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https://www.readbyqxmd.com/read/28412283/complement-factor-c4-activation-in-patients-with-hereditary-angioedema
#1
Anne Aabom, Anette Bygum, Claus Koch
OBJECTIVES: Low complement factor C4 is usually considered a valuable screening tool for patients with the potentially life-threatening Hereditary Angioedema with C1-inhibitor (C1-INH) deficiency (C1-INH-HAE). However, there are patients with C1-INH-HAE presenting with normal C4 levels. This means, that C1-INH-HAE may potentially be overlooked, if screening is performed only by measurement of C4. It has been suggested that measurement of C4 activation products is better suited to avoid false negative results...
April 12, 2017: Clinical Biochemistry
https://www.readbyqxmd.com/read/28381322/efficacy-and-safety-of-an-intravenous-c1-inhibitor-concentrate-for-long-term-prophylaxis-in-hereditary-angioedema
#2
Timothy Craig, Ralph Shapiro, Arthur Vegh, James W Baker, Jonathan A Bernstein, Paula Busse, Markus Magerl, Inmaculada Martinez-Saguer, Marc A Riedl, William Lumry, Debora Williams-Herman, Jonathan Edelman, Henrike Feuersenger, Thomas Machnig, Mikhail Rojavin
BACKGROUND: The plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH) is approved in the United States as an intravenous (IV) on-demand treatment for hereditary angioedema (HAE) attacks, and, in Europe, as on demand and short-term prophylaxis. OBJECTIVE: This analysis evaluated Berinert Patient Registry data regarding IV pnfC1-INH used as long-term prophylaxis (LTP). METHODS: The international registry (2010-2014) collected prospective and retrospective usage, dosing, and safety data on individuals who used pnfC1-INH for any reason...
March 1, 2017: Allergy & Rhinology
https://www.readbyqxmd.com/read/28366476/patient-characteristics-and-individualization-of-biologic-therapy
#3
REVIEW
Steven Draikiwicz, John Oppenheimer
Progress in the understanding of disease processes has provided additional therapeutic targets, best exemplified by the increasing role of biologics in the clinical armamentarium. This article provides a focused review of current treatment paradigms and pathophysiology for asthma, atopic dermatitis, urticaria, as well as C1 inhibitor deficiency. It elucidates the populations in which biologics were studied for the aforementioned disease states, emphasizing characteristics to consider when selecting therapy...
May 2017: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/28328347/prevention-of-hereditary-angioedema-attacks-with-a-subcutaneous-c1-inhibitor
#4
Hilary Longhurst, Marco Cicardi, Timothy Craig, Konrad Bork, Clive Grattan, James Baker, Huamin H Li, Avner Reshef, James Bonner, Jonathan A Bernstein, John Anderson, William R Lumry, Henriette Farkas, Constance H Katelaris, Gordon L Sussman, Joshua Jacobs, Marc Riedl, Michael E Manning, Jacques Hebert, Paul K Keith, Shmuel Kivity, Sergio Neri, Donald S Levy, Maria L Baeza, Robert Nathan, Lawrence B Schwartz, Teresa Caballero, William Yang, Ioana Crisan, María D Hernandez, Iftikhar Hussain, Michael Tarzi, Bruce Ritchie, Pavlina Králíčková, Mar Guilarte, Syed M Rehman, Aleena Banerji, Richard G Gower, Debra Bensen-Kennedy, Jonathan Edelman, Henrike Feuersenger, John-Philip Lawo, Thomas Machnig, Dipti Pawaskar, Ingo Pragst, Bruce L Zuraw
Background Hereditary angioedema is a disabling, potentially fatal condition caused by deficiency (type I) or dysfunction (type II) of the C1 inhibitor protein. In a phase 2 trial, the use of CSL830, a nanofiltered C1 inhibitor preparation that is suitable for subcutaneous injection, resulted in functional levels of C1 inhibitor activity that would be expected to provide effective prophylaxis of attacks. Methods We conducted an international, prospective, multicenter, randomized, double-blind, placebo-controlled, dose-ranging, phase 3 trial to evaluate the efficacy and safety of self-administered subcutaneous CSL830 in patients with type I or type II hereditary angioedema who had had four or more attacks in a consecutive 2-month period within 3 months before screening...
