【遗传性血管性水肿诊断三十年后临床表现】。

Q3 Medicine Revista alergia Mexico Pub Date : 2021-07-01 DOI:10.29262/ram.v68i3.874
Luiz Fernando Bacarini, Ana Luisa Vieira, Mariana Camargo, Tainá Mosca, Wilma Carvalho Neves-Forte
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引用次数: 0

摘要

摘要:遗传性血管性水肿(遗传性血管性水肿,遗传性血管性水肿)的诊断和治疗对于提高患者的生活质量甚至生存是必要的。病例报告:52岁女性血管性水肿30年,影响面部、舌头和手部。它是不对称的,既没有瘙痒也没有荨麻疹,对抗组胺药或皮质类固醇没有反应,在48小时至72小时内自发消退;有血管性水肿家族史。两次发作之间的正常体格检查。排除了自身免疫性和淋巴细胞增生性疾病。C1q、C4、C1-INH值正常。确定HAE C1-INH正常亚型Unknown的诊断。经过两个月的雄激素治疗,危机得到了彻底解决。门诊随访4年,没有血管性水肿危象的报道,这与生活质量的根本改变有关。结论:在排除后天性血管性水肿后,患者在30年的临床表现后被诊断为HAE。
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[Diagnosis of hereditary angioedema after thirty years of clinical manifestations].

Introduction: Diagnosis and treatment of hereditary angioedema (HAE) are necessary to improve the quality of life and even the survival of patients.

Case report: A 52-year-old woman with angioedema for 30 years, which affects the face, tongue, and hands. It is asymmetric, with neither pruritus nor urticaria, without response to antihistamines or corticosteroids, with spontaneous resolution in 48 hours to 72 hours; with a family history of angioedema. Normal physical examination between exacerbations. Autoimmune and lymphoproliferative diseases were ruled out. Values of C1q, C4, C1-INH were normal. The diagnosis of HAE type C1-INH normal subtype Unknown was established. The total resolution of the crises was achieved after two months with androgen therapy. Outpatient follow-up has been given for four years and no angioedema crisis has been reported, which is associated with a radical change in the quality of life.

Conclusion: The patient was diagnosed with HAE after 30 years of clinical manifestations, after acquired angioedema was ruled out.

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来源期刊
Revista alergia Mexico
Revista alergia Mexico Medicine-Immunology and Allergy
CiteScore
0.70
自引率
0.00%
发文量
9
审稿时长
16 weeks
期刊最新文献
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