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C1-inhibitor

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https://www.readbyqxmd.com/read/28093999/health-related-quality-of-life-with-hereditary-angioedema-following-prophylaxis-with-subcutaneous-c1-inhibitor-with-recombinant-hyaluronidase
#1
K Weller, M Maurer, M Fridman, D Supina, M Magerl, J Schranz
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...
January 16, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28069032/glucocorticoid-receptor-gene-polymorphisms-in-hereditary-angioedema-with-c1-inhibitor-deficiency
#2
Zsuzsanna Zotter, Zsolt Nagy, Attila Patócs, Dorottya Csuka, Nóra Veszeli, Kinga Viktória Kőhalmi, Henriette Farkas
BACKGROUND: Hereditary angioedema caused by C1-inhibitor deficiency (C1-INH-HAE) is a rare, autosomal dominant disorder. C1-INH-HAE is characterized by edema-formation, which may occur in response to stress. The individual's response to stress stimuli is partly genetically determined. Activation of the hypothalamic-pituitary-adrenal axis results in the release of cortisol. In turn, the secreted gluco- and mineralocorticoids affect the metabolism, as well as the cardiovascular and immune systems...
January 10, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28025012/catabolism-of-c1-inhibitor-influences-the-response-to-replacement-therapy-in-hereditary-angioedema
#3
Marco Cicardi, Andrea Zanichelli, Chiara Suffritti, Maddalena A Wu, Thomas Machnig, Annalisa De Silvestri, Mario Regazzi, Carmine Tinelli
Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) is a disabling and life-threatening disease for which plasma-derived C1 inhibitor (pdC1-INH) is an effective treatment. Poor responses to pdC1-INH are rare. The aim of this prospective study was to evaluate the pharmacokinetics and pharmacodynamics of C1 inhibitor (C1-INH) in a C1-INH-HAE patient with poor response to treatment to investigate the mechanism underlying poor response to pdC1-INH. Seventeen C1-INH-HAE patients with normal responses to treatment served as retrospective controls...
December 23, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27986601/identification-and-characterization-of-c1-inhibitor-in-nile-tilapia-oreochromis-niloticus-in-response-to-pathogenic-bacteria
#4
Mingmei Ding, Meng Chen, Xiaofang Zhong, Yuhong Wang, Shengli Fu, Xiaoxue Yin, Zheng Guo, Jianmin Ye
C1 inhibitor (C1INH) is a multi-functional serine protease inhibitor in plasmatic cascades, not only inactivating various proteases, but also regulating both complement and contact system activation. In this study, we described the identification and characterization of a C1INH ortholog from Nile tilapia (Oreochromis niloticus) at molecular, protein and cellular levels. The full-length cDNA of Oreochromis niloticus C1INH (OnC1INH) consisted of 1791 bp of nucleotide sequence encoding polypeptides of 596 amino acids...
December 13, 2016: Fish & Shellfish Immunology
https://www.readbyqxmd.com/read/27940765/management-of-children-with-hereditary-angioedema-due-to-c1-inhibitor-deficiency
#5
Michael M Frank, Bruce Zuraw, Aleena Banerji, Jonathan A Bernstein, Timothy Craig, Paula Busse, Sandra Christiansen, Marc Davis-Lorton, H Henry Li, William R Lumry, Marc Riedl
Hereditary angioedema (HAE) is a potentially life-threatening inherited disease characterized by attacks of skin swelling, severe abdominal pain, and upper airway swelling. Attacks typically begin in childhood, but the appropriate diagnosis is often missed. Attacks do not respond to epinephrine, antihistamines, or glucocorticoids. Recently, many effective drugs have been approved for treatment of adults with HAE, and the Medical Advisory Board of the HAE Patient's Association has developed and reported treatment recommendations for adults...
