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C1-inhibitor

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https://www.readbyqxmd.com/read/29130992/genetic-determinants-of-c1-inhibitor-deficiency-angioedema-age-of-onset
#1
Panagiota Gianni, Gedeon Loules, Maria Zamanakou, Maria Kompoti, Dorottya Csuka, Fotis Psarros, Markus Magerl, Dimitru Moldovan, Marcus Maurer, Matthaios G Speletas, Henriette Farkas, Anastasios E Germenis
BACKGROUND: In view of the large heterogeneity in the clinical presentation of hereditary angioedema due to C1 inhibitor deficiency (C1-INH-HAE), great efforts are being made towards detecting measurable biological determinants of disease severity that can help to improve the management of the disease. Considering the central role that plasma kallikrein plays in bradykinin production, we investigated the contribution of the functional polymorphism KLKB1-428G/A to the disease phenotype...
November 9, 2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/29128335/hereditary-angioedema-with-normal-c1-inhibitor-and-f12-mutations-in-42-brazilian-families
#2
Camila Lopes Veronez, Adriana S Moreno, Rosemeire Navickas Constantino-Silva, Luana S M Maia, Mariana P L Ferriani, Fábio F M Castro, Solange Rodrigues Valle, Victor Koji Nakamura, Nathália Cagini, Rozana Fátima Gonçalves, Eli Mansour, Faradiba Sarquis Serpa, Gabriela Andrade Coelho Dias, Miguel Alberto Piccirillo, Eliana Toledo, Marli de Souza Bernardes, Sven Cichon, Christiane Stieber, L Karla Arruda, João Bosco Pesquero, Anete Sevciovic Grumach
BACKGROUND: Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH) is a rare condition with clinical features similar to those of HAE with C1-INH deficiency. Mutations in the F12 gene have been identified in subsets of patients with HAE with normal C1-INH, mostly within families of European descent. OBJECTIVES: Our aim was to describe clinical characteristics observed in Brazilians from 42 families with HAE and F12 gene mutations (FXII-HAE), and to compare these findings with those from other populations...
November 8, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/29112487/pharmacotherapy-for-angiotensin-converting-enzyme-inhibitor-induced-angioedema-a-systematic-review
#3
Claire M Lawlor, Ashwin Ananth, Blair M Barton, Thomas C Flowers, Edward D McCoul
Objective Angioedema is a potentially life-threatening complication of angiotensin-converting enzyme inhibitor (ACEI) use, occurring in up to 0.5% of users. Although the pathophysiology of ACEI-induced angioedema is attributable to elevated serum bradykinin, standard management typically includes corticosteroids and antihistamines. We sought to summarize the evidence supporting pharmacotherapy for ACEI-induced angioedema. Data Sources PubMed, MEDLINE, and Embase portals. Methods A systematic literature review was conducted according to the PRISMA guidelines...
November 1, 2017: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29110694/effects-of-freezer-storage-time-on-levels-of-complement-biomarkers
#4
Angharad R Morgan, Caroline O'Hagan, Samuel Touchard, Simon Lovestone, B Paul Morgan
BACKGROUND: There is uncertainty regarding how stable complement analytes are during long-term storage at - 80 °C. As part of our work program we have measured 17 complement biomarkers (C1q, C1 inhibitor, C3, C3a, iC3b, C4, C5, C9, FB, FD, FH, FI, TCC, Bb, sCR1, sCR2, Clusterin) and the benchmark inflammatory marker C-reactive protein (CRP) in a large set of plasma samples (n = 720) that had been collected, processed and subsequently stored at - 80 °C over a period of 6.6-10.6 years, prior to laboratory analysis...
