Long-Term Prophylaxis of Hereditary Angioedema with a Pasteurized C1 Inhibitor Concentrate

Tallroth, G.A.

doi:10.1159/000321830

Cited by 13 publications

(5 citation statements)

References 41 publications

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“…To focus more clearly on the effect of TA on AE attacks, patients who also took another maintenance treatment (androgen derivatives or C1‐inhibitor concentrate ) and patients treated with progestins were excluded. This last was necessary, as a recent study showed that these could be used effectively as a maintenance treatment, reducing the frequency of attacks.…”

Section: Discussionmentioning

confidence: 99%

Tranexamic acid as maintenance treatment for non-histaminergic angioedema: analysis of efficacy and safety in 37 patients

Wintenberger

Boccon‐Gibod

Launay

et al. 2014

Clinical and Experimental Immunology

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SummaryAngioedema (AE) is a clinical syndrome characterized by localised swelling lasting several hours. The swelling is often recurring and can be lethal if it is located in the laryngeal region. Much progress has been made recently in the treatment of acute episodes, but no consensus has been reached on maintenance treatment. We have performed a national retrospective observational study to assess the use of tranexamic acid (TA) as maintenance treatment for non-histaminergic AE [hereditary AE (HAE) or idiopathic nonhistaminergic AE]. Records for 64 cases were collected from 1 October 2012 to 31 August 2013; 37 of these were included (12 HAE with C1-inhibitor deficiency, six with HAE with normal C1-inhibitor and 19 idiopathic nonhistaminergic AE). When treated with TA over six months, the number of attacks was reduced by 75% in 17 patients, 10 patients showed a lower level of reduction and 10 had the same number of attacks. In no instances were symptoms increased. No thromboembolic events were observed, and the main side effects were digestive in nature. Thus, TA, which is well tolerated and inexpensive, appears to be an effective maintenance treatment for some patients with HAE or idiopathic non-histaminergic AE.

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Section: Discussionmentioning

confidence: 99%

Tranexamic acid as maintenance treatment for non-histaminergic angioedema: analysis of efficacy and safety in 37 patients

Wintenberger

Boccon‐Gibod

Launay

et al. 2014

Clinical and Experimental Immunology

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“…Dose and/or frequency may need adjustment for optimum control. On-demand therapy (C1-INH concentrate, ecallantide, or icatibant) should be available for all patients on prophylaxis because breakthrough attacks occur in most patients on long-term prophylaxis [35,99-101]. …”

Section: Therapy Of Hae-1/2mentioning

confidence: 99%

WAO Guideline for the Management of Hereditary Angioedema

Craig

Pürsün

Bork

et al. 2012

World Allergy Organization Journal

292

428

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Hereditary Angioedema (HAE) is a rare disease and for this reason proper diagnosis and appropriate therapy are often unknown or not available for physicians and other health care providers. For this reason we convened a group of specialists that focus upon HAE from around the world to develop not only a consensus on diagnosis and management of HAE, but to also provide evidence based grades, strength of evidence and classification for the consensus. Since both consensus and evidence grading were adhered to the document meets criteria as a guideline. The outcome of the guideline is to improve diagnosis and management of patients with HAE throughout the world and to help initiate uniform care and availability of therapies to all with the diagnosis no matter where the residence of the individual with HAE exists.

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“…Se esses medicamentos não forem disponíveis, deverá ser feito plasma fresco congelado é de 10 mL/kg, mas o seu uso é ainda controverso. [17][18][19] Medicamentos como C1-INH, progesterona, andrógenos atenuados particularmente o danazol, e os antifibrinolíticos como o ácido tranexâmico e ácido épsilon-aminocapróico têm sido utilizados de forma eficaz na prevenção de crises. [20][21][22][23] Um novo fármaco, um inibidor da calicreína oral (avoralstat), está em estudo para o tratamento profilático do AEH.…”

Section: Discussionunclassified

Angioedema Recidivante Idiopático: Uma Possível Apresentação de Angioedema Hereditário Tipo III

Biermann

Lima

Santos

et al. 2018

SPDV

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RESUMO -Dentro do angioedema idiopático não histaminérgico estão descritos três tipos de angioedema hereditário (AEH).O tipo I e II por défice de C1-INH (inibidor de C1-esterase) é o mais frequente e o tipo III, mais raro, ocorre maioritariamente em mulheres jovens, sem alterações do C1-inibidor (C1-INH), e pode associar-se a mutações no gene do fator XII. Trauma, stress e estrogénios podem ser os precipitantes, o tratamento é ainda um desafio pela ausência de estudos controlados. Apresentamos um caso de possível AEH tipo III numa jovem, agravado pela introdução de contraceptivo oral e melhoria com a sua suspensão e tratamento com ácido tranexâmico, mas sem história familiar e sem mutação do factor XII. PALAVRAS-CHAVE - INTRODUÇÃOO angioedema hereditário (AEH) é uma doença rara, autossómica dominante, com uma prevalência de 1:50 000, representando apenas 2% dos casos de angioedema. O AEH é frequentemente subdiagnosticado e, consequentemente, o diagnóstico é muitas vezes tardio, podendo comprometer o prognóstico. Ao contrário do angioedema histaminérgi-co, mais comum e frequentemente associado à urticária, o AEH é mediado por cininas, tornando os anti-histamínicos, corticosteroides e adrenalina inadequados no tratamento destas formas de angioedema. 1,2Existem três tipos de AEH, do ponto de vista clínico, basicamente indistinguíveis, mas com discretas peculiaridades. O tipo I representa a maioria dos casos (85%), e é resultante da deficiência quantitativa do C1-inibidor (C1-INH), enquanto que o AEH tipo II compreende 15% dos casos e é causado por uma diminuição da atividade funcional do C1-INH, com níveis séricos ou plasmáticos normais da proteína.

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Long-Term Prophylaxis of Hereditary Angioedema with a Pasteurized C1 Inhibitor Concentrate

Cited by 13 publications

References 41 publications

Tranexamic acid as maintenance treatment for non-histaminergic angioedema: analysis of efficacy and safety in 37 patients

Tranexamic acid as maintenance treatment for non-histaminergic angioedema: analysis of efficacy and safety in 37 patients

WAO Guideline for the Management of Hereditary Angioedema

Angioedema Recidivante Idiopático: Uma Possível Apresentação de Angioedema Hereditário Tipo III

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