Ecallantide for the Treatment of Acute Attacks in Hereditary Angioedema

Cicardi, Marco; Levy, Robyn J.; McNeil, Donald L.; Li, H. Henry; Sheffer, Albert L.; Campion, Marilyn; Horn, Patrick T.; Pullman, William E.

doi:10.1056/nejmoa0905079

Cited by 261 publications

(204 citation statements)

References 22 publications

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“…Level I evidence for acute treatment of C1‐INH‐HAE has been reviewed for pdC1‐INH Be , pdC1‐INH Ci , recombinant human C1‐INH (rhC1‐INH) (Rhucin/Ruconest ® ), kallikrein inhibitor ecallantide (Kalbitor ® ), and bradykinin B2 receptor antagonist icatibant (Firazyr ® ) 85, 91, 92, 95, 96, 97, 98, 99. Unfortunately, these treatments have been licensed mainly for adults with pediatric licensing pending and ages for licenses varying by jurisdiction.…”

Section: Resultsmentioning

confidence: 99%

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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

175

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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Section: Resultsmentioning

confidence: 99%

“…It is administered subcutaneously as a 30 mg dose 97. Hypersensitivity, including anaphylaxis, is a known risk of ecallantide treatment and occurs in 3% of treatments; no deaths are reported 108.…”

Section: Resultsmentioning

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

175

308

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“…Au vu de ces données, nous avons émis l'hypothèse de l'existence d'autoanticorps anti-VE-cadhérine (AAVE) dans les pathologies auto-immunes ; ils pourraient présenter un intérêt certain pour les cliniciens, en particulier dans les cas de vascularites auto-immunes primitives ou secondaires à une connectivite. Un test ELISA a été mis au point au associée à une dissociation des jonctions endothéliales faisant suite à la phosphorylation de la VE-cadhérine, et l'apparition de sVE [6]. Au cours d'une crise d'angioedème, le taux de ces deux médiateurs dans la circulation sanguine est augmenté.…”

Section: Exemple 3 : Les Maladies Auto-immunesunclassified

Instabilité des jonctions endothéliales : biomarqueurs du remodelage vasculaire

et al. 2014

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“…Icatibant is a bradykinin B2-receptor antagonist that reverses increased vascular permeability and inhibits vasodilation and extravasation[40]. Ecallantide is a human plasma kallikrein inhibitor that treats HAE symptoms by directly inhibiting plasma kallikrein and decreasing the conversion of high-molecular-weight kininogen to bradykinin[41]. Fresh frozen plasma has been used, but its utility is limited because it contains additional kinins and complement factors, posing a potential threat of worsening HAE symptoms[3,34].…”

Section: Treatment Approachesmentioning

confidence: 99%

“…Antifibrinolytics may also be useful in other patients in whom attenuated androgens or intravenous therapy is not appropriate. However, because of relatively low efficacy and poor safety profile, including the potential to cause hypotension, cardiac arrhythmias, rhabdomyolysis, and thromboembolism, the use of antifibrinolytics is limited[2,9,41,57]. …”

Section: Treatment Approachesmentioning

confidence: 99%

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

Gower¹,

Busse²,

Aygören‐Pürsün³

et al. 2011

World Allergy Organization Journal

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Hereditary angioedema (HAE) caused by C1-esterase inhibitor deficiency is an autosomal-dominant disease resulting from a mutation in the C1-inhibitor gene. HAE is characterized by recurrent attacks of intense, massive, localized subcutaneous edema involving the extremities, genitalia, face, or trunk, or submucosal edema of upper airway or bowels. These symptoms may be disabling, have a dramatic impact on quality of life, and can be life-threatening when affecting the upper airways. Because the manifestations and severity of HAE are highly variable and unpredictable, patients need individualized care to reduce the burden of HAE on daily life. Although effective therapy for the treatment of HAE attacks has been available in many countries for more than 30 years, until recently, there were no agents approved in the United States to treat HAE acutely. Therefore, prophylactic therapy is an integral part of HAE treatment in the United States and for selected patients worldwide. Routine long-term prophylaxis with either attenuated androgens or C1-esterase inhibitor has been shown to reduce the frequency and severity of HAE attacks. Therapy with attenuated androgens, a mainstay of treatment in the past, has been marked by concern about potential adverse effects. C1-esterase inhibitor works directly on the complement and contact plasma cascades to reduce bradykinin release, which is the primary pathologic mechanism in HAE. Different approaches to long-term prophylactic therapy can be used to successfully manage HAE when tailored to meet the needs of the individual patient.

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Ecallantide for the Treatment of Acute Attacks in Hereditary Angioedema

Cited by 261 publications

References 22 publications

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

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

Instabilité des jonctions endothéliales : biomarqueurs du remodelage vasculaire

Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies

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