True about hemochromatosis is -
(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.
Iron deficiency anemia is caused
Kayser Fleischer ring is seen in eyes
DM and slate grey discolouration of skin
Autosomal dominant is the most common form