March 23, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28302171/clinical-characteristics-and-real-life-diagnostic-approaches-in-all-danish-children-with-hereditary-angioedema
#5
Anne Aabom, Klaus E Andersen, Christina Fagerberg, Niels Fisker, Marianne A Jakobsen, Anette Bygum
BACKGROUND: With a potentially early onset, hereditary angioedema (HAE) requires special knowledge also in infancy and early childhood. In children from families with HAE, the diagnosis should be confirmed or refuted early, which can be difficult. Studies of childhood HAE and the diagnostic approaches are limited. Our aim was to investigate the entire Danish cohort of children with HAE and non-HAE children of HAE patients for diagnostic approaches and clinical characteristics. RESULTS: We included 41 children: 22 with HAE and 19 non-HAE...
March 16, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28289183/c1-inhibitor-decreases-the-release-of-vasculitis-like-chemotactic-endothelial-microvesicles
#6
Maria Mossberg, Anne-Lie Ståhl, Robin Kahn, Ann-Charlotte Kristoffersson, Ramesh Tati, Caroline Heijl, Mårten Segelmark, L M Fredrik Leeb-Lundberg, Diana Karpman
The kinin system is activated during vasculitis and may contribute to chronic inflammation. C1-inhibitor is the main inhibitor of the kinin system. In this study, we investigated the presence of the kinin B1 receptor on endothelial microvesicles and its contribution to the inflammatory process. Compared with controls (n=15), patients with acute vasculitis (n=12) had markedly higher levels of circulating endothelial microvesicles, identified by flow cytometry analysis, and significantly more microvesicles that were positive for the kinin B1 receptor (P<0...
March 13, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28286522/assessment-of-105-patients-with-angiotensin-converting-enzyme-inhibitor-induced-angioedema
#7
Eva Rye Rasmussen, Christian von Buchwald, Mia Wadelius, Sumangali Chandra Prasad, Shailajah Kamaleswaran, Kawa Khaled Ajgeiy, Georg Authried, Kristine Appel U Pallesen, Anette Bygum
Objective. To asses a cohort of 105 consecutive patients with angiotensin converting enzyme-inhibitor induced angioedema with regard to demographics, risk factors, family history of angioedema, hospitalization, airway management, outcome, and use of diagnostic codes used for the condition. Study Design. Cohort study. Methods. This was a retrospective cohort study of 105 patients with angiotensin converting enzyme-inhibitor induced angioedema in the period 1995-2014. Results. The cohort consisted of 67 females and 38 males (F : M ratio 1...
2017: International Journal of Otolaryngology
https://www.readbyqxmd.com/read/28286153/safety-of-c1-inhibitor-concentrate-use-for-hereditary-angioedema-in-pediatric-patients
#8
Paula Busse, James Baker, Inmaculada Martinez-Saguer, Jonathan A Bernstein, Timothy Craig, Markus Magerl, Marc Riedl, Ralph Shapiro, Michael Frank, William Lumry, Jeffrey Rosch, Jonathan Edelman, Debora Williams-Herman, Henrike Feuersenger, Mikhail Rojavin
No abstract text is available yet for this article.
March 9, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28284781/diagnosis-course-and-management-of-angioedema-in-patients-with-acquired-c1-inhibitor-deficiency
#9
Andrea Zanichelli, Giulia Maria Azin, Maddalena Alessandra Wu, Chiara Suffritti, Lorena Maggioni, Sonia Caccia, Francesca Perego, Romualdo Vacchini, Marco Cicardi
BACKGROUND: Acquired angioedema due to C1-inhibitor deficiency (C1-INH-AAE) is a rare disease with no prevalence data or approved therapies. OBJECTIVE: To report data on patients with C1-INH-AAE followed at Angioedema Center, Milan (from 1976 to 2015). METHODS: Diagnostic criteria included history of recurrent angioedema without wheals; decreased C1-INH antigen levels and/or functional activity of C1-INH and C4 antigen less than 50% of normal; late symptom onset (>40 years); no family history of angioedema and C1-INH deficiency...
March 8, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28283277/anabolic-androgen-use-in-the-management-of-hereditary-angioedema-not-so-cheap-after-all
#10
Kevin Y Tse, Bruce L Zuraw, Qiaoling Chen, Sandra C Christiansen
BACKGROUND: Hereditary angioedema due to C1 inhibitor deficiency (HAE) is a rare, life-threatening disease that imposes a significant burden on affected patients. 17α-alkylated androgens (anabolic androgens) decrease attack frequency and severity but carry the risk of potentially serious dose-related adverse effects. Despite the emergence of targeted therapies for HAE, continued anabolic androgen use has been driven in part by their low cost. OBJECTIVE: To examine the hidden cost of anabolic androgen use related to the risk of developing non-HAE comorbidities...
