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Thoughts and progress.

机译:思想与进步。

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Hereditary spherocytosis (HS) is a genetic, frequently familial hemolytic blood disease that presents with varying degrees of hemolytic anemia, splenomegaly, and jaundice.The disease arises as a result of defects in any of a number of proteins responsible for maintaining the shape and flexibility of the red blood cell, resulting in an osmotically fragile and characteristically spherical red blood cell. Theoretically, cardiopulmonary bypass can exacerbate hemolysis and subsequent renal dysfunction.There are few reports of open heart surgery for patients with HS and none for orthotopic heart transplantation.We report a 6-year-old boy with HS who underwent orthotopic heart transplantation.
机译:遗传性血球增多症(HS)是遗传性的家族性溶血性血液病,表现为不同程度的溶血性贫血,脾肿大和黄疸,该疾病是由于负责维持形状和柔韧性的多种蛋白质中的任何一种缺陷引起的红细胞,导致渗透性脆弱和典型的球形红细胞。从理论上讲,体外循环可以加重溶血和随后的肾功能不全.HS的开胸手术报道很少,而原位心脏移植则没有报道。我们报道了一个6岁的HS的男孩接受了原位心脏移植。

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