Clinical Features of Hereditary Angioedema in Korean Patients

A Nationwide Multicenter Study

Jae Woo Jung, Dong In Suh, Hye Jung Park, Sujeong Kim, Hyouk Soo Kwon, Min Suk Yang, Chan Sun Park, Joo Hee Kim, Sae Hoon Kim, Yong Won Lee, Gyu Young Hur, Young Min Ye, Yong Eun Kwon, Hye Kyung Park, Cheol Woo Kim, Young Il Koh, Jungwon Park, Jong Myung Lee, Kyung Up Min, Paige Wickner & 1 others Hye Ryun Kang

Research output: Contribution to journalArticle

3 Citations (Scopus)

Abstract

Background: Hereditary angioedema (HAE) is a genetically heterogeneous autosomal dominant disorder characterized by recurrent episodes of nonpruritic, nonpitting edema increasing after puberty. It can be fatal due to laryngeal or gastrointestinal (GI) involvement with varied and changing frequency of mortality according to studies published from the Western countries. Epidemiological and clinical data of HAE in Asian countries are sparse. We sought to examine the clinical characteristics of HAE patients in Korea. Methods: Patients diagnosed with HAE at 15 tertiary hospitals across the country until 2016 were retrospectively reviewed. Results: A total of 65 patients diagnosed with HAE by 2016 were identified. The prevalence of HAE was estimated at 1.3/1,000,000 in Korea. Of the 65 patients, 21 (32.3%) were males. A total of 90.8% patients had type I HAE, while the remaining 9.2% patients had type II HAE. The first symptom developed after 20 years in 73.8% of patients, with a mean age 28.4 ± 14.1 years. The age at diagnosis was 36.5 ± 15.8 years, with a mean time delay of 7.8 ± 10.5 years. While the face (82.3%) and extremities (upper 71.0%, lower 62.9%) were the most frequently involved, the GI tract was affected in 40.5% of Korean HAE patients. Prophylaxis was maintained in 62.5% of patients. There was no reported case of death from HAE so far. Conclusions: The clinical manifestation and severity of HAE may vary according to ethnicity. HAE is more infrequent and GI involvement is less likely in Korea compared with Western countries.

Original languageEnglish
Pages (from-to)272-279
Number of pages8
JournalInternational Archives of Allergy and Immunology
Volume176
Issue number3-4
DOIs
Publication statusPublished - 2018 Jul 1

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Hereditary Angioedemas
Multicenter Studies
Hereditary Angioedema Types I and II
Korea
Puberty
Tertiary Care Centers
Upper Extremity
Gastrointestinal Tract
Edema