April 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28258590/health-related-quality-of-life-among-children-with-hereditary-angioedema
#11
Batya Engel-Yeger, Henriette Farkas, Shmuel Kivity, Nóra Veszeli, Kinga Viktória Kőhalmi, Aharon Kesssel
BACKGROUND: The clinical expressions of hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) and its related burden may negatively affect patient quality of life. This study aims to assess health-related quality of life (HRQoL) in children with C1-INH-HAE. METHODS: Children (N=98: 34 C1-INH-HAE patients, 64 healthy controls) aged 3-18 years were recruited in Israel and Hungary. All individuals completed a demographic questionnaire, a disease activity and site questionnaire, and the Pediatric Quality of Life Inventory (PedsQL(™) ) 4...
March 4, 2017: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/28251901/hereditary-angioedema-with-normal-c1-inhibitor-clinical-characteristics-and-treatment-response-with-plasma-derived-human-c1-inhibitor-concentrate-berinert-%C3%A2-in-a-french-cohort
#12
Laurence Bouillet, Isabelle Boccon-Gibod, Anne Gompel, Bernard Floccard, Ludovic Martin, Claire Blanchard-Delaunay, David Launay, Olivier Fain
Hereditary angioedema (HAE) is a rare genetic disorder characterised by episodes of swelling without urticaria. Berinert® (CSL Behring) is a plasma-derived human C1 inhibitor (C1-INH) concentrate, approved for the treatment of HAE with C1-INH deficiency (C1-INH-HAE), however, it is often used off-label in Europe to treat HAE with normal C1-INH. OBJECTIVES: To report the clinical characteristics of patients with HAE with normal C1-INH (with F12 gene mutation; FXII-HAE) or of unknown origin (U-HAE), and their response to Berinert®...
March 1, 2017: European Journal of Dermatology: EJD
https://www.readbyqxmd.com/read/28250922/hereditary-angioedema-with-normal-c1-inhibitor-in-a-french-cohort-clinical-characteristics-and-response-to-treatment-with-icatibant
#13
Laurence Bouillet, Isabelle Boccon-Gibod, David Launay, Anne Gompel, Gisele Kanny, Vincent Fabien, Oliver Fain
INTRODUCTION: The clinical characteristics and icatibant-treatment outcomes of patients with hereditary angioedema with normal C1 inhibitor (HAE-nC1 INH) are limited. METHODS: We retrospectively analyzed data from French HAE patients enrolled in the Icatibant Outcome Survey registry (from July 2009 to September 2013) to compare disease characteristics and the effectiveness and safety of acute icatibant-treated angioedema attacks in patients with HAE-nC1 INH, HAE with C1 INH deficiency (type I), or dysfunction (type II)...
March 2017: Immunity, Inflammation and Disease
https://www.readbyqxmd.com/read/28225674/inhibiting-plasma-kallikrein-for-hereditary-angioedema-prophylaxis
#14
RANDOMIZED CONTROLLED TRIAL
Aleena Banerji, Paula Busse, Mustafa Shennak, William Lumry, Mark Davis-Lorton, Henry J Wedner, Joshua Jacobs, James Baker, Jonathan A Bernstein, Richard Lockey, H Henry Li, Timothy Craig, Marco Cicardi, Marc Riedl, Ahmad Al-Ghazawi, Carolyn Soo, Ryan Iarrobino, Daniel J Sexton, Christopher TenHoor, Jon A Kenniston, Ryan Faucette, J Gordon Still, Harvey Kushner, Robert Mensah, Chris Stevens, Joseph C Biedenkapp, Yung Chyung, Burt Adelman
Background Hereditary angioedema with C1 inhibitor deficiency is characterized by recurrent, unpredictable swelling episodes caused by uncontrolled plasma kallikrein generation and excessive bradykinin release resulting from cleavage of high-molecular-weight kininogen. Lanadelumab (DX-2930) is a new kallikrein inhibitor with the potential for prophylactic treatment of hereditary angioedema with C1 inhibitor deficiency. Methods We conducted a phase 1b, multicenter, double-blind, placebo-controlled, multiple-ascending-dose trial...
February 23, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/28222436/recurrent-angioedema-occurrence-features-and-concomitant-diseases-in-an-italian-single-center-study
#15
Paola Triggianese, Maria Domenica Guarino, Chiara Pellicano, Mauro Borzi, Elisabetta Greco, Stella Modica, Caterina De Carolis, Roberto Perricone
BACKGROUND: Angioedema (AE) is a potentially life-threatening condition with hereditary (HAE), acquired (AAE), or iatrogenic causes. A careful workup allows for the identification of the etiology of attacks and the appropriate management. In this cohort study, based on a clinical practice setting, we aimed at investigating clinical and laboratory findings concerning different features of patients with recurrent AE who were referred to a single, tertiary-level center for HAE. METHODS: Clinical and laboratory data of patients fulfilling the criteria for C1-inhibitor-deficient HAE (C1-INH-HAE), C1-INH-AAE, angiotensin-converting enzyme inhibitor-related AE (ACEI-RA), and idiopathic AAE (I-AAE) were evaluated...