November 2016: Pediatrics
https://www.readbyqxmd.com/read/27936514/comparing-acquired-angioedema-with-hereditary-angioedema-type-i-ii-findings-from-the-icatibant-outcome-survey
#6
Hilary J Longhurst, Andrea Zanichelli, Teresa Caballero, Laurence Bouillet, Werner Aberer, Marcus Maurer, Olivier Fain, Vincent Fabien, Irmgard Andresen
Icatibant is approved for hereditary angioedema with C1 inhibitor deficiency type I/II (C1-INH-HAE type I/II) and has shown promise in treatment of angioedema due to acquired C1 inhibitor deficiency (C1-INH-AAE). Data from the Icatibant Outcome Survey was analyzed to evaluate effectiveness of icatibant in the treatment of patients with C1-INH-AAE and compare disease characteristics with those with C1-INH-HAE type I/II. Key medical history (including prior occurrence of attacks) was recorded upon IOS enrollment...
December 9, 2016: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/27931305/subcutaneous-administration-of-human-c1-inhibitor-with-recombinant-human-hyaluronidase-in-patients-with-hereditary-angioedema
#7
Marc A Riedl, William R Lumry, H Henry Li, Aleena Banerji, Jonathan A Bernstein, Murat Ba, Janne Bjrkander, 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 2016: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/27913306/effect-of-bradykinin-receptor-antagonism-on-ace-inhibitor-associated-angioedema
#8
Brittany T Straka, Claudia E Ramirez, James B Byrd, Elizabeth Stone, Alencia Woodard-Grice, Hui Nian, Chang Yu, Aleena Banerji, Nancy J Brown
BACKGROUND: The B2 receptor antagonist icatibant is approved for treatment of attacks of hereditary angioedema. Icatibant has been reported to decrease time-to-resolution of angiotensin-converting enzyme (ACE) inhibitor-associated angioedema in 1 study of European patients. OBJECTIVE: We sought to test the hypothesis that a bradykinin B2 receptor antagonist would shorten time-to-resolution from ACE inhibitor-associated angioedema. METHODS: Patients with ACE inhibitor-associated angioedema (defined as swelling of lips, tongue, pharynx, or face during ACE inhibitor use and no swelling in the absence of ACE inhibitor use) were enrolled at Vanderbilt University Medical Center from October 2007 through September 2015 and at Massachusetts General Hospital in 2012...
November 29, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27911318/the-correlation-between-inflammatory-biomarkers-and-polygenic-risk-score-in%C3%A2-alzheimer-s-disease
#9
Angharad R Morgan, Samuel Touchard, Caroline O'Hagan, Rebecca Sims, Elisa Majounie, Valentina Escott-Price, Lesley Jones, Julie Williams, B Paul Morgan
Plasma biomarkers to aid the early diagnosis of Alzheimer's disease (AD) or to monitor disease progression have long been sought and continue to be widely studied. Biomarkers that correlate with AD polygenic risk score, a measure of the polygenic architecture of the disease and highly predictive of AD status, would be excellent candidates. Therefore, we undertook a preliminary study to assess the association of plasma inflammatory biomarkers with an overall AD polygenic risk score as well as with an inflammation-specific AD polygenic risk score in a sample set of 93 AD cases...
November 26, 2016: Journal of Alzheimer's Disease: JAD
https://www.readbyqxmd.com/read/27905115/treatment-for-hereditary-angioedema-with-normal-c1-inh-and-specific-mutations-in-the-f12-gene-hae-fxii
#10
K Bork, K Wulff, G Witzke, J Hardt
Hereditary angioedema with normal C1 esterase inhibitor and mutations in the F12 gene (HAE-FXII) is associated with skin swellings, abdominal pain attacks, and the risk of asphyxiation due to upper airway obstruction. It occurs nearly exclusively in women. We report our experience treating HAE-FXII with discontinuation of potential trigger factors and drug therapies. The study included 72 patients with HAE-FXII. Potential triggers included estrogen-containing oral contraceptives (eOC), hormonal replacement therapy, or angiotensin-converting enzyme inhibitors...
