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What is In hemochromatosis, all of the following are affected except and explain in detail?
Hemochromatosis is a iron storage disorder with genetic heterogenicity resulting in low production of hormone -HEPCIDIN-resulting in increased iron absorption and deposition in various organs. Major clinical manifestations are-cirrhosis of liver, diabetes mellitus, ahritis ,cardiomyopathy ,hypogonadotropic hypogonadism. Symptoms usually develop between 40 and 60 SERUM ferritin is a good index of iron stores. Treatment-weekly or twice weekly phlebotomy of 500ml is indicated to maintain serum ferritin less than 100mcg/l. In cases like anemia and hypoprotienemia-deferoxamine is given parenterally(s.c) as phlebotomy is not indicated Oral chelating agent-deferasirox is effective in thalassemia and secondary iron overload Acquired iron overload -iron loading anemias(sideroblastic anemia, Thalassemia major, chronic hemolytic anemias) ,chronic liver disease. REF:HARRISON 20TH edition pg no. 2977,table-407-1,pg no. 2981