نتایج جستجو برای: c1 esterase

تعداد نتایج: 23038  

Journal: :International archives of allergy and immunology 2012
Louanne M Tourangeau Anthony J Castaldo Donna K Davis James Koziol Sandra C Christiansen Bruce L Zuraw

BACKGROUND C1 inhibitor (C1INH) has recently been approved in the USA for the treatment of acute attacks in hereditary angioedema (HAE) patients. The literature suggests that treatment with C1INH is most effective when administered early in an attack. Home infusion of C1INH allows for the earliest possible intervention since patients can initiate therapy at the first sign of symptoms. METHODS...

2012
Henriette Farkas Lilian Varga

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 f...

Journal: :International archives of allergy and immunology 2015
Angelica Petraroli Veronica Squeglia Nadia Di Paola Alessandro Barbarino Maria Bova Rosanna Spanò Gianni Marone Massimo Triggiani

BACKGROUND Attacks of hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) are commonly treated in the emergency department. Self-administration is emerging as an effective treatment option. In this study, we assessed the impact of home therapy with plasma-derived C1 esterase inhibitor (pdC1-INH) concentrate on treatment outcomes and costs. METHODS This is an observational study in...

2015
Rafael Cicconi Arantes Débora Martins Ferreira Murilo Andrade Santana Faradiba Serpa Marina Moura Lopes Pereira Therezinha Ribeiro Moyses Fernanda Lugão Campinhos

Results Data from 51 patients, 29 (57%) females and 22 (43%) males, from 5 to 88 years old (mean: 32 years) was evaluated. Patients belonged to 7 families, 20 of them from the same family. The mean age of onset was 10 years and of diagnosis 26 years. Fifty (98%) patients were symptomatic, and 28 (55%) had experienced laryngeal edema. Deaths by laryngeal edema had occurred in 6 families. Crisis ...

Journal: :Allergy and asthma proceedings 2012
Bruce L Zuraw Konrad Bork Karen E Binkley Aleena Banerji Sandra C Christiansen Anthony Castaldo Allen Kaplan Marc Riedl Charles Kirkpatrick Markus Magerl Christian Drouet Marco Cicardi

A new form of hereditary angioedema (HAE) with normal C1 inhibitor (C1INH) was first described in 2000. The lack of clear diagnostic criteria, the heterogeneity among affected patients, and the varying names given to this disease have led to substantial confusion among both physicians and patients. This study was designed to bring more clarity to the diagnosis and potential treatment of HAE wi...

Journal: :Gut 1982
J T Whicher M P Barnes A Brown M J Cooper R Read G Walters R C Williamson

Serum levels of the complement proteins C3, C4, C1 inhibitor (C1 INH), factor I (C3b inactivator) and factor H (BIH) and plasma levels of cleavage products of C3 (C3c) and factor B were measured in 26 patients with acute pancreatitis. Breakdown of C3 occurred in 19 patients, as shown by a reduction in C3 level and the presence of C3c. C4 levels, however, did not fall and factor B breakdown prod...

2017
H. Farkas I. Martinez‐Saguer K. Bork T. Bowen T. Craig M. Frank A. E. Germenis A. S. Grumach A. Luczay L. Varga A. Zanichelli Werner Aberer Sladjana Andrejevic Emel Aygoeren‐Pürsün Alena Banerji Noemi‐Anna Bara Murat Bas Jonathan Bernstein Stephen Betschel Janne Björkander Isabelle Boccon‐Gibod Laurence Bouillet Maria Bova Henrik Halle Boysen Manuel Branco‐Ferreira Anette Bygum Teresa Caballero Mauro Cancian Anthony Castaldo Sandra Christiansen Marco Cicardi Christian Drouet Jose Fabiani Mark Gompels Maria Teresa Gonzalez‐Quevedo Jimmy Gooi Richard Gower Nihal Mete Gökmen Vesna Grivcheva‐Panovska Mar Guilarte Okan Gülbahar Erik Hack Roman Hakl György Harmat Miloš Jeseňák Stephen Jolles Allen Kaplan Connie Katelaris Mitja Kosnik Kinga Viktória Kőhalmi Iris Leibovich Marcel Levi Henry Li Hilary J. Longhurst William Lumry Markus Magerl Alejandro Malbran Ludovic Martin Marcus Maurer Enikő Mihály Dumitru Moldovan Mariana Murdjeva Imola Beatrix Nagy Erik W. Nielsen Sandra Nieto Patrik Nordenfelt Kristine Obtulowitzc Maria Pedrosa Grzegorz Porębski Nieves Prior Avner Reshef Marc A. Riedl Bernd Rosenkranz Peter Schmid‐Grendelmeier Spath Péter Matthaios Speletas Maria Staevska Marcin Stobiecki Massimo Triggiani Nóra Veszeli Walter Wuillemin Zhi Yu Xiang Beverley Yamamoto Bruce Zuraw

BACKGROUND The consensus documents published to date on hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) have focused on adult patients. Many of the previous recommendations have not been adapted to pediatric patients. We intended to produce consensus recommendations for the diagnosis and management of pediatric patients with C1-INH-HAE. METHODS During an expert panel meeting t...

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