【丙酮酸激酶缺乏症诊断与治疗的专家共识】。

Q3 Medicine
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引用次数: 0

摘要

丙酮酸激酶缺乏症(Pyruvate kinase deficiency, PKD)是一种罕见的常染色体隐性遗传病,由编码红细胞丙酮酸激酶的pkr基因突变引起,该基因影响红细胞能量产生,进而影响红细胞功能和寿命。PKD以慢性溶血性贫血为特征,其他特征包括慢性溶血性并发症,如铁超载、骨密度降低和心肺并发症。PKD的治疗需要根据患者的病情进行个体化治疗,包括红细胞输注、丙酮酸激酶激活剂和并发症的治疗。本共识围绕PKD的发病机制、临床特点、诊断和治疗等方面进行探讨,旨在为临床医生更好地提供PKD患者的诊断、治疗、监测、并发症预防等医疗服务。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Expert consensus on the diagnosis and treatment of pyruvate kinase deficiency].

Pyruvate kinase deficiency (PKD) is a rare autosomal recessive disorder caused by mutations in the PKLR gene, encoding erythrocyte pyruvate kinase, which affects erythrocyte energy production, and then in turn affects erythrocyte function and longevity. PKD is characterized by chronic hemolytic anemia, and other features include chronic hemolytic complications, such as iron overload, decreased bone mineral density, and cardiopulmonary complications. The treatment of PKD requires individualized approach based on the patient's condition, including red blood cell transfusions, pyruvate kinase activators, and treatment for complications. This consensus focuses on the pathogenesis, clinical characteristics, diagnosis and treatment of PKD, and aims to provide better medical service for clinicians, such as diagnosis, treatment, monitoring, and prevention of complications for PKD patients.

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来源期刊
Zhonghua yi xue za zhi
Zhonghua yi xue za zhi Medicine-Medicine (all)
CiteScore
0.80
自引率
0.00%
发文量
400
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