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Showing posts with label hemochromatosis. Show all posts
Showing posts with label hemochromatosis. Show all posts

What are the cardinal manifestations of hemochromatosis?

What is haemochromatosis ?
It is an iron storage disease that is characterised by increased Intestinal iron absorption.There is deposition of iron in parenchymal cels with tissue damage -The disease is clinically manifested as the following
  • Excessive skin pigmentation (due to increased melanin and iron .Melanin is responsible for bronzing of skin
  • Diabetes mellitus (bronze diabetes)
  • Arthropathy
  • Cardiac involvement- Congestive cardia faiure or cardiomyopathy
  • Hepatic involvement - Cirrhosis or hepatocellular carcinoma with hepatocellular failure
  • Loss of libido and testicular atrophy.
What are the cause of death in Hemochromatosis
  • CCF
  • Hepatocellular failure
  • Hepatocellular carcinoma
What is the treatment of hemochromatosis
  • Phlebotomy.
  • Iron chelation by desferrioxamme.
  • Treatment of CCF. hepato-cellular failure .diabetes mellilus etc.