Safety and Efficacy of Nanofiltered C1 Esterase Inhibitor (Human) (Cinryze™) for the Treatment of Laryngeal Attacks in Subjects with Hereditary Angioedema (HAE)

Riedl, Marc A.; Baker, James; Hurewitz, David; Lumry, William R.; Vegh, Arthur B.; White, Martha V.; Busse, Paula J.; Rubinstein, Arye; Kalfus, Ira

doi:10.1016/j.jaci.2010.12.929

Cited by 2 publications

(11 citation statements)

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“…[15,[20][21][22] The median time to onset of clinical relief with C1-INH NF concentrate was also consistent regardless of symptom site or number of attacks (table IV). [15,[20][21][22] HAE attacks may be treated successfully with C1-INH NF concentrate regardless of patient age. [20][21][22][23] The proportion of attacks achieving unequivocal relief of the defining symptom within 4 hours of C1-INH NF concentrate infusion was 89% in 22 pediatric patients (aged 2 to <18 years), and 86% in adult patients.…”

Section: Parametermentioning

confidence: 70%

“…Of note, none of the laryngeal attacks required intubation. [22] Subsequent repeated administration did not diminish the clinical efficacy of C1-INH NF concentrate. [15,[20][21][22] In analyses of patients treated for ten or more attacks at any site, or up to seven gastrointestinal, cutaneous, or laryngeal attacks, a clinical response within 4 hours of C1-INH NF concentrate administration was consistently shown for over two-thirds of attacks, regardless of symptom site or number of attacks (table IV).…”

Section: Parametermentioning

confidence: 93%

“…Two randomized, double-blind, placebo-controlled trials (reported together in one paper) have been conducted; [4] one investigated the efficacy of C1-INH NF in the treatment of an angioedema attack, and the other evaluated the use of the agent in the routine prevention of attacks. [4] Results of two open-label studies of the efficacy on C1-INH NF concentrate in the treatment [15] or routine prevention [16] of attacks, subset analyses of the open-label acute-treatment study, [20][21][22][23][24] and pooled analyses of trial data [25] were also presented (available as abstracts and or posters [15,16,[20][21][22][23][24][25][26] ). Additional results from these trials are derived from the EMA documents.…”

Section: Pharmacokinetic Profilementioning

confidence: 99%

“…The median time to the beginning of unequivocal relief of the first attack at any anatomic location was 45 minutes. [15] Moreover, according to subset analyses, C1-INH NF concentrate was effective regardless of the symptom site, providing unequivocal and or clinical relief in most gastrointestinal, [20] cutaneous (facial and extremity), [21] laryngeal, [22] and genitourinary [15] attacks at both 1 and 4 hours post-infusion (table IV). Of note, none of the laryngeal attacks required intubation.…”

Section: Parametermentioning

confidence: 99%

“…[15,[20][21][22] In analyses of patients treated for ten or more attacks at any site, or up to seven gastrointestinal, cutaneous, or laryngeal attacks, a clinical response within 4 hours of C1-INH NF concentrate administration was consistently shown for over two-thirds of attacks, regardless of symptom site or number of attacks (table IV). [15,[20][21][22] The median time to onset of clinical relief with C1-INH NF concentrate was also consistent regardless of symptom site or number of attacks (table IV). [15,[20][21][22] HAE attacks may be treated successfully with C1-INH NF concentrate regardless of patient age.…”

Section: Parametermentioning

confidence: 99%

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Nanofiltered Human C1 Inhibitor Concentrate (Cinryze®)

