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https://www.readbyqxmd.com/read/28905884/clinical-utility-gene-card-for-hereditary-angioedema-with-normal-c1-inhibitor-haenc1
#1
Christiane Stieber, Sven Cichon, Markus Magerl, Markus M Nöthen
No abstract text is available yet for this article.
October 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28866304/clinical-and-biological-response-to-rituximab-treatment-in-3-patients-with-acquired-c1-inhibitor-deficiency
#2
Paula J Busse, Yael Gernez
No abstract text is available yet for this article.
August 30, 2017: Annals of Allergy, Asthma & Immunology
https://www.readbyqxmd.com/read/28832655/complement-inhibition-attenuates-acute-kidney-injury-after-ischemia-reperfusion-and-limits-progression-to-renal-fibrosis-in-mice
#3
Juan S Danobeitia, Martynas Ziemelis, Xiaobo Ma, Laura J Zitur, Tiffany Zens, Peter J Chlebeck, Edwin S Van Amersfoort, Luis A Fernandez
The complement system is an essential component of innate immunity and plays a major role in the pathogenesis of ischemia-reperfusion injury (IRI). In this study, we investigated the impact of human C1-inhibitor (C1INH) on the early inflammatory response to IRI and the subsequent progression to fibrosis in mice. We evaluated structural damage, renal function, acute inflammatory response, progression to fibrosis and overall survival at 90-days post-injury. Animals receiving C1INH prior to reperfusion had a significant improvement in survival rate along with superior renal function when compared to vehicle (PBS) treated counterparts...
2017: PloS One
https://www.readbyqxmd.com/read/28825570/treatment-of-hereditary-angioedema-due-to-c1-inhibitor-deficiency-in-argentina
#4
Eloisa Malbrán, Alejandra Menéndez, Alejandro Malbrán
The benefits of the worldwide approval of new drugs for the treatment of acute C1-INH-HAE attacks may still not reach all patients. Identifying the current barriers in the access to medication, as well as conducting a detailed assessment of the progress in this area, is essential to achieve universal treatment. Two hundred and twenty five patients registered in the Argentina Hereditary Angioedema Patient Association (AHAEPA) were randomly selected and invited to participate in a web based questionnaire on accessibility to icatibant and pdC1-INH, self-treatment, delay to treatment, and coverage...
2017: Medicina
https://www.readbyqxmd.com/read/28795390/potential-influence-of-endothelial-adsorption-on-the-delayed-time-to-maximum-concentration-of-biopharmaceuticals
#5
Joannes A A Reijers, Martijn J C Dane, Anton Jan van Zonneveld, Jacobus Burggraaf, Matthijs Moerland
BACKGROUND AND OBJECTIVES: Maximum plasma concentration of biopharmaceuticals sometimes occurs long after completion of intravenous infusion. The objective of this research was to study the hypothetical adsorption of biopharmaceuticals to endothelium and infusion material, which may theoretically explain this phenomenon. METHODS: Infusion procedures were mimicked in an artificial vessel covered with a confluent monolayer of endothelial cells. Three monoclonal antibodies (MAbs) and C1 inhibitor were studied...
August 9, 2017: European Journal of Drug Metabolism and Pharmacokinetics
https://www.readbyqxmd.com/read/28781749/pediatric-hereditary-angioedema-an-update
#6
REVIEW
Geetika Sabharwal, Timothy Craig
Hereditary angioedema (HAE) with C1-inhibitor (C1-Inh) deficiency (C1-Inh-HAE) is a rare, life-threatening, and disabling genetic disorder characterized by self-limited tissue swelling caused by deficiency or dysfunction of C1-Inh. Our aim in this update is to discuss new advances in HAE therapy, focusing mainly on the various treatment options that have become available recently and also drugs that are under trial for prophylaxis to prevent attacks. There is a paradigm shift to where the treatment of HAE is headed, focusing now on prophylactic treatment rather than abortive management...
