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Effects and timing of tranexamic acid on transfusion requirements in patients undergoing cardiac surgery with cardiopulmonary bypass.

机译:氨甲环酸对接受心脏体外循环的心脏手术患者的输血需求的影响和时机。

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摘要

Aceruloplasminemia is a rare autosomal recessive disease that affects the iron metabolism of the body. When there is a lack of ceruloplasmin ferroxidase activity, iron accumulates, especially in the brain, pancreas, liver, and retina. The first symptom is generally a persistent hypochromic microcytic anemia with a mild high-serum ferritin level. The affected patients are usually recognized at later ages, when the neurological symptoms appear. The neurological outcome has an adverse effect on the prognosis, which may result in fatality. Therefore, early diagnosis and intervention may prevent a devastating neurological damage. Here, we report a case of aceruloplasminemia in a teenage girl with hypochromic microcytic anemia.
机译:铜蓝蛋白血症是一种罕见的常染色体隐性遗传疾病,会影响人体的铁代谢。当缺乏铜蓝蛋白铁氧化酶活性时,铁会积累,特别是在脑,胰腺,肝脏和视网膜中。第一个症状通常是持续的低色素性小细胞性贫血,伴有轻度的高血清铁蛋白水平。当出现神经系统症状时,通常会在较晚的年龄识别出受影响的患者。神经学预后对预后有不利影响,可能导致死亡。因此,早期诊断和干预可以防止破坏性的神经系统损害。在这里,我们报告了一名患有低色素性小细胞性贫血的少女中的铜蓝蛋白血症病例。

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