遗传性血管性水肿:文献综述和国家管理指南。

Ramez Azzam, Jacques Boutros, Carla Irani
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引用次数: 0

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情况,以避免并发症和提高护理质量。方法:我们提出了两个遗传性血管性水肿的情况下,随后讨论了基于文献回顾最近的指导方针和进展在这种情况下。目的:强调本病的临床特点、诊断和治疗,并在现有药物的基础上提出切实可行的局部治疗方案。结论:遗传性血管性水肿的治疗仍在不断发展。应该在药物治疗方面做出更多努力,因为药物治疗非常昂贵,而且在世界范围内无法获得。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
HEREDITARY ANGIOEDEMA: A Literature Review and National Management Guidelines.

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.

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