2017: F1000Research
https://www.readbyqxmd.com/read/28780879/-effect-of-danazol-treatment-on-growth-in-pediatric-patients-with-hereditary-angioedema-due-to-c1-inhibitor-deficiency
#7
Kinga Viktória Kőhalmi, Nóra Veszeli, Andrea Luczay, Lilian Varga, Henriette Farkas
INTRODUCTION: Attenuated androgens are used for the prevention of angioedema attacks of hereditary angioedema with C1-inhibitor deficiency. After prepuberty, their use can lead to growth retardation. AIM: We assessed the effect of danazol on the growth of pediatric patients with hereditary angioedema. METHOD: In the retrospective study on 42 patients diagnosed with hereditary angioedema, we calculated the deviation from the mid-parental target height, and analyzed it against the gender, the dose and duration of danazol treatment administered before the age of 21 years and before the age of 16 years...
August 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28754491/recombinant-human-c1-esterase-inhibitor-for-prophylaxis-of-hereditary-angio-oedema-a-phase-2-multicentre-randomised-double-blind-placebo-controlled-crossover-trial
#8
Marc A Riedl, Vesna Grivcheva-Panovska, Dumitru Moldovan, James Baker, William H Yang, Bruno M Giannetti, Avner Reshef, Sladjana Andrejevic, Richard F Lockey, Roman Hakl, Shmuel Kivity, Joseph R Harper, Anurag Relan, Marco Cicardi
BACKGROUND: Hereditary angio-oedema is a recurrent, oedematous disorder caused by deficiency of functional C1 inhibitor. Infusions of plasma-derived C1 esterase inhibitor deter attacks of hereditary angio-oedema, but the prophylactic effect of recombinant human C1 esterase inhibitor has not been rigorously studied. We aimed to assess the efficacy of recombinant human C1 esterase inhibitor for prophylaxis of hereditary angio-oedema. METHODS: We conducted this phase 2, multicentre, randomised, double-blind, placebo-controlled crossover trial at ten centres in Canada, the Czech Republic, Israel, Italy, Macedonia, Romania, Serbia, and the USA...
July 25, 2017: Lancet
https://www.readbyqxmd.com/read/28742139/acquisition-of-c1-inhibitor-by-bordetella-pertussis-virulence-associated-gene-8-results-in-c2-and-c4-consumption-away-from-the-bacterial-surface
#9
Elise S Hovingh, Bryan van den Broek, Betsy Kuipers, Elena Pinelli, Suzan H M Rooijakkers, Ilse Jongerius
Whooping cough, or pertussis, is a contagious disease of the respiratory tract that is re-emerging worldwide despite high vaccination coverage. The causative agent of this disease is the Gram-negative Bordetella pertussis. Knowledge on complement evasion strategies of this pathogen is limited. However, this is of great importance for future vaccine development as it has become apparent that a novel pertussis vaccine is needed. Here, we unravel the effect of Virulence associated gene 8 (Vag8) of B. pertussis on the human complement system at the molecular level...
July 2017: PLoS Pathogens
https://www.readbyqxmd.com/read/28734272/glycosylation-patterns-of-selected-proteins-in-individual-serum-and-cerebrospinal-fluid-samples
#10
Isabella Karlsson, Lorena Ndreu, Alessandro Quaranta, Gunnar Thorsén
A method we previously developed has been applied to the determination of the glycosylation pattern of specific proteins in biological samples. Six proteins (alpha-1-antitrypsin, transferrin, haptoglobin, C1 inhibitor, alpha-1 acid glycoprotein, and immunoglobulin G) were studied in serum samples from five individuals and cerebrospinal fluid (CSF) samples from three individuals, to investigate the expected normal distribution of glycosylation patterns and to assess whether this methodology can be used to discriminate between samples from different individuals...
