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https://www.readbyqxmd.com/read/28634542/angioedema-perioperative-management
#1
Andrew A Maynard, Christina F Burger, Joseph J Schlesinger
OBJECTIVE: To describe the perioperative management of a patient with acquired angioedema (AAE). METHODS: A 66-year-old Caucasian male presented from an outside hospital with a history of acquired angioedema and gastrointestinal stromal tumor-related intractable urticaria and mastocytosis. He was admitted for urgent laparoscopic partial gastrectomy, secondary to gastric outlet obstruction symptomatology. Previous combined attacks were characterized by a widespread rash, abdominal pain and respiratory distress resulting in hospitalization...
2017: SAGE open medical case reports
https://www.readbyqxmd.com/read/28601681/mutation-of-angiopoietin-1-gene-associates-with-a-new-type-of-hereditary-angioedema
#2
Valeria Bafunno, Davide Firinu, Maria D'Apolito, Giorgia Cordisco, Stefania Loffredo, Angelica Leccese, Maria Bova, Maria Pina Barca, Rosa Santacroce, Marco Cicardi, Stefano Del Giacco, Maurizio Margaglione
BACKGROUND: Hereditary angioedema (HAE) is a rare genetic disease usually due to mutation within the C1 inhibitor or the coagulation Factor XII gene. However, in a series of patients with HAE no causative variants have been described and the pathophysiology of the disease remains unknown (U-HAE). Identification of causative genes in U-HAE is valuable for understanding the cause of the disease. OBJECTIVE: We conducted genetic studies in Italian patients with U-HAE to identify novel causative genes...
June 7, 2017: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/28601641/treatment-effect-and-safety-of-icatibant-in-pediatric-patients-with-hereditary-angioedema
#3
Henriette Farkas, Avner Reshef, Werner Aberer, Teresa Caballero, Laura McCarthy, James Hao, Wolfram Nothaft, Jennifer Schranz, Jonathan A Bernstein, H Henry Li
BACKGROUND: Clinical manifestations of hereditary angioedema with C1 inhibitor deficiency (C1-INH-HAE) usually begin in childhood, often intensifying during puberty. Currently there are insufficient efficacy/safety data for HAE therapies in children and adolescents due to the small number of pediatric patients enrolled in studies. OBJECTIVE: The objective of this phase 3 study was to evaluate the efficacy/safety of a single subcutaneous dose of icatibant (0.4 mg/kg; maximum 30 mg) in pediatric patients with C1-INH-HAE...
June 7, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28595743/the-role-of-the-complement-system-in-hereditary-angioedema
#4
Dorottya Csuka, Nóra Veszeli, Lilian Varga, Zoltán Prohászka, Henriette Farkas
Hereditary angioedema (HAE) is a rare, but potentially life-threatening disorder, characterized by acute, recurring, and self-limiting edematous episodes of the face, extremities, trunk, genitals, upper airways, or the gastrointestinal tract. HAE may be caused by the deficiency of C1-inhibitor (C1-INH-HAE) but another type of the disease, hereditary angioedema with normal C1-INH function (nC1-INH-HAE) was also described. The patient population is quite heterogeneous as regards the location, frequency, and severity of edematous attacks, presenting large intra- and inter-individual variation...
June 5, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28577900/a-novel-prophylaxis-with-c1-inhibitor-concentrate-in-hereditary-angioedema-during-erythema-marginatum
#5
Kinga Viktória Kőhalmi, Nóra Veszeli, László Cervenak, Lilian Varga, Henriette Farkas
BACKGROUND: Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. The characteristic episodes of subcutaneous/submucosal edema formation may be preceded by erythema marginatum (EM) - the occurrence of a 'map-like' pattern on the skin. EM can occur as an isolated finding or accompanying a hereditary angiooedema (HAE) attack as well. Nevertheless, it is unknown whether a HAE attack can be prevented by the proper prophylactic treatment during EM...
May 31, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28539578/drug-induced-inhibition-of-angiotensin-converting-enzyme-and-dipeptidyl-peptidase-4-results-in-nearly-therapy-resistant-bradykinin-induced-angioedema-a-case-report
#6
Janina Hahn, Susanne Trainotti, Thomas K Hoffmann, Jens Greve
BACKGROUND Bradykinin is an underestimated mediator of angioedema. One subgroup of bradykinin induced angioedema is angioedema triggered by treatment with angiotensin converting enzyme (ACE) inhibitors. Due to its localization in the head and neck region and its unpredictable course, it is a possibly life-threatening condition. There is not an officially approved treatment for ACE inhibitor induced angioedema. CASE REPORT We present a case of an 83-year-old woman, who presented to our ENT department because of acute swelling of the tongue...
May 25, 2017: American Journal of Case Reports
https://www.readbyqxmd.com/read/28473832/how-escherichia-coli-circumvent-complement-mediated-killing
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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