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C1-inhibitor

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https://www.readbyqxmd.com/read/29343682/intermittent-c1-inhibitor-deficiency-associated-with-recessive-inheritance-functional-and-structural-insight
#1
Sonia Caccia, Chiara Suffritti, Thomas Carzaniga, Romina Berardelli, Silvia Berra, Vincenzo Martorana, Annamaria Fra, Christian Drouet, Marco Cicardi
C1-inhibitor is a serine protease inhibitor (serpin) controlling complement and contact system activation. Gene mutations result in reduced C1-inhibitor functional plasma level causing hereditary angioedema, a life-threatening disorder. Despite a stable defect, the clinical expression of hereditary angioedema is unpredictable, and the molecular mechanism underlying this variability remains undisclosed. Here we report functional and structural studies on the Arg378Cys C1-inhibitor mutant found in a patient presenting reduced C1-inhibitor levels, episodically undergoing normalization...
January 17, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29316335/exposure-response-model-of-subcutaneous-c1-inhibitor-concentrate-to-estimate-the-risk-of-attacks-in-patients-with-hereditary-angioedema
#2
Ying Zhang, Michael A Tortorici, Dipti Pawaskar, Ingo Pragst, Thomas Machnig, Matthew Hutmacher, Bruce Zuraw, Marco Cicardi, Timothy Craig, Hilary Longhurst, Jagdev Sidhu
Subcutaneous C1-inhibitor (HAEGARDA, CSL Behring), is a US Food and Drug Administration (FDA)-approved, highly concentrated formulation of a plasma-derived C1-esterase inhibitor (C1-INH), which, in the phase III Clinical Studies for Optimal Management in Preventing Angioedema with Low-Volume Subcutaneous C1-inhibitor Replacement Therapy (COMPACT) trial, reduced the incidence of hereditary angioedema (HAE) attacks when given prophylactically. Data from the COMPACT trial were used to develop a repeated time-to-event model to characterize the timing and frequency of HAE attacks as a function of C1-INH activity, and then develop an exposure-response model to assess the relationship between C1-INH functional activity levels (C1-INH(f)) and the risk of an attack...
January 9, 2018: CPT: Pharmacometrics & Systems Pharmacology
https://www.readbyqxmd.com/read/29316218/local-administration-of-nuclear-factor-of-activated-t-cells-nfat-c1-inhibitor-to-suppress-early-resorption-and-inflammation-induced-by-bone-morphogenetic-protein-2
#3
Ri Youn Kim, Yeju Seong, Tae Hyung Cho, Beomseok Lee, In Sook Kim, Soon Jung Hwang
Nuclear factor of activated T cells (NFAT)-c1 is known as a key regulator in osteoclast differentiation and immune response. This study is a follow-up to our previous study showing the anti-resorptive activity of VIVIT, a peptide type NFATc1 inhibitor, using absorbable collagen sponge (ACS). This study aimed to investigate the effective concentration range of local VIVIT that suppresses early excessive osteoclast activation and inflammation induced by high-dose recombinant human bone morphogenetic protein (rhBMP)-2 and concomitantly enhances bone healing in a rat critical-sized calvaria defect model...
January 9, 2018: Journal of Biomedical Materials Research. Part A
https://www.readbyqxmd.com/read/29315141/the-treatment-of-antibody-mediated-rejection-in-kidney-transplantation-an-updated-systematic-review-and-meta-analysis
#4
Susan S Wan, Tracey D Ying, Kate Wyburn, Darren M Roberts, Melanie Wyld, Steven J Chadban
BACKGROUND: Current treatments for antibody-mediated rejection (AMR) in kidney transplantation are based on low-quality data from a small number of controlled trials. Novel agents targeting B-cells, plasma-cells and the complement system have featured in recent studies of AMR. METHODS: We conducted a systematic review and meta-analysis of controlled trials in kidney transplant recipients using Medline, EMBASE and CENTRAL from inception to February 2017. RESULTS: Of 14,380 citations we identified 21 studies, including 10 randomized controlled trials, involving 751 participants...
January 8, 2018: Transplantation
https://www.readbyqxmd.com/read/29303100/-vulvar-angioedema-during-pregnancy
#5
E P Olthof, M R van de Scheur, A E Weis-Potters
BACKGROUND: Angioedema is a condition of acute and extensive fluid accumulation in skin or mucosae due to increased blood vessel permeability. Angioedema can have several causes, including pregnancy. CASE DESCRIPTION: A healthy 33-year-old pregnant woman had acute, substantial swelling of the labia minora with no other symptoms. Based on clinical criteria, angioedema was diagnosed. Laboratory testing did not find any C1 inhibitor deficiency and hereditary angioedema was excluded for that reason...
2018: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/29279246/complement-system-biomarkers-in-first-episode-psychosis
#6
Maja Kopczynska, Wioleta Zelek, Samuel Touchard, Fiona Gaughran, Marta Di Forti, Valeria Mondelli, Robin Murray, Michael C O'Donovan, B Paul Morgan
Several lines of evidence implicate immunological/inflammatory factors in development of schizophrenia. Complement is a key driver of inflammation, and complement dysregulation causes pathology in many diseases. Here we explored whether complement dysregulation occurred in first episode psychosis (FEP) and whether this provides a source of biomarkers. Eleven complement analytes (C1q, C3, C4, C5, factor B [FB], terminal complement complex [TCC], factor H [FH], FH-related proteins [FHR125], Properdin, C1 inhibitor [C1inh], soluble complement receptor 1 [CR1]) plus C-reactive protein (CRP) were measured in serum from 136 first episode psychosis (FEP) cases and 42 mentally healthy controls using established in-house or commercial ELISA...