Lyseng‐Williamson

2011

BioDrugs

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Intravenous nanofiltered human C1 inhibitor (C1-INH NF) concentrate (Cinryze®) is used as a direct replacement of deficient levels of plasma C1 inhibitor in patients with hereditary angioedema (HAE). In the EU, C1-INH NF concentrate 1000 U is indicated in the treatment, pre-procedural prevention, and routine prevention of angioedema attacks in adults and adolescents with HAE. Intravenous C1-INH NF concentrate 1000 U effectively relieved angioedema attacks in patients with HAE. In a randomized, double-blind trial in pediatric and adult patients, the median time to onset of unequivocal relief from an attack was significantly shorter with C1-INH NF concentrate than with placebo. In an open-label trial, both unequivocal relief and clinical relief were shown in the majority of attacks within 1 and 4 hours of infusion of C1-INH NF concentrate, regardless of the site (i.e. gastrointestinal, cutaneous, laryngeal, or genitourinary) of the defining symptom. When administered prior to a procedure, open-label intravenous C1-INH NF concentrate 1000 U reduced the incidence of angioedema attacks during and after a variety of dental, surgical, or interventional diagnostic procedures in pediatric and adult patients with HAE. Routine preventative treatment with intravenous C1-INH NF concentrate 1000 U every 3 or 4 days reduced the number of angioedema attacks. In a randomized, double-blind, crossover trial in pediatric and adult patients with HAE, the mean normalized number of attacks per 12-week period was significantly lower during routine prevention with C1-INH NF concentrate than with placebo. Routine prevention with C1-INH NF concentrate reduced the median monthly attack rate from baseline in an open-label trial. Intravenous C1-INH NF concentrate was well tolerated in clinical trials in patients with HAE. No cases of viral transmission were reported.

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Section: Parametermentioning

confidence: 70%

Section: Parametermentioning

confidence: 93%

Section: Pharmacokinetic Profilementioning

confidence: 99%

Section: Parametermentioning

confidence: 99%

Section: Parametermentioning

confidence: 99%

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Nanofiltered Human C1 Inhibitor Concentrate (Cinryze®)

Lyseng‐Williamson

2011

BioDrugs

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Human Plasma-Derived, Nanofiltered, C1-Inhibitor Concentrate (Cinryze®), a Novel Therapeutic Alternative for the Management of Hereditary Angioedema Resulting from C1-Inhibitor Deficiency

Farkas

2012

Biol Therapy

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Hereditary angioedema resulting from the deficiency of the C1 inhibitor (HAE-C1-INH) is a rare, but potentially life-threatening disorder characterized by paroxysmal episodes of subcutaneous or submucosal edema. Early diagnosis is essential. Management is aimed at the prompt elimination of full-fledged attacks, as well as at the prevention of edematous episodes. The most straightforward means for therapy is supplementation with the deficient C1-INH protein. Placebo-controlled and open clinical studies have established that nanofiltered, human C1-INH concentrate, Cinryze® (ViroPharma Inc., Exton, PA, USA) (C1-INHCi), administered in 1,000 U doses is an effective and safe remedy for edematous episodes of HAE-C1-INH, regardless of the localization of the attack. Clinical manifestations rapidly improve and then resolve completely following treatment with this medicinal product. Additionally, C1-INHCi is also appropriate for pre-procedural or for routine prophylaxis. The administration of 1,000 U C1-INHCi before the (dental, surgical, or interventional diagnostic) procedure reduced the incidence of edematous episodes compared with placebo, and this reduction proved significant during routine prophylaxis with the administration of this dose every 3–4 days. Relapses did not occur, and repeated dosing had no influence on the efficacy of the preparation. Patients also tolerated treatment with C1-INHCi well. The safety of this preparation was confirmed by the absence of viral transmission as well as by the lack of antibody formation against C1-INH during treatment. Nowadays, C1-INHCi for intravenous use is the only medicinal product indicated both for the prevention and management of edematous attacks.

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Safety and Efficacy of Nanofiltered C1 Esterase Inhibitor (Human) (Cinryze™) for the Treatment of Laryngeal Attacks in Subjects with Hereditary Angioedema (HAE)

Cited by 2 publications

References 0 publications

Nanofiltered Human C1 Inhibitor Concentrate (Cinryze®)

Nanofiltered Human C1 Inhibitor Concentrate (Cinryze®)

Human Plasma-Derived, Nanofiltered, C1-Inhibitor Concentrate (Cinryze®), a Novel Therapeutic Alternative for the Management of Hereditary Angioedema Resulting from C1-Inhibitor Deficiency

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