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? 發問於 健康疾病及病徵其他 - 疾病 · 1 十年前

Protein C deficiency

想問 ( Protein C deficiency ) 是什麼疾病,會引起什麼併發症,可不可以用肝臟移植的方法去徹底根治這疾病?

更新:

聽說香港有病人有這問題,用外科手術 ( Liver Translant ) 去除這問題及不需終生食藥?

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    什麼疾病:

    Protein C deficiency is a rare genetic trait that predisposes to thrombotic disease. It was first described in 1981. The disease belongs to a group of genetic disorders know as thrombophilias.

    The prevalence of protein C deficiency has been estimated to about 0,2% of the general population.

    Protein C deficiency is associated with an increased incidence of venous thromboembolism (relative risk 7), whereas no association with arterial thrombotic disease has been found.

    The main function of protein C is its anticoagulant property as an inhibitor of coagulation factors V and VIII. There are two main types of protein C mutations that lead to protein C deficiency:

    Type I: Quantitative defects of protein C (low production or short protein halflife)

    Type II: Qualitative defects, in which interaction with other molecules is abnormal. Defects in interaction with thrombomodulin, phospholipids, factors V/VIII and others have been described.

    Homozygous protein C mutations often causes a severe thrombotic disorder known as purpura fulminans.proteinc defiency has only had 16 caes before 1999

    Treatment治療:

    Primary phrophylaxis with aspirin, heparin or warfarin should be considered in known familial cases.

    Anticoagulant prophylaxis is given to all who develop a venous clot regardless of underlying cause. Studies have demonstrated an increased risk of recurrent venours thromboembolic events in patients with protein C deficiency. Therefore, long-term anticoagulation therapy with warfarin should be considered in these patients.

    Homozygous protein C defect constitutes a potentially life-threatening disease, and warrants the use of supplemental protein C concentrates.

    ==>根據佢講係無得徹底根治的,要終身食藥..

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