Clinical manifestations, diagnosis, and treatment of hereditary angioedema: survey data from 94 physicians in Japan

Ohsawa, Isao; Honda, Daisuke; Nagamachi, Seiji; Hisada, Atsuko; Shimamoto, Mamiko; Inoshita, Hiroyuki; Mano, Satoshi; Tomino, Yasuhiko

doi:10.1016/j.anai.2015.03.010

Cited by 37 publications

(42 citation statements)

References 30 publications

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“…Similar to the data from 2013 and other published literature, 23,24 our results indicated that significant progress was made in effectively managing HAE, but a high burden of disease remained for patients with HAE. Additional recent advances in HAE therapy that are more effective and easier to use are expected to further reduce the burden of disease.…”

Section: Resultssupporting

confidence: 81%

Hereditary angioedema from the patient's perspective: A follow-up patient survey

Banerji

Liu

Busse

et al. 2018

allergy asthma proc

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Section: Resultssupporting

confidence: 81%

Hereditary angioedema from the patient's perspective: A follow-up patient survey

Banerji

Liu

Busse

et al. 2018

allergy asthma proc

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“…In the USA, 600 deaths caused by HAE were identified over the 12-year period from 1999 to 2010 [38]. In contrast, the mortality rates of HAE in Japan and China were reported to be 11.7 and 11.4%, respectively [33, 39]. In this study, no mortality due to HAE was found in 65 Korean HAE cases.…”

Section: Discussionmentioning

confidence: 51%

Clinical Features of Hereditary Angioedema in Korean Patients: A Nationwide Multicenter Study

Jung

Suh

Park

et al. 2018

Int Arch Allergy Immunol

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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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“…Newborns may experience erythema marginatum as a prodromal symptom, but rarely swelling 4, 27. The reported age of onset of attacks varies from 4.4 to 18 years with mean age of first attack at the age of ten 3, 5, 6, 7, 8, 9, 10, 11, 12, 13. Colic may be an unrecognized symptom of C1‐INH‐HAE in infancy 28, 29, 30.…”

Section: Resultsmentioning

confidence: 99%

“…Edema involving the submucosa of the upper airways may cause airway obstruction and without treatment may lead to suffocation and death. The reported age of onset of attacks varies from 4.4 to 18 years with mean age of first attack at the age of ten 3, 5, 6, 7, 8, 9, 10, 11, 12, 13. Early onset of symptoms may predict a more severe course of disease 3, 14, 15.…”

mentioning

confidence: 99%

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

Farkas

Martinez‐Saguer²,

Bork

et al. 2016

Allergy

174

308

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BackgroundThe consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1‐INH‐HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1‐INH‐HAE.MethodsDuring an expert panel meeting that took place during the 9th C1 Inhibitor Deficiency Workshop in Budapest, 2015 (www.haenet.hu), pediatric data were presented and discussed and a consensus was developed by voting.ResultsThe symptoms of C1‐INH‐HAE often present in childhood. Differential diagnosis can be difficult as abdominal pain is common in pediatric C1‐INH‐HAE, but also commonly occurs in the general pediatric population. The early onset of symptoms may predict a more severe subsequent course of the disease. Before the age of 1 year, C1‐INH levels may be lower than in adults; therefore, it is advisable to confirm the diagnosis after the age of one year. All neonates/infants with an affected C1‐INH‐HAE family member should be screened for C1‐INH deficiency. Pediatric patients should always carry a C1‐INH‐HAE information card and medicine for emergency use. The regulatory approval status of the drugs for prophylaxis and for acute treatment is different in each country. Plasma‐derived C1‐INH, recombinant C1‐INH, and ecallantide are the only agents licensed for the acute treatment of pediatric patients. Clinical trials are underway with additional drugs. It is recommended to follow up patients in an HAE comprehensive care center.ConclusionsThe pediatric‐focused international consensus for the diagnosis and management of C1‐INH‐HAE patients was created.

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Clinical manifestations, diagnosis, and treatment of hereditary angioedema: survey data from 94 physicians in Japan

Cited by 37 publications

References 30 publications

Hereditary angioedema from the patient's perspective: A follow-up patient survey

Hereditary angioedema from the patient's perspective: A follow-up patient survey

Clinical Features of Hereditary Angioedema in Korean Patients: A Nationwide Multicenter Study

International consensus on the diagnosis and management of pediatric patients with hereditary angioedema with C1 inhibitor deficiency

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