Diretrizes brasileiras para o diagnóstico e tratamento do angioedema
                        hereditário – 2017

Giavina‐Bianchi, Pedro; Arruda, L. Karla; Aun, Marcelo Vívolo; Campos, Régis A.; Chong-Neto, Herberto José; Constantino-Silva, Rosemeire Navickas; Fernandes, Fátima F.; Ferraro, Marcelo Rosanova; Ferriani, Mariana Paes Leme; França, Alfeu Tavares; Fusaro, Gustavo; Garcia, Juliana F.B.; Komninakis, Shirley Vasconcelos; Maia, Luana S.M.; Mansour, Eli; Moreno, Adriana S.; Motta, Antônio Abílio; Pesquero, João Bosco; Portilho, Nathália Coelho; Serpa, Faradiba Sarquis; Solé, Dírceu; Toledo, Eliana; Valle, Solange Oliveira Rodrigues; Veronez, Camila; Grumach, Anete Sevciovic

doi:10.5935/2526-5393.20170005

Cited by 16 publications

(80 citation statements)

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“…Family history was evidenced in approximately 2/3 of the patients, as described previously ( 13 , 20 ). The lack of affected relatives in approximately 25% of the cases may be associated with new mutations, although clinically these patients do not differ from those with familial inheritance ( 15 ).…”

Section: Discussionmentioning

confidence: 73%

“…However, the rapid increase in the population with HAE observed in approximately 8 years of work reflects the referral of patients from several regions of the country due to this center's status as a reference center. Thus, the high number of new cases in our region is expected, but the lack of disease recognition by a significant number of physicians, even among specialists, can make this action difficult ( 13 ).…”

Section: Discussionmentioning

confidence: 99%

“…Some patients reported signs and symptoms that preceded attacks, usually within 24 h: erythema serpiginous, irritability, weakness, nausea, and asthenia are the most prevalent in the literature ( 2 , 5 , 13 ). In this study, only 9/46 patients reported prodromes, much lower than the number described by Reshef et al ( 26 ), in which 82.5 to 95.7% of the patients (depending on the questionnaire applied) recognized a prodrome.…”

Section: Discussionmentioning

confidence: 99%

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Hereditary angioedema with C1 inhibitor (C1-INH) deficit: the strength of recognition (51 cases)

Fragnan

Tolentino

Borba

et al. 2018

Braz J Med Biol Res

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Hereditary angioedema (HAE) is a rare autosomal dominant disease due to C1 esterase inhibitor deficiency (C1-INH). The disease is characterized by subcutaneous and submucosal edema in the absence of urticaria due to the accumulation of bradykinin. This descriptive study aimed to evaluate the clinical characteristics of patients with a confirmed diagnosis of HAE referred to our Outpatient Clinic between December 2009 and November 2017. Fifty-one patients (38 F, 13 M) with a mean age of 32 years (range: 7–70 y) were included. Family history of HAE was reported in 70% (36/51) of the cases; 33/46 patients became symptomatic by 18 years of age. The median time between onset of symptoms and diagnosis was 13 years (3 mo–50 y). The most frequent triggering factors for attacks were stress (74.4%), trauma (56.4%), and hormonal variations (56%). The main symptoms were subcutaneous edema in 93.5% (43/46) of patients, gastrointestinal symptoms in 84.8% (39/46), and obstruction in the upper airways in 34.8% (16/46). Hospitalization occurred in 65.2%, of whom 13.3% had to be transferred to the Intensive Care Unit. Prophylactic treatment was instituted in 87% (40/46) of patients, and 56.5% (26/46) required additional treatment to control attacks. Owing to our data collection over a period of 8 years, a significant number of patients were identified by this HAE reference center. Despite early recognition and prophylactic treatment, a high percentage of patients were hospitalized. HAE is still diagnosed late, reinforcing the need for more reference centers specialized in diagnosis and educational projects for health professionals.

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Section: Discussionmentioning

confidence: 73%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Hereditary angioedema with C1 inhibitor (C1-INH) deficit: the strength of recognition (51 cases)

Fragnan

Tolentino

Borba

et al. 2018

Braz J Med Biol Res

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“…Using the guidelines as an orientation, but attending patients with their various phenotypes in a personalized way, is an art. The publication of the "Brazilian Guidelines for the Diagnosis and Treatment of Hereditary Angioedema" in 2011 1 , 2 was a milestone that boosted the care, education and research in HAE in the country. Knowledge about the disease improved, patient diagnoses increased and were made earlier, and new treatments became available, providing great improvement in HAE management.…”

mentioning

confidence: 99%

“…In this scenario, a group of experts from the Brazilian Association of Allergy and Immunology (ASBAI) and the Brazilian Group for the Study of Hereditary Angioedema (GEBRAEH) updated the 2011 Guidelines. While 9 specialists from 7 services participated in the first guidelines, 27 participants representing 13 services elaborated the "Brazilian Guidelines for the Diagnosis and Treatment of Hereditary Angioedema - 2017" 3 , 4 . In the 2017 guidelines, the flowcharts and algorithms for the diagnosis and treatment of HAE were updated, with the inclusion of new drugs that became available in Brazil.…”

mentioning

confidence: 99%

Improving the Management of Hereditary Angioedema

Giavina‐Bianchi

Kalil

2018

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Current challenges and future opportunities in patient‐focused management of hereditary angioedema: A narrative review

Grumach

Gadir

Kessel

et al. 2023

Clinical & Translational All

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Patients with hereditary angioedema (HAE) experience a high burden of disease due to unpredictable, painful, disfiguring, and potentially life-threatening HAE attacks.Multiple HAE-specific medications for the on-demand treatment, short-term and long-term prophylaxis of HAE attacks have entered the market in recent years; however, the availability and access to these medications may vary between different countries. For this review, PubMed and EMBASE databases were searched for guidelines, consensus statements, and other publications on HAE management as well as publications on quality of life in patients with HAE. The current guidelines and recent literature on HAE management in specific countries are summarized with the aim to highlight the similarities and differences between guideline recommendations and the country-specific clinical practice. Improvement in quality of life, which is a key goal in HAE management, is also discussed and the countryspecific trends are highlighted. Finally, the ways to achieve a more patient-centric approach to HAE management within the framework set by the clinical management guidelines are examined.

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Diretrizes brasileiras para o diagnóstico e tratamento do angioedema hereditário – 2017

Cited by 16 publications

References 0 publications

Hereditary angioedema with C1 inhibitor (C1-INH) deficit: the strength of recognition (51 cases)

Hereditary angioedema with C1 inhibitor (C1-INH) deficit: the strength of recognition (51 cases)

Improving the Management of Hereditary Angioedema

Current challenges and future opportunities in patient‐focused management of hereditary angioedema: A narrative review

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