Which one of the following inherited disorders produce thrombosis
Correct Answer: Homocysteinemia
Description: ref Robbins 7/e p 131, 8/e p122 , Harrison 18/e p462,9/e p123 Homocysteine (Hcy) is a sulfhydryl amino acid derived from the metabolic conversion of methionine, which is dependent on vitamins (folic acid, B12 and B6) as cofactors or cosubstrates. Severe hyperhomocysteinemia (homocystinuria), due to inherited metabolic defects of Hcy metabolism, is associated with very high risk of venous thromboembolism (VTE); treatment with vitamins is associated with dramatic decrease of VTE risk
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