Clinical features of hereditary angioedema in Chinese patients: New findings and differences from other populations

Xu, Yingyang; Jiang, Ying; Zhi, Yuxiang; Yin, Jia; Wang, Lianglu; Wen, Liping; Gu, Jinxin; Guan, Kaopeng; Zhang, Hongyu

doi:10.1684/ejd.2013.2105

Cited by 30 publications

(38 citation statements)

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“…We found a preponderance of HAE‐C1‐INH type I that is in accordance with the literature, but only two cases of HAE‐C1‐INH type II (1.4%), lower than the 15% to 20% estimated . Probably, the limited availability of C1‐INH function assays may explain the low incidence of HAE type II .…”

Section: Discussionsupporting

confidence: 90%

“…Probably, the limited availability of C1‐INH function assays may explain the low incidence of HAE type II . Steiner et al also described two cases of HAE type II (1.98%) in a study of 104 Swiss patients, as well as, Xu et al (two cases; 1.27% of 158 Chinese patients). On the other hand, Kargarsharif et al diagnosed HAE type I in 63.3% of 51 Iranian patients and HAE type II in 36.7%.…”

Section: Discussionmentioning

confidence: 98%

“…Four other patients (4.2%) referred syncope associated with severe abdominal pain. Xu et al identified syncope during severe abdominal attacks in two females out of 104 Chinese patients. Extensive edema, ascites, and vomiting can lead to hypovolemia and syncope …”

Section: Discussionmentioning

confidence: 99%

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Hereditary angioedema: a prospective study of a Brazilian single‐center cohort

et al. 2019

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Background Hereditary angioedema (HAE) is rare and still underdiagnosed in some countries. We aimed to describe HAE diagnosis and sociodemographic and clinical features in patients with HAE due to C1 inhibitor deficiency (HAE-C1-INH) followed up at a tertiary-level center in Rio de Janeiro, Brazil.Methods A descriptive, cross-sectional study with prospective data collection of 138Brazilian patients with HAE was performed. From the total, 107 patients with HAE-C1-INH were selected. Data were assessed based on a specific questionnaire. ResultsOne hundred and five patients had HAE type I (76.1%), and two had HAE type II (1.4%). Seventy-two were female (67.3%), and 35 were male (32.7%). Mean age was 38.0 AE 15.0 years (range: 12-73 years). A long delay (17.7 AE 12.6 years) until diagnosis was observed. About 86.9% had a familial history. Cutaneous edema (95.8%), abdominal pain (88.5%), and laryngeal edema (65.6%) were the most frequent symptoms. Triggering factors (95.8%) and prodromal symptoms (47.9%) were referred. Attacks were severe in 55.1% and moderate in 24.3%. Eleven (10.3%) were asymptomatic. HAE attacks were more frequent and severe (P = 0.021) in females. ConclusionsWe observed a considerable delay in diagnosis, even with familial history.The severity of HAE attacks, especially in females, highlights the need for an awareness of disease by gynecologists and obstetricians. Screening of familial members, including asymptomatic individuals, is critical for earlier diagnosis. Regional evaluation of patient profiles can be helpful to draw more attention about HAE and to improve quality of life.

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Section: Discussionsupporting

confidence: 90%

Section: Discussionmentioning

confidence: 98%

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Hereditary angioedema: a prospective study of a Brazilian single‐center cohort

et al. 2019

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“…Based on the variety of clinical manifestations and rare clinical cases, previous data have shown a signi cant diagnostic delay of 8-10 years for type 1 HAE in Europe and the United States [7][8][9] . And the mean delay in diagnosis of HAE for Chinese patients can be as long as 12.64 years according to a 2013 survey [10] . Long diagnostic delay and inappropriate treatment will aggravate the humanistic and economic burden for patients [11][12][13][14] .…”

