Recombinant Human C1-Esterase Inhibitor to Treat Acute Hereditary Angioedema Attacks in Adolescents

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منابع مشابه

85 Clinical Efficacy of Recombinant Human C1 Inhibitor in Patients with Acute Hereditary Angioedema Attacks

84 Immuno-Safety of Recombinant Human C1 Inhibitor in Patients With Hereditary Angioedema: An Integrated Analysis Erik Hack, MD, PhD, Anurag Relan, MD, Leonard Kaufman, PhD, and Rienk Pijpstra, MD. Department of Immunology, Dermatology/Allergology & Reumatology, University Medical Center, Utrecht, Utrecht, Netherlands; Pharming Technologies BV, Leiden, Netherlands; Veeda Clinical Research, Brus...

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Recombinant Human C1 Esterase Inhibitor in the Management of Hereditary Angioedema

Hereditary angioedema (HAE), a rare autosomal dominant genetic disorder, is caused by a deficiency in functional C1 esterase inhibitor (C1-INH). This potentially life-threatening condition manifests as recurrent attacks of subcutaneous and submucosal swelling of the skin, gastrointestinal tract and larynx. The management of HAE includes treatment of acute episodes, short-term prophylaxis in pre...

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[Hereditary angioedema due to C1-esterase inhibitor deficiency].

The paper presents a case of family oedema by shortage of C1-esterase inhibitor, characterized by appearance at an early age (8 months), participation in some attacks of nasal mucous membrane, with nasal obstruction and sinusal manifestations and also the abnormally long period, up to 9 days, of some localized oedemas. The crises were absent during pregnancy. The study of four generations of th...

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Self-administration of intravenous C1 esterase inhibitor in hereditary angioedema.

somal dominant disorder that results in episodes of acute edema in various organs, including the gastrointestinal tract, skin and larynx. It is estimated to affect about 1 in 50 000–100 000 people. The symptoms, including abdominal pain, laryngeal edema and subcutaneous edema, usually begin in childhood and persist throughout life with unpredictable severity. Hereditary angioedema is classified...

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Recent developments in the treatment of acute abdominal and facial attacks of hereditary angioedema: focus on human C1 esterase inhibitor

Hereditary angioedema (HAE) is a potentially fatal genetic disorder typified by a deficiency (type I) or dysfunction (type II) of the C1-inhibitor (C1-INH) and characterized by swelling of the extremities, face, trunk, abdominal viscera, and upper airway. Type III is normal estrogen-sensitive C1-INH HAE. Bradykinin, the main mediator of HAE, binds to endothelial B2 receptors, increasing vascula...

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ژورنال

عنوان ژورنال: The Journal of Allergy and Clinical Immunology: In Practice

سال: 2017

ISSN: 2213-2198

DOI: 10.1016/j.jaip.2016.11.005