Recent Advances in Management and Treatment of Hereditary Angioedema

Sardana, Niti; Craig, Timothy J.

doi:10.1542/peds.2011-0546

Cited by 12 publications

(12 citation statements)

References 49 publications

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“…19 Effective prophylactic therapy, both short-and long-term, has long been provided by attenuated androgens and antifibrinolytics, but their use is limited by untoward side effects. 17,18 The introduction of new and effective therapies for patients with HAE has revolutionized the treatment landscape. Though plasmaderived C1 INH therapy for HAE has been available abroad for more than three decades, these drugs were only imported into the United States and approved by the Food and Drug Administration (FDA) within the last 5-6 years.…”

Section: Resultsmentioning

confidence: 99%

“…Both acute and prophylactic treatment of HAE has been revolutionized in the past decade, with the addition of new, effective agents added to the armamentarium for therapy. 17,18 Though HAE often presents in childhood or adolescence, published literature focused on the treatment of HAE in pediatric patients is scarce. Comprehensive consensus recommendations for HAE disease management were issued in 2010, but the guidelines fail to address pediatric HAE specifically with any robust, evidence-based support.…”

Section: Introductionmentioning

confidence: 99%

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Management of Hereditary Angioedema in Childhood

WuEveline

FrankMichael

2014

Pediatric Allergy, Immunology, and Pulmonology

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Background: Hereditary angioedema (HAE) is a rare disease characterized by recurrent, self-limiting attacks of subcutaneous and submucosal edema. Though a majority of patients will experience symptom onset before 20 years of age, there is a paucity of published literature regarding the management of HAE in children and adolescents. Methods: A comprehensive literature review regarding the management of pediatric HAE due to C1-esterase inhibitor deficiency (C1 INH) was performed. Results: A collection of case reports and case series suggest antifibrinolytics and attenuated androgens at low dose are safe and effective options for short-and long-term prophylaxis in pediatric HAE. Plasma-derived C1 INH preparations are available for both on-demand and prophylactic therapy in the United States, and post-hoc analyses of the pediatric patients enrolled in the larger clinical trials support their use in pediatric HAE. Ecallantide and icatibant are kinin-pathway modulators, but only ecallantide is currently approved for use in children 12 years of age and older in the United States. There was no available literature regarding the use of icatibant in pediatric HAE, but ecallantide's efficacy and tolerability were demonstrated in a post-hoc analysis of pediatric patients that participated in four larger, prospective studies. Conclusions: Within the limits of the present literature review, the currently available and approved therapies used in the treatment of adults with HAE also appear safe and effective for use in children and adolescents at appropriate doses. Options available in the United States include plasma-derived C1-INH, attenuated androgens, and antifibrinolytics for prophylactic prevention and plasma-derived C1 INH and ecallantide for on-demand, acute therapy.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Management of Hereditary Angioedema in Childhood

WuEveline

FrankMichael

2014

Pediatric Allergy, Immunology, and Pulmonology

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“…Contudo, o danazol é contraindicado em gestantes e crianças 14 . Nestas, seus efeitos colaterais incluem fechamento precoce de epífises ósseas (resultando em baixa estatura) e virilização 15 . As pacientes em idade fértil devem ser advertidas sobre os riscos e que necessitam de troca de medicação durante a gestação 11 .…”

Section: Tratamentounclassified

“…Apesar de eficaz para a maioria dos sintomas, apresenta como desvantagem tempo de inicio de ação longo, em média de 12h 16 . Em muitos países, a terapia com ácido tranexâmico já foi quase completamente substituída por outras drogas, com tempo de inicio de ação mais curto, como o antagonista do receptor de bradicinina e o concentrado do inibidor de C1 15 , apenas recentemente disponíveis no Brasil 13 . Como opção, pode-se utilizar também o plasma fresco.…”

Section: Tratamentounclassified

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Angioedema Hereditário: Uma Atualização.

et al. 2015

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Objetivo: O angioedema hereditário é uma doença genética, de herança autossômica dominante caracterizada pela deficiência quantitativa e qualitativa do inibidor de C1 resultando em edema de vários órgãos. O presente estudo tem como objetivo uma revisão da literatura, visando atualização do diagnóstico, classificação e tratamento desta patologia.Método: Revisão da literatura recente de artigos originais e diretrizes nacionais e internacionais.Resultados: As manifestações clínicas de angioedema hereditário são dor abdominal, vômitos, diarreia e edema de membros, face, laringe e genitais. O diagnóstico se faz por anamnese, exame físico e exames laboratoriais, entre eles dosagem sérica de C4 e do inibidor da C1-esterase. A classificação é baseada na deficiência ou não da enzima C1 esterase. A abordagem terapêutica segue orientações gerais para evitar fatores desencadeantes, medicamentos para o manejo das crises e tratamento profilático. Andrógenos atenuados são utilizados para tratamento preventivo e antifibrinolíticos e plasma fresco são empregados nas crises. Novas drogas inibidoras de bradicinina e concentrados de C1 derivados de plasma têm sido utilizados no tratamento e prevenção das crises. Conclusões: O angioedema hereditário é doença grave, com alta morbidade e mortalidade. O diagnóstico correto desta condição pelos profissionais da saúde é essencial para seu manejo adequado.

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2018

Atlas Der Pädiatrischen Dermatologie

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Recent Advances in Management and Treatment of Hereditary Angioedema

Abstract: In this article we review the changing therapeutic options available for patients in 2011 and beyond.

Cited by 12 publications

References 49 publications

Management of Hereditary Angioedema in Childhood

Management of Hereditary Angioedema in Childhood

Angioedema Hereditário: Uma Atualização.

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