November 6, 2017: BMC Research Notes
https://www.readbyqxmd.com/read/29107952/hereditary-angioedema-assessing-the-hypothesis-for-underlying-autonomic-dysfunction
#5
Maddalena A Wu, Francesco Casella, Francesca Perego, Chiara Suffritti, Nada Afifi Afifi, Eleonora Tobaldini, Andrea Zanichelli, Chiara Cogliati, Nicola Montano, Marco Cicardi
BACKGROUND: Attacks of Hereditary Angioedema due to C1-inhibitor deficiency (C1-INH-HAE)are often triggered by stressful events/hormonal changes. OBJECTIVE: Our study evaluates the relationship between autonomic nervous system (ANS) and contact/complement system activation. METHODS: Twenty-three HAE patients (6 males, mean age 47.5±11.4 years) during remission and 24 healthy controls (8 males, mean age 45.3±10.6 years) were studied. ECG, beat-by-beat blood pressure, respiratory activity were continuously recorded during rest (10') and 75-degrees-head-up tilt (10')...
2017: PloS One
https://www.readbyqxmd.com/read/29107384/binding-of-human-complement-c1-sterase-inhibitor-to-leptospira-spp
#6
Leandro Carvalho Dantas Breda, Silvio Arruda Vasconcellos, Dewton de Moraes Vasconcelos, Lourdes Isaac
Leptospirosis is an important zoonosis of global importance caused by bacteria Leptospira spp. Pathogenic Leptospira is resistant to Complement System killing while non-pathogenic Leptospira is rapidly killed by exposure to normal human serum (NHS). Pathogenic Leptospira interact with Complement Regulators such as Factor H, C4b binding protein and Vitronectin avoiding Complement activation and killing by Alternative and Classical Pathways. One important regulator is C1-inhibitor (C1INH) that interacts with C1s or MASPs controlling the cleavage of C4 and C2 molecules, thereby inhibiting the activation of the Classical and Lectin Pathways...
October 13, 2017: Immunobiology
https://www.readbyqxmd.com/read/29093422/comparison-of-the-frequency-of-angioedema-attack-before-and-during-pregnancy-in-a-patient-with-type-i-hereditary-angioedema
#7
Atsushi Satomura, Takayuki Fujita, Tomohiro Nakayama
The patient was a 38-year-old Japanese woman who had been diagnosed with hereditary angioedema type I at 7 years of age based on her family history. She had undergone four pregnancies. She gave birth to a healthy baby girl after her first pregnancy and had reported few episodes of angioedema. However, she subsequently required abortions due to frequent angioedema episodes that occurred during her three subsequent pregnancies. Thus, our patient showed two clinical pregnancy courses. After treating her with C1-inhibitor concentrate, her symptoms of angioedema disappeared...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29093383/clinical-features-of-hereditary-and-mast-cell-mediated-angioedema-focusing-on-the-differential-diagnosis-in-japanese-patients
#8
Isao Ohsawa, Daisuke Honda, Atsuko Hisada, Hiroyuki Inoshita, Kisara Onda-Tsueshita, Satoshi Mano, Nobuyuki Sato, Yuya Nakamura, Tatsuo Shimizu, Hiromichi Gotoh, Yoshikazu Goto, Yusuke Suzuki, Yasuhiko Tomino
Background The present study was designed to identify the clinical characteristics that permit the differential diagnosis of hereditary angioedema (HAE) and mast cell-mediated angioedema (Mast-AE) during the first consultation. Methods The medical histories and laboratory data of 46 patients with HAE and 41 patients with Mast-AE were compared. Results The average age of onset in the HAE group (19.8±9.0 years) was significantly lower than that in the Mast-AE group (35.2±12.0 years). The incidence of familial angioedema (AE) in the HAE group (73...
November 1, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29070276/multiple-doses-of-icatibant-used-during-pregnancy
#9
Lauren W Kaminsky, Theodore Kelbel, Fay Ansary, Timothy Craig
BACKGROUND: Hereditary angioedema (HAE) is a life-long disease that often manifests by puberty. Treatment of attacks is essential to improve quality of life and to decrease morbidity and mortality. During pregnancy, treatment is limited because multiple treatment options, including icatibant, are not approved for use during pregnancy. OBJECTIVE: We report the outcomes of three pregnancies during which icatibant was used by a patient with HAE with normal C1-inhibitor for treatment of attacks...
