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遗传性血管性水肿:文献综述与国家管理指南

HEREDITARY ANGIOEDEMA: A Literature Review and National Management Guidelines.

作者信息

Azzam Ramez, Boutros Jacques, Irani Carla

出版信息

J Med Liban. 2015 Apr-Jun;63(2):97-101. doi: 10.12816/0012558.

Abstract

BACKGROUND

Hereditary angioedema, a rare and potentially life-threatening condition, is the result of a defect in the C1 esterase inhibitor. Primary care physicians should be familiar with this condition to avoid complications and improve quality of care.

METHODS

We present two cases of hereditary angioedema followed by a discussion based on a literature review of the recent guidelines and advances in this condition.

OBJECTIVES

To highlight the clinical aspects, diagnosis and treatment of this condition and propose a practical local management based on the available medication.

CONCLUSION

Hereditary angioedema management is still evolving. More efforts should be made concerning the drug therapy which is very costly and not available worldwide.

摘要

背景

遗传性血管性水肿是一种罕见且可能危及生命的疾病,是C1酯酶抑制剂缺陷的结果。初级保健医生应熟悉这种疾病,以避免并发症并提高护理质量。

方法

我们介绍了两例遗传性血管性水肿病例,随后基于对该疾病近期指南和进展的文献综述进行了讨论。

目的

强调这种疾病的临床特征、诊断和治疗,并根据现有药物提出切实可行的本地管理方法。

结论

遗传性血管性水肿的管理仍在不断发展。对于成本高昂且并非在全球范围内都可获得的药物治疗,应做出更多努力。

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