2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28222330/hereditary-c1-inhibitor-deficiency-is-associated-with-high-spontaneous-amidase-activity
#16
Delphine Charignon, Arije Ghannam, Denise Ponard, Christian Drouet
BACKGROUND: Angioedema diagnosis classically targets the complement system (via C1 inhibitor (C1Inh) function and antigenic C4 level) and contact phase activation (via amidase activity). Bradykinin is responsible for angioedema attacks and is produced from contact phase activation secondary to failed C1Inh control. OBJECTIVE: We aimed to compare the diagnostic performances of spontaneous amidase activity and antigenic C4 level in C1Inh hereditary angioedema (C1Inh-HAE) patients...
February 18, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28194776/mutational-spectrum-of-the-serping1-gene-in-swiss-patients-with-hereditary-angioedema
#17
Urs C Steiner, Melanie Keller, Pirmin Schmid, Sven Cichon, Walter A Wuillemin
Hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disease caused by mutations in the C1 inhibitor gene SERPING1. Phenotype and clinical features of the disease are very heterogeneous, varying even in the same family. Compared to HAE cohorts in other countries the genetic background of the Swiss HAE patients has not been elucidated yet. In the present study we investigated the mutational spectrum of the SERPING1 gene in nineteen patients of nine unrelated Swiss families...
February 14, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28178173/screening-for-hereditary-angioedema-hae-at-13-emergency-centers-in-osaka-japan-a-prospective-observational-study
#18
MULTICENTER STUDY
Tomoya Hirose, Futoshi Kimbara, Masahiro Shinozaki, Yasuaki Mizushima, Hidehiko Yamamoto, Masashi Kishi, Takeyuki Kiguchi, Shigeru Shiono, Mitsuhiro Noborio, Akihiro Fuke, Hiroshi Akimoto, Takaaki Kimura, Shinichiro Kaga, Takahiko Horiuchi, Takeshi Shimazu
Hereditary angioedema (HAE) with deficiency of C1 inhibitor (C1-INH) is an autosomal-dominant disease characterized by recurrent episodes of potentially life-threatening angioedema. The objective is to study the incidence of HAE among patients who visit the emergency department.This was a 3-year prospective observational screening study involving 13 urban tertiary emergency centers in Osaka prefecture, Japan. Patients were included if they met the following criteria: unexplained edema of the body, upper airway obstruction accompanied by edema, anaphylaxis, acute abdomen with intestinal edema (including ileus and acute pancreatitis), or asthma attack...
February 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28151501/-angioedema-and-the-role-of-bradykinins-new-treatments-and-implications-in-patients-with-heart-failure
#19
Marta Mansi, Maddalena Alessandra Wu, Andrea Zanichelli, Marco Cicardi
The definition of angioedema is an edema of subcutaneous and submucosal tissues due to increased vascular permeability and fluid extravasation. It can affect different areas, including extremities, genitals, upper airways and intestinal mucosa. The symptoms are disabling and this condition can be fatal if it involves the larynx. We can distinguish different forms of angioedema (hereditary and acquired) with different pathogenetic mechanisms, therefore responding to different treatments. Bradykinin-mediated angioedema (such as hereditary angioedema due to C1-inhibitor deficiency) does not respond to the standard therapy used for histamine-mediated angioedema...
December 2016: Giornale Italiano di Cardiologia
https://www.readbyqxmd.com/read/28093999/health-related-quality-of-life-with-hereditary-angioedema-following-prophylaxis-with-subcutaneous-c1-inhibitor-with-recombinant-hyaluronidase
#20
RANDOMIZED CONTROLLED TRIAL
Karsten Weller, Marcus Maurer, Moshe Fridman, Dylan Supina, Jennifer Schranz, Markus Magerl
BACKGROUND: To estimate health-related quality-of-life changes in patients with hereditary angioedema due to C1-inhibitor (C1-INH) deficiency who received subcutaneous C1-INH with recombinant hyaluronidase (rHuPH20) for attack prophylaxis in a randomized, double-blind, dose-ranging, cross-over study. METHODS: Patients with type I/II hereditary angioedema received 1000 U of C1-INH with 24,000 U of rHuPH20 or 2000 U of C1-INH with 48,000 U of rHuPH20 every 3-4 days for 8 weeks and then crossed over for another 8-week period...
March 16, 2017: Allergy and Asthma Proceedings:
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