October 25, 2017: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/28731906/c1-inhibitor-treatment-decreases-renal-injury-in-an-established-brain-dead-rat-model
#11
Felix Poppelaars, Neeltina M Jager, Juha Kotimaa, Henri G D Leuvenink, Mohamed R Daha, Cees van Kooten, Marc A Seelen, Jeffrey Damman
BACKGROUND: Kidneys derived from brain-dead (BD) donors have lower graft survival rates compared to kidneys from living donors. Complement activation plays an important role in brain death. The aim of our study was therefore to investigate the effect of C1-inhibitor (C1-INH) on BD-induced renal injury. METHODS: Brain death was induced in rats by inflating a subdurally placed balloon catheter. Thirty minutes after BD, rats were treated with saline, low-dose or high-dose C1-INH...
July 21, 2017: Transplantation
https://www.readbyqxmd.com/read/28715060/the-complex-interaction-between-polycystic-ovary-syndrome-and-hereditary-angioedema-case-reports-and-review-of-the-literature
#12
Marina Iahn-Aun, Marcelo Vivolo Aun, Antonio Abílio Motta, Jorge Kalil, Pedro Giavina-Bianchi, Sylvia Asaka Hayashida, Edmund Chada Baracat, Gustavo Arantes Maciel
Importance: Hereditary angioedema (HAE) is a rare but severe disease, with high risk of death, and attacks have been associated to high estrogen levels. Polycystic ovary syndrome (PCOS) is a common hyperandrogenic condition, which is frequently treated with combined oral contraceptives. Objective: The aim of this study was to describe 2 clinical cases of young women diagnosed as having PCOS who developed HAE attacks after the introduction of combined estrogen-progestin pills to treat PCOS symptoms...
July 2017: Obstetrical & Gynecological Survey
https://www.readbyqxmd.com/read/28707765/tissue-microarray-methodology-identifies-complement-pathway-activation-and-dysregulation-in-progressive-multiple-sclerosis
#13
Sam Loveless, James W Neal, Owain W Howell, Katharine E Harding, Patrick Sarkies, Rhian Evans, Ryan J Bevan, Svetlana Hakobyan, Claire L Harris, Neil P Robertson, B Paul Morgan
BACKGROUND: The complement pathway has potential contributions to both white (WM) and grey matter (GM) pathology in Multiple Sclerosis (MS). A quantitative assessment of complement involvement is lacking. OBJECTIVE: Tissue MicroArray methodology was used in conjunction with immunohistochemistry to investigate the localization of complement pathway proteins in progressive MS cortical GM and subcortical WM. METHODS: Antibodies targeting complement proteins C1q, C3b, regulatory proteins C1 inhibitor (C1INH), complement receptor 1 (CR1), clusterin, factor H (FH) and the C5a anaphylatoxin receptor (C5aR) were utilised alongside standard markers of tissue pathology...
July 14, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28692169/the-relationship-between-anxiety-and-quality-of-life-in-children-with-hereditary-angioedema
#14
Aharon Kessel, Henriette Farkas, Shmuel Kivity, Nóra Veszeli, Kinga Viktória Kőhalmi, Batya Engel-Yeger
BACKGROUND: The severe life-threatening characteristics of hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE) can affect anxiety levels among pediatric patients. This emotional burden together with the physical restrictions of C1-INH-HAE may decrease children's health-related quality of life (HRQoL). OBJECTIVE: (1) To compare anxiety state and trait between children with C1-INH-HAE and healthy controls (2) To examine the relationship between the level of anxiety of children with C1-INH-HAE, their disease activity/affected sites and their HRQoL (3) To predict the HRQoL of children with C1-INH-HAE based on their anxiety level and disease activity/affected sites...
