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https://www.readbyqxmd.com/read/27913306/effect-of-bradykinin-receptor-antagonism-on-ace-inhibitor-associated-angioedema
#1
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 one study of European patients. 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
#2
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
#3
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
#4
K Bork, K Wulff, G Witzke, S Rietz, J Hardt
Tamoxifen had been described as a triggering factor for hereditary angioedema (HAE) due to C1 inhibitor (C1-INH) deficiency (HAE-C1-INH).(1, 2) We report on a patient with HAE and normal C1-INH and a further patient with HAE-C1-INH with a life-threatening exacerbation due to tamoxifen. Patient 1: A now 73 year-old woman had a family history negative for angioedema. She reported 3 lip swellings during the second and third trimester of her second pregnancy at age 23. This article is protected by copyright. All rights reserved...
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
#5
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
#6
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.
November 16, 2016: 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
#7
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
#8
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...
November 12, 2016: Molecular Immunology
https://www.readbyqxmd.com/read/27826093/cytokine-and-estrogen-stimulation-of-endothelial-cells-augment-activation-of-the-prekallikrein-high-molecular-weight-kininogen-complex-implications-for-hereditary-angioedema-hae
#9
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 due to release of heat shock protein-90 (Hsp90). While bradykinin formation is typically initiated by factor XII autoactivation it is also possible to activate factor XII either by kallikrein, thus formed, or plasmin. RATIONALE: Since 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
#10
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...
October 28, 2016: Structure
https://www.readbyqxmd.com/read/27810412/on-the-value-of-therapeutic-interventions-targeting-the-complement-system-in-acute-myocardial-infarction
#11
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
#12
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
#13
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
https://www.readbyqxmd.com/read/27788880/assessment-of-inhibitory-antibodies-in-patients-with-hereditary-angioedema-treated-with-plasma-derived-c1-inhibitor
#14
Henriette Farkas, Lilian Varga, Dumitru Moldovan, Krystyna Obtulowicz, Todor Shirov, Thomas Machnig, Henrike Feuersenger, Jonathan Edelman, Debora Williams-Herman, Mikhail Rojavin
BACKGROUND: Limited data are available regarding C1 inhibitor (C1-INH) administration and anti-C1-INH antibodies. OBJECTIVE: To assess the incidence of antibody formation during treatment with pasteurized, nanofiltered plasma-derived C1-INH (pnfC1-INH) in patients with hereditary angioedema with C1-INH deficiency (C1-INH-HAE) and the comparative efficacy of pnfC1-INH in patients with and without antibodies. METHODS: In this multicenter, open-label study, patients with C1-INH-HAE (≥12 years of age) were given 20 IU/kg of pnfC1-INH per HAE attack that required treatment and followed up for 9 months...
November 2016: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/27783665/regulation-of-complement-and-contact-system-activation-via-c1-inhibitor-potentiation-and-factor-xiia-activity-modulation-by-sulfated-glycans-structure-activity-relationships
#15
Ann-Kathrin Schoenfeld, Eric Lahrsen, Susanne Alban
The serpin C1 inhibitor (C1-INH) is the only regulator of classical complement activation as well as the major regulator of the contact system. Its importance is demonstrated by hereditary angioedema (HAE), a severe disease with potentially life-threatening attacks due to deficiency or dysfunction of C1-INH. C1-INH replacement is the therapy of choice in HAE. In addition, C1-INH showed to have beneficial effects in other diseases characterized by inappropriate complement and contact system activation. Due to some limitations of its clinical application, there is a need for improving the efficacy of therapeutically applied C1-INH or to enhance the activity of endogenous C1-INH...
2016: PloS One
https://www.readbyqxmd.com/read/27742086/misdiagnosis-trends-in-patients-with-hereditary-angioedema-from-the-real-world-clinical-setting
#16
Andrea Zanichelli, Hilary J Longhurst, Marcus Maurer, Laurence Bouillet, Werner Aberer, Vincent Fabien, Irmgard Andresen, Teresa Caballero
BACKGROUND: Hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE) causes swelling in the skin and upper airways and pain in the abdomen because of mucosal swelling. C1-INH-HAE is frequently misdiagnosed, leading to delays in diagnosis, inadequate treatment, and unnecessary procedures. OBJECTIVE: To evaluate the history of misdiagnosis in patients participating in the Icatibant Outcome Survey (IOS). METHODS: The IOS is an observational study in which safety and effectiveness of icatibant have been evaluated since 2009...
October 2016: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/27727922/delayed-diagnosis-of-c1-inhibitor-deficiency-associated-with-significant-morbidity-and-mortality
#17
Sujoy Khan
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27725554/hereditary-angioedema-with-recurrent-abdominal-pain-in-a-patient-with-a-novel-mutation
#18
Hiromasa Yakushiji, Arito Kaji, Keitarou Suzuki, Motohiro Yamada, Takahiko Horiuchi, Masahiro Sinozaki
We describe a patient with hereditary angioedema type I. The patient had experienced recurrent abdominal pain around the time of her menstrual period for 13 years. A laboratory examination showed reduced functional and antigenic levels of C4 and C1 inhibitor (C1-INH). To establish a diagnosis, we carried out a DNA analysis of the patient's C1-INH gene. We determined that the patient was heterozygous for a single base pair transposition of T to C at nucleotide 4429 in exon 4, which had not been reported in the literature...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27715075/-current-treatment-options-for-hereditary-angioedema
#19
Roman Hakl
Hereditární angioedém je vzácné dominantně dědičné onemocnění způsobené deficitem inhibitoru C1-esterázy (C1-INH). Onemocnění se klinicky projevuje recidivami lokalizovaných otoků podkoží a sliznic. Nemoc je hendikepující a může být i smrtelná. Charakteristická je extrémní variabilita v četnosti a závažnosti symptomů. Článek se zabývá organizací péče o pacienty a zahrnuje aktuální léčebné možnosti onemocnění. Strategie léčby zahrnuje krátkodobou a dlouhodobou profylaxi a léčbu atak...
2016: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/27699634/use-of-a-c1-inhibitor-concentrate-in-adults-%C3%A2-65-years-of-age-with-hereditary-angioedema-findings-from-the-international-berinert-%C3%A2-c1-inh-registry
#20
Anette Bygum, Inmaculada Martinez-Saguer, Murat Bas, Jeffrey Rosch, Jonathan Edelman, Mikhail Rojavin, Debora Williams-Herman
BACKGROUND: Treatment of hereditary angioedema (HAE) in 'older adults' (those aged ≥65 years) has not been well studied. The international Berinert Patient Registry collected data on the use of intravenous plasma-derived, pasteurized, nanofiltered C1-inhibitor concentrate (pnfC1-INH; Berinert(®)/CSL Behring) in patients of any age, including many older adults. METHODS: This observational registry, conducted from 2010 to 2014 at 30 US and seven European sites, gathered prospective (post-enrollment) and retrospective (pre-enrollment) usage and adverse event (AE) data on subjects treated with pnfC1-INH...
November 2016: Drugs & Aging
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