Recombinant human C1 inhibitor for the prophylaxis of hereditary angioedema attacks: a pilot study

Reshef, Avner; Moldovan, Dumitru; Obtułowicz, Krystyna; Leibovich, Iris; Mihály, Enikő; Visscher, Sonja; Relan, Anurag

doi:10.1111/all.12060

Cited by 43 publications

(27 citation statements)

References 28 publications

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“…Importantly, after a single administration of rhC1INH, there were no thrombotic or anaphylactic events in patients treated with rhC1INH, no patients developed neutralizing antibodies to rhC1INH, and antibodies against C1INH and host-related impurities were infrequent, consistent with findings in prior rhC1INH clinical trials. 8e10, 12,29 Evaluation of the safety and immunogenicity of repeated rhC1INH administration in this population of patients from an open-label extension study remains ongoing. However, the authors' previous experience in open-label studies suggests that treatment with rhC1INH for repeated attacks is safe and efficacious.…”

Section: Discussionmentioning

confidence: 99%

Recombinant human C1-esterase inhibitor relieves symptoms of hereditary angioedema attacks: phase 3, randomized, placebo-controlled trial

Riedl

Bernstein

et al. 2014

Annals of Allergy, Asthma & Immunology

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Section: Discussionmentioning

confidence: 99%

Recombinant human C1-esterase inhibitor relieves symptoms of hereditary angioedema attacks: phase 3, randomized, placebo-controlled trial

Riedl

Bernstein

et al. 2014

Annals of Allergy, Asthma & Immunology

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“…No drug-related adverse events or immunogenic reactions to the C1-Inh or rabbit proteins have been observed so far (93)(94)(95). Recombinant C1-Inh has also been shown to be effective in long-term prophylaxis (96).…”

Section: Treatmentmentioning

confidence: 95%

Hereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment

Zeerleder

Levi

2016

Annals of Medicine

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Uncontrolled generation of bradykinin (BK) due to insufficient levels of protease inhibitors controlling contact phase (CP) activation, increased activity of CP proteins, and/or inadequate degradation of BK into inactive peptides increases vascular permeability via BK-receptor 2 (BKR2) and results in subcutaneous and submucosal edema formation. Hereditary and acquired angioedema due to C1-inhibitor deficiency (C1-INH-HAE and -AAE) are diseases characterized by serious and potentially fatal attacks of subcutaneous and submucosal edemas of upper airways, facial structures, abdomen, and extremities, due to inadequate control of BK generation. A decreased activity of C1-inhibitor is the hallmark of C1-INH-HAE (types 1 and 2) due to a mutation in the C1-inhibitor gene, whereas the deficiency in C1-inhibitor in C1-INH-AAE is the result of autoimmune phenomena. In HAE with normal C1-inhibitor, a significant percentage of patients have an increased activity of factor XIIa due to a FXII mutation (FXII-HAE). Treatment of C1-inhibitordependent angioedema focuses on restoring control of BK generation by inhibition of CP proteases by correcting the balance between CP inhibitors and BK breakdown or by inhibition of BK-mediated effects at the BKR2 on endothelial cells. This review will address the pathophysiology, clinical picture, diagnosis and available treatment in C1-inhibitor-dependent angioedema focusing on BK-release and its regulation. ä KEY MESSAGESInadequate control of bradykinin formation results in the formation of characteristic subcutaneous and submucosal edemas of the skin, upper airways, facial structures, abdomen and extremities as seen in hereditary and acquired C1-inhibitor-dependent angioedema. Diagnosis of hereditary and acquired C1-inhibitor-dependent angioedema may be troublesome as illustrated by the fact that there is a significant delay in diagnosis; a certain grade of suspicion is therefore crucial for quick diagnosis. Submucosal edema formation in hereditary and acquired C1-inhibitor-dependent angioedema is potentially life threatening and can occur at any age. To date effective therapies for acute and prophylactic treatment are available. ARTICLE HISTORY

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“…54 HAE is a rare disease (1 patient per 10 000-50 000 people), with autosomal dominant inheritance, caused by mutations in the gene governing the production abdominal organ edema) C1-INH concentrate (Berinert, Cynryze) at a dose of 20 U/kg [59][60][61][62][63][64][65][66][67] should be administered immediately, or recombinant human C1-INH (Ruconest) at a dose of 50 U/kg. 54,[68][69][70] Alternatively, icatibant, bradykinin 1 B 2 R blocker, may be administered subcutaneously.…”

Section: Characteristic Features Of Bradykinin-mediatedmentioning

confidence: 99%

Bradykinin-mediated angioedema

Obtułowicz¹

2016

Polish Archives of Internal Medicine

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Recombinant human C1 inhibitor for the prophylaxis of hereditary angioedema attacks: a pilot study

Abstract: Weekly administrations of 50 U/kg rhC1INH appeared to reduce the frequency of HAE attacks and were generally safe and well tolerated.

Cited by 43 publications

References 28 publications

Recombinant human C1-esterase inhibitor relieves symptoms of hereditary angioedema attacks: phase 3, randomized, placebo-controlled trial

Recombinant human C1-esterase inhibitor relieves symptoms of hereditary angioedema attacks: phase 3, randomized, placebo-controlled trial

Hereditary and acquired C1-inhibitor-dependent angioedema: from pathophysiology to treatment

Bradykinin-mediated angioedema

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