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Search results for: Anti human C1 Esterase Inhibitor

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#33808005   2021/03/05 To Up

Heat-Inactivation of Human Serum Destroys C1 Inhibitor, Pro-motes Immune Complex Formation, and Improves Human T Cell Function.

Heat-inactivation of sera is used to reduce possible disturbing effects of complement factors in cell-culture experiments, but it is controversially discussed whether this procedure is appropriate or could be neglected. Here, we report a strong impact of heat-inactivation of human sera on the activation and effector functions of human CD4+ T cells. While T cells cultured with native sera were characterized by a higher proliferation rate and higher expression of CD28, heat-inactivated sera shaped T cells towards on-blast formation, higher cytokine secretion (interferon γ, tumor necrosis factor, and interleukin-17), stronger CD69 and PD-1 expression, and increased metabolic activity. Heat-inactivated sera contained reduced amounts of complement factors and regulators like C1 inhibitor, but increased concentrations of circulating immune complexes. Substitution of C1 inhibitor reduced the beneficial effect of heat-inactivation in terms of cytokine release, whereas surface-molecule expression was affected by the addition of complex forming anti-C1q antibody. Our data clearly demonstrate a beneficial effect of heat-inactivation of human sera for T cell experiments but indicate that beside complement regulators and immune complexes other components might be relevant. Beyond that, this study further underpins the strong impact of the complement system on T cell function.
Matthias A Fante, Sonja-Maria Decking, Christina Bruss, Stephan Schreml, Peter J Siska, Marina Kreutz, Kathrin Renner

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#33602658   // To Up

Treatment of Hereditary Angioedema.

Hereditary angioedema due to C1-esterase inhibitor deficiency (C1-INH-HAE) is a rare autosomal dominant disease. In the last decade, new drugs and new indications for old drugs have played a role in the management of C1-INH-HAE. This review examines current therapy for C1-INH-HAE and provides a brief summary of drugs that are under development. Increased knowledge of the pathophysiology of C1-INH-HAE has been crucial for advances in the field, with inhibition of the kallikrein-kinin system (plasma kallikrein, activated factor XII) as a key area in the discovery of new drugs, some of which are already marketed for treatment of C1-INH-HAE. Pharmacological treatment is based on 3 pillars: treatment of acute angioedema attacks (on-demand treatment), short-term (preprocedure) prophylaxis, and long-term prophylaxis. The 4 drugs that are currently available for the treatment of acute angioedema attacks (purified plasma-derived human C1 esterase inhibitor concentrate, icatibant acetate, ecallantide, recombinant human C1 esterase inhibitor) are all authorized for self-administration, except ecallantide. Purified plasma-derived human C1 esterase inhibitor concentrate is the treatment of choice for short-term prophylaxis. Tranexamic acid, danazol, intravenous and subcutaneous nanofiltered purified plasma-derived human C1 esterase inhibitor concentrate, and lanadelumab can be used for long-term prophylaxis. New drugs are being investigated, mainly as long-term prophylaxis, and are aimed at blocking the kallikrein-kinin system by means of antiprekallikrein, antikallikrein, and anti-activated FXII action.
T Caballero

3000 related Products with: Treatment of Hereditary Angioedema.

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#33472675   2021/01/20 To Up

Evaluation of the efficacy and safety of icatibant and C1 esterase/kallikrein inhibitor in severe COVID-19: study protocol for a three-armed randomized controlled trial.

SARS-CoV-2, the virus that causes COVID-19, enters the cells through a mechanism dependent on its binding to angiotensin-converting enzyme 2 (ACE2), a protein highly expressed in the lungs. The putative viral-induced inhibition of ACE2 could result in the defective degradation of bradykinin, a potent inflammatory substance. We hypothesize that increased bradykinin in the lungs is an important mechanism driving the development of pneumonia and respiratory failure in COVID-19.
Eli Mansour, Flávia F Bueno, José C de Lima-Júnior, Andre Palma, Milena Monfort-Pires, Bruna Bombassaro, Eliana P Araujo, Ana Flavia Bernardes, Raisa G Ulaf, Thyago A Nunes, Luciana C Ribeiro, Antônio Luís E Falcão, Thiago Martins Santos, Plinio Trabasso, Rachel P Dertkigil, Sergio S Dertkigil, Rafael P Maia, Tatiana Benaglia, Maria Luiza Moretti, Licio A Velloso

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#33441533   2021/01/14 To Up

Tissue Plasminogen Activator-Induced Angioedema Involving a Posterior Cerebral Artery Infarct: A Case Presentation.

