Recombinant human C1 esterase inhibitor as short-term prophylaxis in patients with hereditary angioedema
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چکیده
منابع مشابه
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...
متن کاملCinryze, a human plasma-derived c1 esterase inhibitor for prophylaxis of hereditary angioedema.
Vol. 34 No. 6 • June 2009 • P&T® 293 INTRODUCTION Hereditary angioedema (HAE) is a rare genetic disorder resulting from an inherited deficiency or dysfunction of the C1 inhibitor (C1-INH), a molecule that inhibits kallikrein and other serine proteinases. HAE is characterized by un predictable and recurrent attacks of inflammation affecting the hands, feet, face, abdomen, urogenital tract, and l...
متن کاملSelf-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...
متن کامل[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...
متن کامل84 Immuno-Safety of Recombinant Human C1 Inhibitor in Patients With Hereditary Angioedema: An Integrated Analysis
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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ژورنال
عنوان ژورنال: The Journal of Allergy and Clinical Immunology: In Practice
سال: 2020
ISSN: 2213-2198
DOI: 10.1016/j.jaip.2019.08.011