July 10, 2017: Pediatric Allergy and Immunology
https://www.readbyqxmd.com/read/28690642/breakthrough-attacks-in-patients-with-hereditary-angioedema-receiving-long-term-prophylaxis-are-responsive-to-icatibant-findings-from-the-icatibant-outcome-survey
#15
Werner Aberer, Marcus Maurer, Laurence Bouillet, Andrea Zanichelli, Teresa Caballero, Hilary J Longhurst, Amandine Perrin, Irmgard Andresen
BACKGROUND: Patients with hereditary angioedema (HAE) due to C1-inhibitor deficiency (C1-INH-HAE) experience recurrent attacks of cutaneous or submucosal edema that may be frequent and severe; prophylactic treatments can be prescribed to prevent attacks. However, despite the use of long-term prophylaxis (LTP), breakthrough attacks are known to occur. We used data from the Icatibant Outcome Survey (IOS) to evaluate the characteristics of breakthrough attacks and the effectiveness of icatibant as a treatment option...
2017: Allergy, Asthma, and Clinical Immunology
https://www.readbyqxmd.com/read/28687112/burden-of-illness-and-quality-of-life-measures-in-angioedema-conditions
#16
REVIEW
Teresa Caballero, Nieves Prior
Burden of illness studies and evaluation of health-related quality of life using validated questionnaires have become an important task in the comprehensive management of angioedema conditions, mainly angioedema associated with chronic spontaneous urticaria and hereditary angioedema caused by C1-inhibitor deficiency. A review of the principal tools and studies is presented. Both diseases present a higher proportion of psychiatric disorders, impair work and studies productivity, and produce high direct and indirect costs...
August 2017: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/28687110/hereditary-angioedema-with-normal-c1-inhibitor-update-on-evaluation-and-treatment
#17
REVIEW
Markus Magerl, Anastasios E Germenis, Coen Maas, Marcus Maurer
A new form of hereditary angioedema (HAE) was identified in the year 2000. Its clinical appearance resembles HAE types I and II, which are caused by mutations that result in a deficiency of C1 inhibitor (C1-INH). In patients with the new form of HAE, C1-INH plasma levels and function values are normal, so it's termed HAE with normal C1-INH (HAE-nC1). HAE-nC1, in a subgroup of patients, is thought to be caused by mutations that affect the F12 gene. The diagnosis of HAE-nC1 is based on history and clinical criteria...
August 2017: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/28687109/prophylactic-therapy-for-hereditary-angioedema
#18
REVIEW
Hilary Longhurst, Emily Zinser
Long-term prophylaxis is needed in many patients with hereditary angioedema and poses many challenges. Attenuated androgens are effective in many but are limited by side effect profiles. There is less evidence for efficacy of tranexamic acid and progestagens; however, the small side effect profile makes tranexamic acid an option for prophylaxis in children and progestagens an option for women. C1 inhibitor is beneficial, but at present requires intravenous delivery and may need dose titration for maximum efficacy...
August 2017: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/28687108/acute-management-of-hereditary-angioedema-attacks
#19
REVIEW
Constance H Katelaris
Several treatment modalities have become available for management of acute hereditary angioedema (HAE) attacks in the last 15 years. Most are now available to patients in North America, Europe, United Kingdom, and Australia, but few options exist in developing countries. Preferred contemporary use of the treatments to be discussed is "on demand," because control remains with the patient and delays in treatment access avoided. Four treatments-plasma-derived C1 inhibitor concentrate, recombinant C1 inhibitor concentrate, ecallantide, and icatibant-are reviewed in this article...
August 2017: Immunology and Allergy Clinics of North America
https://www.readbyqxmd.com/read/28687107/laboratory-approaches-for-assessing-contact-system-activation
#20
REVIEW
Sandra C Christiansen, Bruce L Zuraw
Hereditary angioedema (HAE) is a rare autosomal dominant disease clinically characterized by recurrent, often unpredictable attacks of subcutaneous and mucosal swelling. Acute episodes are debilitating, painful, disfiguring, and potentially fatal. HAE type I and type II result from a deficiency in the plasma level of functional C1 inhibitor. HAE with normal levels of C1 inhibitor has been recognized. There is evidence that contact activation underlies the recurrent attacks of swelling. This article reviews laboratory parameters to detect contact system activation and implications for diagnosis of HAE and other forms of bradykinin-mediated angioedema...
August 2017: Immunology and Allergy Clinics of North America
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