All are seen in hemochromatosis except?
Answer is D (Desferrioxamine is the treatment of choice): The treatment of choice for Hematochromatosis is removed of excess body iron by Phlebotomy and not with the use of chelating agents like desfernoxamine. Removal of Excessive Body iron Phlebotomy is the treatment of choice Iron removal is best accomplished by once or twice weekly phlebotomy of 500m1, - These should be continued until the serum ferritin level is < 50pg/L (May be required for 1-2 years) - Thereafter phlebotomies are performed at appropriate intervals to maintain ferritin levels between 50-100 tg/L (usually one phlebotomy every 3 months) Chelating agents (such as Desferoxamine) are alternative agents and indicated when anemia and hypoproteinemia are severe enough to preclude phlebotomy Treatment of Haematochromatosis Phlebotomy is the treatment of choice in Hematochromatosis Therapy of Haematochromatosis involves removal of excessive body iron and suppoive treatment of damaged organs Alcohol consumption should he eleminatedQ as increases ruck hematochronhth,sLs ten /oh/ Ahropathv, Hypogonadism, Diabetes and Bronzing (pigmentation) of skin are all characteristic manifestation of hematochromatosis Note: The combination of skin hyperpigmentation and insulin deficiency (diabetes) is called Bronze diabetes. The characterstic clinical features of Haemoehromatosis in order frequency areQ : Hepatomegaly (95%1 Skin pigmentation (90%) Diabetes mellitus (65%) Ahropathy (25-50%) Cardiac disease (15%) Hypogonadism
Hypogonadism
Ahropathy
Bronze diabetes
Desferrioxamine is the treatment of choice