic pathology 9th)HemochromatosisTRIAD:1) Pigmentary liver cirrhosis.2) Diabetes mellitus (Pancreatic damage)3) Skin Pigmentation]BRONZE DIABETES* The most common form is an autosomal recessive disease of adult onset caused by a mutation in the HFE gene.A) Idiopathic or hereditary hemochromatosis:* There is a defect in the regulation of intestinal absorption of dietary iron, leading to net iron accumulation of 0.5 to 1.0 g/year.* Hepcidin, produced by the liver, form a web of iron, metabolic control networks that are only recently become elucidated.* Deposits sites - liver, pancreas, spleen, heart and endocrine glands *** Manifests typically after 20 gm of storage iron has accumulated.B) Secondary or acquired hemochromatosis:* Multiple blood transfusion.* Ineffective erythropoiesis (as in G thalassemia and myelodysplastic syndrome).* Increased iron intake.The occurrence of hepatocellular carcinoma is a late complication of hemochromatosis - induced cirrhosis.* K.F. Ring (produced by the alloy of copper in the eye seen in Chalcosis - deposited in Descemet's membrane of the cornea.