Hereditary Hemochromatosis : Case Report and Review Literatures
Keywords:
Hemochromatosis, HLA B7, Desferioxamine, Methanesultonate, Blood lettingAbstract
Abstract: Heredtary hemochromatosis is a common inhented disease in Europe but uncommon in Asia. This report showed two cases of heredtary hemochromatosis from one Thai family. The first case was 43-year-old with drabes. He presented with the symptoms of impotence and dark skin. He occasionally consumed alcoholic beverage and never received blood transfusion. His abnormal physical fings were bronze-colored skin, hairiess and small testes. The helpful investigations included : transterrin sation was 91 %, liver tissue with Prussian-blue staing showed inoreased iron content. He was diagnosed to be hereditary hemochromatosis and his HLA typing was HLA B7/. After screening family members, it was found that his younger brotther had the symptom of impotence along with transferrin saturalion of 94 % and highty iron-deposited Iver tissue. The patient was initially treated intermittent blood letting. Later, had daveloped anemia, so he was treated with iron-chelating agent (desferioxamine methanesulfonate) instead. Afteatment, his glycemic control came to acceptable level with lower dose of oral hypoglycemic agent.
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