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https://www.readbyqxmd.com/read/28473832/how-escherichia-coli-circumvent-complement-mediated-killing
#1
REVIEW
Afonso G Abreu, Angela S Barbosa
Complement is a crucial arm of the innate immune response against invading bacterial pathogens, and one of its main functions is to recognize and destroy target cells. Similar to other pathogens, Escherichia coli has evolved mechanisms to overcome complement activation. It is well known that capsular polysaccharide may confer resistance to complement-mediated killing and phagocytosis, being one of the strategies adopted by this bacterium to survive in serum. In addition, proteases produced by E. coli have been shown to downregulate the complement system...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28462021/differential-transcript-profile-of-inhibitors-with-potential-anti-venom-role-in-the-liver-of-juvenile-and-adult-bothrops-jararaca-snake
#2
Cícera Maria Gomes, Karen de Morais-Zani, Stephen Lu, Diego de Souza Buarque, Glória Regina Cardoso Braz, Kathleen Fernandes Grego, Aparecida Sadae Tanaka, Anita Mitico Tanaka-Azevedo
BACKGROUND: Snakes belonging to the Bothrops genus are vastly distributed in Central and South America and are responsible for most cases of reported snake bites in Latin America. The clinical manifestations of the envenomation caused by this genus are due to three major activities-proteolytic, hemorrhagic and coagulant-mediated by metalloproteinases, serine proteinases, phospholipases A2 and other toxic compounds present in snake venom. Interestingly, it was observed that snakes are resistant to the toxic effects of its own and other snake's venoms...
2017: PeerJ
https://www.readbyqxmd.com/read/28453344/pediatric-hereditary-angioedema-as-a-cause-of-acute-compartment-syndrome-of-the-hand-and-forearm-a-case-report
#3
Chelsea Venditto, Zachary Jager, John LoGiudice, Hani Matloub
BACKGROUND: Compartment syndrome of the upper extremity is a surgical emergency that, when left untreated, can have dire consequences. Its causes are numerous, one of which is the uncommon entity hereditary angioedema, an autosomal dominant disease resulting in edema in a variety of potential locations, including the extremities. This is only the second time hereditary angioedema has been mentioned in the literature as a cause of compartment syndrome. METHODS: We present a case of hereditary angioedema leading to hand and forearm compartment syndrome in a 13-year-old pediatric patient...
May 2017: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/28452302/subcutaneous-administration-of-human-c1-inhibitor-with-recombinant-human-hyaluronidase-in-patients-with-hereditary-angioedema
#4
Marc A Riedl, William R Lumry, H Henry Li, Aleena Banerji, Jonathan A Bernstein, Murat Baş, Janne Björkander, Markus Magerl, Marcus Maurer, Kevin Rockich, Hongzi Chen, Jennifer Schranz
BACKGROUND: The currently approved method of C1 inhibitor (C1 INH) administration for patients with hereditary angioedema with C1 INH deficiency (HAE) is by intravenous injection. A C1 INH subcutaneous formulation may provide an attractive mode of administration for some patients. OBJECTIVE: To evaluate efficacy and safety of two doses of subcutaneous, plasma-derived C1 INH with the dispersing agent, recombinant human hyaluronidase (rHuPH20) to prevent angioedema attacks in patients with HAE...
November 1, 2016: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28444979/false-negative-single-antigen-bead-assay-is-it-always-an-effect-of-prozone
#5
Dharmendra Jain, Jui Choudhuri, Rajni Chauhan, Pranav Dorwal, Deepak Sharma, Aseem K Tiwari, Vimarsh Raina
BACKGROUND: Bead based flow cytometry and Luminex play a major role in identification of alloantibodies in renal transplant work-up. Strong sensitization events may lead to prozone phenomenon that can affect single antigen bead (SAB) assay and result in false negativity. However, this can also be due to high titer of other blocking antibodies. While methods like, heat inactivation, C1 inhibitor, Ethylene diamine tetra-acetic-acid and Dithio threitol treatment can remove interfering antibodies of complement and IgM, these methods are not optimal if false negativity is due to prozone effect, which is high titer of antibodies alone...
April 26, 2017: Journal of Clinical Laboratory Analysis
https://www.readbyqxmd.com/read/28444847/complement-dependence-of-murine-costimulatory-blockade-resistant-cellular-cardiac-allograft-rejection
#6
Nicholas Chun, Robert L Fairchild, Yansui Li, Jinhua Liu, Ming Zhang, William M Baldwin, Peter S Heeger
Building upon studies showing that ischemia/reperfusion-(IR)-injury is complement-dependent, we tested links among complement activation, transplant associated ischemia-reperfusion injury, and murine cardiac allograft rejection. We transplanted BALB/c hearts subjected to 8h cold ischemic storage (CIS) into CTLA4Ig-treated WT or c3(-/-) B6 recipients. Whereas allografts subjected to 8h CIS rejected in WT recipients with a median survival time (MST) of 37d, identically treated hearts survived >60d in c3(-/-) mice (p<0...
April 26, 2017: American Journal of Transplantation
https://www.readbyqxmd.com/read/28441992/safety-of-a-c1-inhibitor-concentrate-in-pregnant-women-with-hereditary-angioedema
#7
James Fox, Arthur B Vegh, Inmaculada Martinez-Saguer, Walter A Wuillemin, Jonathan Edelman, Debora Williams-Herman, Mikhail Rojavin, Tanja Rosenberg
BACKGROUND: Increased estrogen levels during pregnancy can exacerbate hereditary angioedema (HAE), yet disease and treatment ramifications remain poorly studied in pregnant women. OBJECTIVE: Data from the international Berinert Patient Registry were used to evaluate outcomes of pregnancies exposed to plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH) during routine HAE management. METHODS: This observational registry, conducted between 2010 and 2014 at 30 U...
May 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28412283/complement-factor-c4-activation-in-patients-with-hereditary-angioedema
#8
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
#9
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
#10
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
#11
RANDOMIZED CONTROLLED TRIAL
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
Batya Engel-Yeger, Henriette Farkas, Shmuel Kivity, Nóra Veszeli, Kinga Viktória Kőhalmi, Aharon Kessel
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 aimed 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
#19
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
#20
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
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