December 23, 2017: Schizophrenia Research
https://www.readbyqxmd.com/read/29249827/schizophrenia-risk-and-urban-birth-are-associated-with-proteomic-changes-in-neonatal-dried-blood-spots
#7
Jason D Cooper, Sureyya Ozcan, Renee M Gardner, Nitin Rustogi, Susanne Wicks, Geertje F van Rees, F Markus Leweke, Christina Dalman, Håkan Karlsson, Sabine Bahn
In the present study, we tested whether there were proteomic differences in blood between schizophrenia patients after the initial onset of the disorder and controls; and whether those differences were also present at birth among neonates who later developed schizophrenia compared to those without a psychiatric admission. We used multiple reaction monitoring mass spectrometry to quantify 77 proteins (147 peptides) in serum samples from 60 first-onset drug-naive schizophrenia patients and 77 controls, and 96 proteins (152 peptides) in 892 newborn blood-spot (NBS) samples collected between 1975 and 1985...
December 18, 2017: Translational Psychiatry
https://www.readbyqxmd.com/read/29249111/-hereditary-angioedema-by-c1-inhibitor-deficit-diagnostic-and-therapeutic-challenges-case-report
#8
Álvaro José Mayorga, Gerardo José Ayestas-Moreno
BACKGROUND: Hereditary angioedema is a disease which manifests itself with episodes of spontaneous edema on skin, mucosal and airway. Treatment includes acute and prophylactic approach to minimize the attacks and severity. In many parts of the world, androgen derivatives, antifibrinolytic and fresh frozen plasma are the therapies available for prophylaxis. CLINICAL REPORT: 16 years old teenager of without history of immune decease, has in the course of 1-year repetitive episodes of painless, non-pruritic angioedema, does not respond to antihistamine therapy, corticosteroids or adrenaline; fresh frozen plasma is applied in 1 occasion exacerbating episode with severity...
October 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/29233911/n-and-o-glycosylation-analysis-of-human-c1-inhibitor-reveals-extensive-mucin-type-o-glycosylation
#9
Kathrin Stavenhagen, H Mehmet Kayili, Stephanie Holst, Carolien Koeleman, Ruchira Engel, Diana Wouters, Sacha Zeerleder, Bekir Salih, Manfred Wuhrer
Human C1-inhibitor (C1-Inh) is a serine protease inhibitor and the major regulator of the contact activation pathway as well as the classical and lectin complement pathways. It is known to be a highly glycosylated plasma glycoprotein. However, both the structural features and biological role of C1-Inh glycosylation are largely unknown. Here, we performed for the first time an in-depth site-specific N- and O-glycosylation analysis of C1-Inh combining various mass spectrometric approaches, including C18-porous graphitized carbon (PGC)-LC-ESI-QTOF-MS/MS applying stepping-energy collision-induced dissociation (CID) and electron-transfer dissociation (ETD)...
December 12, 2017: Molecular & Cellular Proteomics: MCP
https://www.readbyqxmd.com/read/29226721/investigational-drugs-in-phase-i-and-phase-ii-clinical-trials-for-hereditary-angioedema
#10
Henriette Farkas, Márta Lídia Debreczeni, Kinga Viktória Kőhalmi
Hereditary angioedema (HAE) with C1-inhibitor deficiency (C1-INH-HAE) is a rare bradykinin-mediated disease characterized by recurrent subcutaneous and/or submucosal angioedematous attacks (HAE attacks), which occur unpredictably. The recurrent HAE attacks do not respond to conventional treatments, and may evolve into a life-threatening condition; therefore, special therapy is required. Areas covered: The agents used so far for the acute management of HAE attacks act by blocking the release of bradykinin, or its binding to its receptor...
January 2018: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/29214395/pharmacological-management-of-hereditary-angioedema-with-c1-inhibitor-deficiency-in-pediatric-patients
#11
REVIEW
Henriette Farkas
Hereditary angioedema with C1-inhibitor deficiency (C1-INH-HAE) is a form of bradykinin-mediated angioedema. It is a rare disorder with an onset during childhood in most instances. Therefore, familiarity with the options for the management of pediatric cases is indispensable. The recurrent angioedematous episodes do not respond to conventional treatments and may evolve into a life-threatening condition. In view of the recommendations adopted by international consensus in 2016, patient management and follow-up should be guided by an individualized strategy...
December 7, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/29214154/the-search-for-biomarkers-in-hereditary-angioedema
#12
REVIEW
Allen P Kaplan, Coen Maas
The unpredictable nature of attacks of tissue swelling in hereditary angioedema requires the identification of reliable biomarkers to monitor disease activity as well as response to therapy. At present, one can assess a C4 level (by ELISA) to assist in diagnosis but neither C4 nor C1 inhibitor levels reflect clinical course or prognosis. We will here review a collection of plasma proteins involved in blood coagulation, fibrinolysis, and innate immunity (Figure 1). A main focus is those proteins that are key to the formation of bradykinin (BK); namely, factor XII, plasma prekallikrein/kallikrein, high-molecular weight kininogen, and BK itself since overproduction of BK is key to the disease...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/29130992/genetic-determinants-of-c1-inhibitor-deficiency-angioedema-age-of-onset
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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