All Science Journal Classification (ASJC) codes

  • Immunology and Allergy
  • Immunology

Cite this

Jung, J. W., Suh, D. I., Park, H. J., Kim, S., Kwon, H. S., Yang, M. S., ... Kang, H. R. (2018). Clinical Features of Hereditary Angioedema in Korean Patients: A Nationwide Multicenter Study. International Archives of Allergy and Immunology, 176(3-4), 272-279. https://doi.org/10.1159/000488350
Jung, Jae Woo ; Suh, Dong In ; Park, Hye Jung ; Kim, Sujeong ; Kwon, Hyouk Soo ; Yang, Min Suk ; Park, Chan Sun ; Kim, Joo Hee ; Kim, Sae Hoon ; Lee, Yong Won ; Hur, Gyu Young ; Ye, Young Min ; Kwon, Yong Eun ; Park, Hye Kyung ; Kim, Cheol Woo ; Koh, Young Il ; Park, Jungwon ; Lee, Jong Myung ; Min, Kyung Up ; Wickner, Paige ; Kang, Hye Ryun. / Clinical Features of Hereditary Angioedema in Korean Patients : A Nationwide Multicenter Study. In: International Archives of Allergy and Immunology. 2018 ; Vol. 176, No. 3-4. pp. 272-279.
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title = "Clinical Features of Hereditary Angioedema in Korean Patients: A Nationwide Multicenter Study",
abstract = "Background: Hereditary angioedema (HAE) is a genetically heterogeneous autosomal dominant disorder characterized by recurrent episodes of nonpruritic, nonpitting edema increasing after puberty. It can be fatal due to laryngeal or gastrointestinal (GI) involvement with varied and changing frequency of mortality according to studies published from the Western countries. Epidemiological and clinical data of HAE in Asian countries are sparse. We sought to examine the clinical characteristics of HAE patients in Korea. Methods: Patients diagnosed with HAE at 15 tertiary hospitals across the country until 2016 were retrospectively reviewed. Results: A total of 65 patients diagnosed with HAE by 2016 were identified. The prevalence of HAE was estimated at 1.3/1,000,000 in Korea. Of the 65 patients, 21 (32.3{\%}) were males. A total of 90.8{\%} patients had type I HAE, while the remaining 9.2{\%} patients had type II HAE. The first symptom developed after 20 years in 73.8{\%} of patients, with a mean age 28.4 ± 14.1 years. The age at diagnosis was 36.5 ± 15.8 years, with a mean time delay of 7.8 ± 10.5 years. While the face (82.3{\%}) and extremities (upper 71.0{\%}, lower 62.9{\%}) were the most frequently involved, the GI tract was affected in 40.5{\%} of Korean HAE patients. Prophylaxis was maintained in 62.5{\%} of patients. There was no reported case of death from HAE so far. Conclusions: The clinical manifestation and severity of HAE may vary according to ethnicity. HAE is more infrequent and GI involvement is less likely in Korea compared with Western countries.",
author = "Jung, {Jae Woo} and Suh, {Dong In} and Park, {Hye Jung} and Sujeong Kim and Kwon, {Hyouk Soo} and Yang, {Min Suk} and Park, {Chan Sun} and Kim, {Joo Hee} and Kim, {Sae Hoon} and Lee, {Yong Won} and Hur, {Gyu Young} and Ye, {Young Min} and Kwon, {Yong Eun} and Park, {Hye Kyung} and Kim, {Cheol Woo} and Koh, {Young Il} and Jungwon Park and Lee, {Jong Myung} and Min, {Kyung Up} and Paige Wickner and Kang, {Hye Ryun}",
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Jung, JW, Suh, DI, Park, HJ, Kim, S, Kwon, HS, Yang, MS, Park, CS, Kim, JH, Kim, SH, Lee, YW, Hur, GY, Ye, YM, Kwon, YE, Park, HK, Kim, CW, Koh, YI, Park, J, Lee, JM, Min, KU, Wickner, P & Kang, HR 2018, 'Clinical Features of Hereditary Angioedema in Korean Patients: A Nationwide Multicenter Study', International Archives of Allergy and Immunology, vol. 176, no. 3-4, pp. 272-279. https://doi.org/10.1159/000488350

Clinical Features of Hereditary Angioedema in Korean Patients : A Nationwide Multicenter Study. / Jung, Jae Woo; Suh, Dong In; Park, Hye Jung; Kim, Sujeong; Kwon, Hyouk Soo; Yang, Min Suk; Park, Chan Sun; Kim, Joo Hee; Kim, Sae Hoon; Lee, Yong Won; Hur, Gyu Young; Ye, Young Min; Kwon, Yong Eun; Park, Hye Kyung; Kim, Cheol Woo; Koh, Young Il; Park, Jungwon; Lee, Jong Myung; Min, Kyung Up; Wickner, Paige; Kang, Hye Ryun.