November 6, 2016: Allergy
https://www.readbyqxmd.com/read/27878882/tamoxifen-may-cause-life-threatening-angioedema-attacks-in-patients-with-hereditary-angioedema
#11
LETTER
K Bork, K Wulff, G Witzke, S Rietz, J Hardt
No abstract text is available yet for this article.
November 23, 2016: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/27871580/c1-esterase-inhibitor-for-short-term-prophylaxis-in-a-patient-with-hereditary-angioedema-with-normal-c1-inhibitor-function
#12
Savio K H Yu, Jeannie Callum, Asim Alam
Hereditary angioedema with normal C1-esterase inhibitor (HAE-nC1INH) perioperative is a rare condition which could have potential disastrous ramifications for the anesthesiologist in the perioperative period. However, there is limited evidence and/or guidelines on the optimal way to manage these patients. We present the case of a patient with HAE-nC1INH who was successfully managed in the perioperative period with plasma derived C1-esterase inhibitor (pdC1INH). A 29-year-old woman with a diagnosis of HAE-nC1INH presented to the preoperative consultation in preparation for an upcoming total thyroidectomy...
December 2016: Journal of Clinical Anesthesia
https://www.readbyqxmd.com/read/27865714/short-term-prophylactic-use-of-c1-inhibitor-concentrate-in-hereditary-angioedema-findings-from-an-international-patient-registry
#13
Markus Magerl, Michael Frank, William Lumry, Jonathan Bernstein, Paula Busse, Timothy Craig, Inmaculada Martinez-Saguer, Marc A Riedl, Ralph Shapiro, Jonathan Edelman, Debora Williams-Herman, Daniel N Wood, Henrike Feuersenger, Mikhail Rojavin
No abstract text is available yet for this article.
January 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/27862254/hk2-proximal-tubule-epithelial-cells-synthesize-and-secrete-plasma-proteins-predominantly-through-the-apical-surface
#14
Ke-Wei Zhao, Elsa J Brochmann Murray, Samuel S Murray
Renal proximal tubule epithelial cells (PTECs) are known to reabsorb salts and small plasma proteins filtered through Bowman's capsule. Following acute kidney injury, PTECs assume some characteristics of hepatocytes in producing various plasma proteins. We now demonstrate that even at a resting state, a PTEC cell line, HK2 expresses mRNAs for and synthesizes and secretes plasma proteins in a complex with complement C3, an α2 -macroglobulin family chaperone, including albumin, transferrin, α1 -antitrypsin, α1 -antichymotrypsin, α2 -HS-glycoprotein, ceruloplasmin, haptoglobin, C1-inhibitor, secreted phosphoprotein-24, and insulin-like growth factor-1...
November 9, 2016: Journal of Cellular Biochemistry
https://www.readbyqxmd.com/read/27846429/effects-of-bothrops-atrox-venom-and-two-isolated-toxins-on-the-human-complement-system-modulation-of-pathways-and-generation-of-anaphylatoxins
#15
Danilo L Menaldo, Carolina P Bernardes, Anna L Jacob-Ferreira, Cíntia G Nogueira-Santos, Tania M Casare-Ogasawara, Luciana S Pereira-Crott, Suely V Sampaio
The complement system plays important biological roles, including the activation of inflammatory processes in response to the generation of proteolytic fragments of its components. Here we evaluated the effects of Bothrops atrox venom and two of its toxins (the P-I metalloprotease Batroxase and the acidic phospholipase A2 BatroxPLA2) on the human complement system, evaluating their effects on the classical (CP), lectin (LP) and alternative (AP) pathways, as well as on different complement components associated to the generation of anaphylatoxins...
