The prevalence of hereditary angioedema in a Chinese cohort with decreased complement 4 levels

Qi Cui, MD; Qingxiu Xu, MD; Yaqi Yang, MD, PhD; Wenjing Li, MD; Nan Huang, MD; Hao Chen, MD, PhD; Dongxia Ma, MD, PhD; Shuchen Zhang, MD; Lin Yang, MD, PhD; Rongfei Zhu, MD, PhD

World Allergy Organization Journal (Jan 2022)

The prevalence of hereditary angioedema in a Chinese cohort with decreased complement 4 levels

Qi Cui, MD,
Qingxiu Xu, MD,
Yaqi Yang, MD, PhD,
Wenjing Li, MD,
Nan Huang, MD,
Hao Chen, MD, PhD,
Dongxia Ma, MD, PhD,
Shuchen Zhang, MD,
Lin Yang, MD, PhD,
Rongfei Zhu, MD, PhD

Affiliations

Qi Cui, MD: Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
Qingxiu Xu, MD: Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
Yaqi Yang, MD, PhD: Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
Wenjing Li, MD: Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
Nan Huang, MD: Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
Hao Chen, MD, PhD: Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
Dongxia Ma, MD, PhD: Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
Shuchen Zhang, MD: Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
Lin Yang, MD, PhD: Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China
Rongfei Zhu, MD, PhD: Corresponding author. Department of Allergy, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, 430030, China.; Department of Allergy, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, China

Journal volume & issue: Vol. 15, no. 1
p. 100620

Abstract

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Objective: Hereditary angioedema (HAE) is a rare, life-threatening autosomal dominant disorder. We aimed to investigate the prevalence of HAE in a Chinese population with a decreased Complement 4 (C4) level. Methods: All the patients present in Tongji Hospital with C4 below lower normal range were included from January 2019 to June 2020. The individual data were extracted from the database and categorized by diagnosis. Patients suspected of HAE were further evaluated by C1 inhibitor level and function test to confirm the HAE diagnosis. Results: A total of 8226 patients were enrolled in our study, among whom 18 had symptoms similar to HAE and received C1 inhibitor level and function tests. Two (1 male and 1 female) of the 18 patients were identified as HAE patients. This means the prevalence of HAE was 2.43/10 000 among the C4-decreased population and 10.1/10 000 in the C4-decreased population with etiology undetermined. The 2 HAE patients had experienced skin and oropharynx edema attack and received tracheotomy. The female patient had a family history. Laboratory tests showed significant decrease of C4 and C1 inhibitor levels in the 2 patients, both of whom were diagnosed as type 1 HAE. Conclusion: The prevalence of HAE is low in C4-decreased patients. In a large cohort, C4 level can serve as a practical indicator to screen the HAE patients, but further testing of C1 inhibitor activity and levels is needed to confirm the diagnosis of HAE.

Published in World Allergy Organization Journal

ISSN: 1939-4551 (Online)
Publisher: Elsevier
Country of publisher: Netherlands
LCC subjects: Medicine: Internal medicine: Specialties of internal medicine: Immunologic diseases. Allergy
Website: https://www.sciencedirect.com/journal/world-allergy-organization-journal

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