Section: Type 1 Hae With Low C1-inh Expression and Function Accounts mentioning

confidence: 99%

The Natural Course of Hereditary Angioedema in a Chinese Cohort

Cao

Liu

Zhi³

2020

Preprint

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Background: Hereditary angioedema (HAE) is a rare disease with potential life-threatening risks. To study the natural course of HAE under therapy-free conditions throughout patient life is essential for practitioners and patients to avoid possible risk factors and guide treatment.Objectives: Describe the natural course of HAE and explore possible risk factors, providing new clues for guiding clinical prevention and treatment.Methods: A web-based survey was conducted in 103 Chinese patients with type 1 HAE. Disease progression at different age stages was provided by each participant. The data for exploring the natural course of HAE composed of two parts: one came from the participants who had never adopted any prophylactic drug for HAE; the other was from the patients with a history of medication, but only the periods before they got confirmed diagnosis and received medications were analyzed. The demographic characteristics, lifestyles, disease severity, and family history were also collected.Results: Among 103 patients, 14 (13.6%) had their first HAE attack before ten years old and 51 (49.5%) between 10 and 19. The disease worsened in 83.3% of the patients in their twenties. The proportion of patients with symptoms alleviated increased after the age of 30 years old, but the disease maintained relatively severe in most cases before 50. The participants also reported 233 members shared similar symptoms of angioedema in their family and 30 had died of laryngeal edema with the median death age of 46 years old. The disease severity was not observed to be affected significantly by gender, BMI, alcohol or smoking.Conclusions: We summarized HAE progression patterns under therapy-free conditions, showing the natural course of HAE development along with aging. Long-term prophylaxis and symptomatic treatment are recommended for all HAE patients, especially young and middle-aged and might be adjusted depending on the disease progression.

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“…Based on the variety of clinical manifestations and rare clinical cases, previous data have shown a significant diagnostic delay of 8-10 years for type 1 HAE in Europe and the United States [7][8][9] . And the mean delay in diagnosis of HAE for Chinese patients can be as long as 12.64 years according to a 2013 survey [10] . Long diagnostic delay and inappropriate treatment will aggravate the humanistic and economic burden for patients [11][12][13][14] .…”

Section: Introductionmentioning

confidence: 99%

The Natural Course of Hereditary Angioedema in a Chinese Cohort

Cao

Liu

Zhi³

2020

Preprint

View full text Add to dashboard Cite

Background Hereditary angioedema (HAE) is a rare disease with potential life-threatening risks. Until now, few studies have focused on the natural course of HAE, which is essential for practitioners and patients to predict disease progression and avoid possible risk factors. Objectives Describe the natural course of HAE and explore possible risk factors, providing new clues for guiding clinical prevention and treatment. Methods A web-based survey was conducted in 103 Chinese patients with type 1 HAE. Disease progression under therapy-free conditions was collected at different age stages. The demographic characteristics, lifestyles, disease severity and family history were also provided by each participant. Results Among 103 patients, 14 (13.6%) had their first HAE attack before ten years old and 51 (49.5%) between 10 and 19. The disease worsened in 83.3% of the patients in their twenties. The proportion of patients with symptoms alleviated increased after the age of 30 years old, but the disease maintained relatively severe in most cases before 50. The participants also reported 233 members shared similar symptoms of angioedema in their family and 30 had died of laryngeal edema with the median death age of 46 years old. The disease severity was not observed to be affected significantly by gender, BMI, alcohol or smoking. Conclusions We summarized HAE progression patterns in therapy-free conditions, showing the natural course of disease development along with aging. Long-term prophylaxis and symptomatic treatment are recommended for all HAE patients, especially young and middle-aged and might be adjusted depending on the disease progression.

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Clinical features of hereditary angioedema in Chinese patients: New findings and differences from other populations

Cited by 30 publications

References 0 publications

Hereditary angioedema: a prospective study of a Brazilian single‐center cohort

Hereditary angioedema: a prospective study of a Brazilian single‐center cohort

The Natural Course of Hereditary Angioedema in a Chinese Cohort

The Natural Course of Hereditary Angioedema in a Chinese Cohort

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