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Analysis of Clinical Presentations of Bruton Disease: A Review of 20 Years of Accumulated Data from Pediatric Patients at Severance Hospital

机译:布鲁顿氏病的临床表现分析:遣散医院小儿患者20年累积数据回顾

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摘要

PurposeX-linked agammaglobulinemia (XLA) is a humoral immunodeficiency disease caused by a mutation in the Bruton tyrosine kinase (BTK) gene resulting in defective B cell differentiation. Because it is a relatively rare disorder, it is difficult for clinicians to have a comprehensive understanding of XLA due to a lack of exposure to the disease. Clinical presentations of patients with XLA were analyzed and discussed to improve care plans.
机译:目的X连锁的丙种球蛋白血症(XLA)是一种体液免疫缺陷病,由Bruton酪氨酸激酶(BTK)基因突变导致缺陷的B细胞分化引起。由于它是一种相对罕见的疾病,由于缺乏该疾病的暴露,临床医生很难全面了解XLA。分析并讨论了XLA患者的临床表现,以改善护理计划。

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