December 2016: Molecular Immunology
https://www.readbyqxmd.com/read/27826093/cytokine-and-estrogen-stimulation-of-endothelial-cells-augments-activation-of-the-prekallikrein-high-molecular-weight-kininogen-complex-implications-for-hereditary-angioedema
#16
Kusumam Joseph, Baby G Tholanikunnel, Allen P Kaplan
BACKGROUND: When the prekallikrein-high molecular weight kininogen complex is bound to endothelial cells, prekallikrein is stoichiometrically converted to kallikrein because of release of heat shock protein-90 (Hsp90). Although bradykinin formation is typically initiated by factor XII autoactivation, it is also possible to activate factor XII either by kallikrein, thus formed, or by plasmin. OBJECTIVE: Because attacks of hereditary angioedema can be related to infection and/or exposure to estrogen, we questioned whether estrogen or cytokine stimulation of endothelial cells could augment release of Hsp90 and prekallikrein activation...
November 5, 2016: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/27818099/how-dextran-sulfate-affects-c1-inhibitor-activity-a-model-for-polysaccharide-potentiation
#17
Madelon Dijk, Jolande Holkers, Patrick Voskamp, Bruno M Giannetti, Willem-Jan Waterreus, Harrie A van Veen, Navraj S Pannu
C1-inhibitor is a key inhibitor of the complement and contact activation systems, and mutations in the protein can cause hereditary angioedema. Through an unknown mechanism, polysaccharides can increase C1-inhibitor activity against some of its target proteases. Here we present the crystal structures of the serine protease inhibitor (serpin) domain of active C1-inhibitor by itself and in complex with dextran sulfate. Unlike previously described interactions of serpins with polysaccharides, the structures and isothermal titration calorimetry experiments together reveal that dextran sulfate binds to C1-inhibitor's F1 helix with low affinity and does not invoke an allosteric change...
December 6, 2016: Structure
https://www.readbyqxmd.com/read/27810412/on-the-value-of-therapeutic-interventions-targeting-the-complement-system-in-acute-myocardial-infarction
#18
REVIEW
Reindert W Emmens, Diana Wouters, Sacha Zeerleder, S Marieke van Ham, Hans W M Niessen, Paul A J Krijnen
The complement system plays an important role in the inflammatory response subsequent to acute myocardial infarction (AMI). The aim of this study is to create a systematic overview of studies that have investigated therapeutic administration of complement inhibitors in both AMI animal models and human clinical trials. To enable extrapolation of observations from included animal studies toward post-AMI clinical trials, ex vivo studies on isolated hearts and proof-of-principle studies on inhibitor administration before experimental AMI induction were excluded...
October 14, 2016: Translational Research: the Journal of Laboratory and Clinical Medicine
https://www.readbyqxmd.com/read/27795218/-angioedema
#19
Luisa Holguín-Gómez, Luz Adriana Vásquez-Ochoa, Ricardo Cardona
Angioedema is defined as edema of the skin or mucosa, including the respiratory and the gastrointestinal mucosa, which is self-limiting, and in most cases is completely resolved in less than 72 hours. It occurs due to increased permeability of the mucosal and submucosal capillaries and postcapillary venules, with resulting plasma extravasation. There are different types of angioedema: histaminergic (which may be mediated by immunoglobulin E), hereditary, from acquired C1 inhibitor deficiency, from angiotensin converting enzyme inhibitor, bradykinin-mediated, and non-histaminergic idiopathic angioedema...
October 2016: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/27788882/hereditary-angioedema-with-f12-mutation-clinical-features-and-enzyme-polymorphisms-in-9-southwestern-spanish-families
#20
Macarena Piñero-Saavedra, Teresa González-Quevedo, Blanca Saenz de San Pedro, Cristina Alcaraz, Pedro Bobadilla-González, Lourdes Fernández-Vieira, Belén Hinojosa, Raúl García-Lozano
BACKGROUND: Information on F12 mutation hereditary angioedema (HAE) is still limited, but Spain is now recognized as having one of the highest concentrations of cases in Western Europe. OBJECTIVE: To describe unique features of HAE in Spanish carriers of the F12 mutation and investigate a potential role for angiotensin-converting enzyme (ACE) and aminopeptidase-P polymorphisms in disease expression. METHODS: This was a prospective observational cohort study of 35 individuals (80% females) from 9 unrelated families carrying the p...
November 2016: Annals of Allergy, Asthma & Immunology
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