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2 results on '"Leibovich I"'

1. Hereditary angioedema: new hopes for an orphan disease.

Author

Reshef A, Leibovich I, and Goren A

Subjects
Abdominal Pain drug therapy, Abdominal Pain etiology, Angioedemas, Hereditary classification, Angioedemas, Hereditary physiopathology, Bradykinin adverse effects, Bradykinin pharmacology, Bradykinin therapeutic use, Clinical Trials as Topic, Double-Blind Method, Female, Humans, Kallikreins antagonists & inhibitors, Male, Multicenter Studies as Topic, Nausea drug therapy, Nausea etiology, Peptides pharmacology, Treatment Outcome, Angioedemas, Hereditary drug therapy, Bradykinin analogs & derivatives, Bradykinin Receptor Antagonists, Peptides therapeutic use
Abstract

Hereditary angioedema is a rare genetic disorder, manifested by recurrent edema leading to disfigurement, organ dysfunction and life-threatening respiratory impairment that may become fatal. The hallmark of HAE is C1 esterase inhibitor deficiency, but recent evidence points at bradykinin as the main mediator that causes hyperpermeability of small vasculature, leading to accumulation of edema fluid. Current therapeutic options for HAE are limited, and consist of drugs, replacement therapy, and supportive treatment. In view of many disadvantages of the current therapeutic modalities, new approaches to the treatment of HAE are now being offered. This review summarizes our experience with a new line of medications developed for the treatment of acute exacerbations and prophylaxis of HAE--icatibant: bradykinin receptor antagonist, ecallantide: kallikrein inhibitor, and two C1 INH preparations: Berinert-P, human plasma-derived concentrate, and Rhucin: novel recombinant C1-INH produced in transgenic rabbits. Preliminary results of these studies are encouraging and may bring new hope to the patients with this distressing condition. The exact number of HAE patients in Israel is unknown and because patients are treated individually and comprehensive laboratory assessment is partial, many cases might be missed or not treated according to accepted guidelines. We offer a new specialty center for HAE patients, addressing the medical and psychosocial needs of patients and their families.

Published
2008

2. Evidence-based recommendations for the therapeutic management of angioedema owing to hereditary C1 inhibitor deficiency: consensus report of an International Working Group

Author

Cicardi, M, Bork, K, Caballero, T, Craig, T, Hh, L, Longhurst, H, Reshef, A, Zuraw, B, Aberer, W, Aygören Pürsün, E, Banerji, A, Bjorkander, J, Boccon Gibod, I, Bouillet, L, Grenoble, F, Bova, M, Bowen, T, Calgary, C, Branco, F, Bygum, A, Cancian, M, Castel Branco, M, de Carolis, C, Mihály, E, Fabiani, J, Farkas, H, Gompels, M, Gower, R, Groffik, A, Grumach, A, Guillarte, M, Hack, E, Hernandez, L, Kaplan, A, Lara, A, Leibovich, I, Li, H, Lock, B, Lumry, W, Malbran, A, Martinez Saguer, I, Matta, C, Maurer, M, Moldovan, D, Montinaro, V, Nieto, S, Nordenfelt, P, Obtulovicz, K, Perricone, R, Prior, N, Riedl, M, Rodrigues do, V, Savoca, C, Spaeth, P, Staubach Renz, P, Stobiecki, M, Triggiani, M, Vacchini, R, Varga, L, Zanichelli, A, Zarchi, K, Zeerleder, S, and Zingale, L

Subjects
C1 inhibitor, Immunology, Angioedemas, Hereditary, Angioedemas, Complement C1 Inactivator Proteins, bradykinin receptor antagonist, Bradykinin, hereditary angioedema, Settore MED/16 - Reumatologia, kallikrein inhibitor, Hereditary, consensus document, Bradykinin Receptor Antagonists, Complement C1 Inhibitor Protein, Humans, Kallikreins, Peptides, Immunology and Allergy
Abstract

Angioedema owing to hereditary deficiency of C1 inhibitor (HAE) is a rare, life-threatening, disabling disease. In the last 2 years, the results of well-designed and controlled trials with existing and new therapies for this condition have been published, and new treatments reached the market. Current guidelines for the treatment for HAE were released before the new trials and before the new treatments became available and were essentially based on observational studies and expert opinion. To provide evidence-based HAE treatment guidelines supported by the new studies, a conference was held in Gargnano del Garda, Italy, from September 26 to 29, 2010. The meeting hosted 58 experienced HAE expert physicians, representatives of pharmaceutical companies and representatives of HAE patients' associations. Here, we report the topics discussed during the meeting and evidence-based consensus about management approaches for HAE in adult/adolescent patients.

Published
2012

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