In: International Archives of Allergy and Immunology, Vol. 176, No. 3-4, 01.07.2018, p. 272-279.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Clinical Features of Hereditary Angioedema in Korean Patients

T2 - A Nationwide Multicenter Study

AU - Jung, Jae Woo

AU - Suh, Dong In

AU - Park, Hye Jung

AU - Kim, Sujeong

AU - Kwon, Hyouk Soo

AU - Yang, Min Suk

AU - Park, Chan Sun

AU - Kim, Joo Hee

AU - Kim, Sae Hoon

AU - Lee, Yong Won

AU - Hur, Gyu Young

AU - Ye, Young Min

AU - Kwon, Yong Eun

AU - Park, Hye Kyung

AU - Kim, Cheol Woo

AU - Koh, Young Il

AU - Park, Jungwon

AU - Lee, Jong Myung

AU - Min, Kyung Up

AU - Wickner, Paige

AU - Kang, Hye Ryun

PY - 2018/7/1

Y1 - 2018/7/1

N2 - Background: Hereditary angioedema (HAE) is a genetically heterogeneous autosomal dominant disorder characterized by recurrent episodes of nonpruritic, nonpitting edema increasing after puberty. It can be fatal due to laryngeal or gastrointestinal (GI) involvement with varied and changing frequency of mortality according to studies published from the Western countries. Epidemiological and clinical data of HAE in Asian countries are sparse. We sought to examine the clinical characteristics of HAE patients in Korea. Methods: Patients diagnosed with HAE at 15 tertiary hospitals across the country until 2016 were retrospectively reviewed. Results: A total of 65 patients diagnosed with HAE by 2016 were identified. The prevalence of HAE was estimated at 1.3/1,000,000 in Korea. Of the 65 patients, 21 (32.3%) were males. A total of 90.8% patients had type I HAE, while the remaining 9.2% patients had type II HAE. The first symptom developed after 20 years in 73.8% of patients, with a mean age 28.4 ± 14.1 years. The age at diagnosis was 36.5 ± 15.8 years, with a mean time delay of 7.8 ± 10.5 years. While the face (82.3%) and extremities (upper 71.0%, lower 62.9%) were the most frequently involved, the GI tract was affected in 40.5% of Korean HAE patients. Prophylaxis was maintained in 62.5% of patients. There was no reported case of death from HAE so far. Conclusions: The clinical manifestation and severity of HAE may vary according to ethnicity. HAE is more infrequent and GI involvement is less likely in Korea compared with Western countries.

AB - Background: Hereditary angioedema (HAE) is a genetically heterogeneous autosomal dominant disorder characterized by recurrent episodes of nonpruritic, nonpitting edema increasing after puberty. It can be fatal due to laryngeal or gastrointestinal (GI) involvement with varied and changing frequency of mortality according to studies published from the Western countries. Epidemiological and clinical data of HAE in Asian countries are sparse. We sought to examine the clinical characteristics of HAE patients in Korea. Methods: Patients diagnosed with HAE at 15 tertiary hospitals across the country until 2016 were retrospectively reviewed. Results: A total of 65 patients diagnosed with HAE by 2016 were identified. The prevalence of HAE was estimated at 1.3/1,000,000 in Korea. Of the 65 patients, 21 (32.3%) were males. A total of 90.8% patients had type I HAE, while the remaining 9.2% patients had type II HAE. The first symptom developed after 20 years in 73.8% of patients, with a mean age 28.4 ± 14.1 years. The age at diagnosis was 36.5 ± 15.8 years, with a mean time delay of 7.8 ± 10.5 years. While the face (82.3%) and extremities (upper 71.0%, lower 62.9%) were the most frequently involved, the GI tract was affected in 40.5% of Korean HAE patients. Prophylaxis was maintained in 62.5% of patients. There was no reported case of death from HAE so far. Conclusions: The clinical manifestation and severity of HAE may vary according to ethnicity. HAE is more infrequent and GI involvement is less likely in Korea compared with Western countries.

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U2 - 10.1159/000488350

DO - 10.1159/000488350

M3 - Article

VL - 176

SP - 272

EP - 279

JO - International Archives of Allergy and Immunology

JF - International Archives of Allergy and Immunology

SN - 1018-2438

IS - 3-4

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