October 1, 2017: Allergy & Rhinology
https://www.readbyqxmd.com/read/29059678/endocan-a-novel-marker-of-endothelial-dysfunction-in-c1-inhibitor-deficient-hereditary-angioedema
#10
Mustafa Demirturk, Timur Selcuk Akpinar, Murat Kose, Aslı Gelincik, Bahattin Colakoğlu, Suna Buyukozturk
BACKGROUND: Hereditary angioedema (HAE) related to C1-inhibitor deficiency is a rare autosomal dominant disorder. Vascular cell adhesion molecules (VCAM) are known as endothelial activation markers. Endocan (also called ESM-1) is proposed as an endothelial dysfunction indicator. We aimed to investigate endothelial activation in attack-free periods in HAE patients by measuring their levels of endocan and VCAM-1. METHODS: Twenty-six HAE patients (22 female, mean age 40 ± 13 years) and 38 healthy control patients (13 female, mean age 36...
2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/29057741/pregnancy-and-postpartum-in-hereditary-angioedema-with-c1-inhibitor-deficit-in-women-who-have-no-access-to-therapy
#11
A Mrg Machado, R Mg Pires, R O Martins, A S Grumach
No abstract text is available yet for this article.
2017: Journal of Investigational Allergology & Clinical Immunology
https://www.readbyqxmd.com/read/29046193/health-related-quality-of-life-in-danish-children-with-hereditary-angioedema
#12
Anne Aabom, Dan Nguyen, Niels Fisker, Anette Bygum
BACKGROUND: The potentially life-threatening disease hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) can have considerable impact on the health-related quality of life (HRQoL) in adult patients. Half the patients with C1-INH-HAE develop symptoms before the age of 10 years. However, the HRQoL in children with C1-INH-HAE is almost unexplored. OBJECTIVE: To investigate HRQoL in Danish children with C1-INH-HAE, including possible correlations to disease severity and attack frequency...
November 1, 2017: Allergy and Asthma Proceedings:
https://www.readbyqxmd.com/read/28986975/complete-kinetic-follow-up-of-symptoms-and-complement-parameters-during-a-hereditary-angioedema-attack
#13
Nóra Veszeli, Kinga Viktória Kőhalmi, Erika Kajdácsi, Dominik Gulyás, György Temesszentandrási, László Cervenak, Henriette Farkas, Lilian Varga
We studied the kinetics of C1-inhibitor (C1-INH) and other complement parameters in a self-limited edematous attack (EA) in a patient with hereditary angioedema due to C1-INH deficiency to better understand the pathomechanism of the evolution, course, and complete resolution of EAs. C1-INH concentration and functional activity (C1-INHc+f ), C1(q,r,s), C3, C4, C3a, C4a, C5a and SC5b-9 levels were measured in blood samples obtained during the 96-hour observation period. The highest C1-INHc+f , C4, and C1(q,r,s) levels were measured at baseline, and their continuous decrease was observed during the entire observation period...
October 7, 2017: Allergy
https://www.readbyqxmd.com/read/28950264/an-abc-of-the-warning-signs-of-hereditary-angioedema
#14
REVIEW
Anete Sevciovic Grumach, Natasha Ferraroni, Maria Margarita Olivares, Maria Concepción López-Serrano, Anette Bygum
Hereditary angioedema (HAE) with C1 inhibitor deficiency is a genetic disorder that clinically manifests with attacks of angioedema in the subcutaneous and submucosal tissues, mainly in the extremities, abdomen, and upper airway. During attacks, vascular permeability is increased due to increased bradykinin (BK). This means that special therapies are needed for attacks that do not respond to traditional antiallergic therapies involving antihistamines, corticosteroids, and epinephrine. The recurring attacks may disable patients and lead to frequent visits to emergency rooms where misdiagnoses are common...
2017: International Archives of Allergy and Immunology
https://www.readbyqxmd.com/read/28905884/clinical-utility-gene-card-for-hereditary-angioedema-with-normal-c1-inhibitor-haenc1
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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