BACKGROUND Angioedema is characterized by localized swelling of subcutaneous or submucosal tissue resulting from fluid extravasation due to the loss of vascular integrity. It most commonly occurs with exposure to allergens and certain medications, namely nonsteroidal anti-inflammatory agents and angiotensin-converting enzyme inhibitors. There have been few incidences of angioedema following the administration of tissue plasminogen activator. CASE REPORT We describe an 84-year-old woman with a history of hypertension managed with lisinopril who presented with an acute onset of right-sided hemiparesis, slurred speech, and right-sided hemianopsia. Urgent computed tomography of the head revealed subacute infarct of the left pons without hemorrhage. Intravenous alteplase was administered and within 30 min our patient developed severe orolingual edema requiring emergent intubation. Subsequent imaging revealed acute to subacute infarct of the left occipital lobe in the posterior cerebral artery region, consistent with her initial presenting symptoms. CONCLUSIONS Angioedema induced by tissue plasminogen activator occurs in approximately 1-5% of patients receiving thrombolysis for ischemic stroke and can be life-threatening. The risk is increased in patients taking angiotensin-converting enzyme inhibitors, in patients with ischemic strokes of the middle cerebral artery, and in the presence of C1 esterase inhibitor deficiency. This phenomenon is usually self-limited and treatment is supportive, although evidence supports the use of antihistamines, steroids, epinephrine, and complement inhibitors. Due to the severity of angioedema and the potential progression to airway compromise, it is crucial to closely monitor patients receiving tissue plasminogen activator.
Shahnaz Duymun, Vidhya Reddy, Emma Bentley, Anjali Bose-Kolanu

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#32894844   2020/09/07 To Up

Acquired Angioedema due to C1 Inhibitor Deficiency Preceding Splenic Marginal Zone Lymphoma: Further Insights from Clinical Practice.

Acquired angioedema due to C1 inhibitor deficiency (AAE-C1-INH) is a very rare disease. In clinical practice, it may be difficult to differentiate AAE-C1-INH from hereditary angioedema due to C1-INH deficiency (HAE-C1-INH). In both conditions, patients are at an increased risk of death from asphyxiation due to upper airway obstruction. The association of AAE-C1-INH with lymphoproliferative and autoimmune diseases, and with presence of anti-C1-INH antibodies has been well documented, and treatment of the underlying condition may result in complete remission of angioedema.
Mariana Paes Leme Ferriani, Orlando Trevisan-Neto, Julia S Costa, Janaina M L Melo, Adriana S Moreno, Marina M Dias, Pedro M M Garibaldi, Gabriel C Pereira, Fernando Chahud, Fabiola Traina, L Karla Arruda

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#32717572   2020/07/24 To Up

Mapping the binding site of C1-inhibitor for polyanion cofactors.

The serpin, C1-inhibitor (also known as SERPING1), plays a vital anti-inflammatory role in the body by controlling pro-inflammatory pathways such as complement and coagulation. The inhibitor's action is enhanced in the presence of polyanionic cofactors, such as heparin and polyphosphate, by increasing the rate of association with key enzymes such as C1s of the classical pathway of complement. The cofactor binding site of the serpin has never been mapped. Here we show that residues Lys284, Lys285 and Arg287 of C1-inhibitor play key roles in binding heparin and delivering the rate enhancement seen in the presence of polyanions and thus most likely represent the key cofactor binding residues for the serpin. We also show that simultaneous binding of the anion binding site of C1s by the polyanion is required to deliver the rate enhancement. Finally, we have shown that it is unlikely that the two positively charged zones of C1-inhibitor and C1s interact in the encounter complex between molecules as ablation of the charged zones did not in itself deliver a rate enhancement as might have been expected if the zones interacted. These insights provide crucial information as to the mechanism of action of this key serpin in the presence and absence of cofactor molecules.
Lilian Hor, Jing Pan, James C Whisstock, Robert N Pike, Lakshmi C Wijeyewickrema

1729 related Products with: Mapping the binding site of C1-inhibitor for polyanion cofactors.

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#32668719   2020/07/13 To Up

A Novel C1-Esterase Inhibitor Oxygenator Coating Prevents FXII Activation in Human Blood.

The limited hemocompatibility of currently used oxygenator membranes prevents long-term use of artificial lungs in patients with lung failure. To improve hemocompatibility, we developed a novel covalent C1-esterase inhibitor (C1-INH) coating. Besides complement inhibition, C1-INH also prevents FXII activation, a very early event of contact phase activation at the crossroads of coagulation and inflammation. Covalently coated heparin, as the current anticoagulation gold standard, served as control. Additionally, a combination of both coatings (C1-INH/heparin) was established. The coatings were tested for their hemocompatibility by dynamic incubation with freshly drawn human whole blood. The analysis of various blood and plasma parameters revealed that C1-INH-containing coatings were able to markedly reduce FXIIa activity compared to heparin coating. Combined C1-INH/heparin coatings yielded similarly low levels of thrombin-antithrombin III complex formation as heparin coating. In particular, adhesion of monocytes and platelets as well as the diminished formation of fibrin networks were observed for combined coatings. We could show for the first time that a covalent coating with complement inhibitor C1-INH was able to ameliorate hemocompatibility. Thus, the early inhibition of the coagulation cascade is likely to have far-reaching consequences for the other cross-reacting plasma protein pathways.
Katharina Gerling, Sabrina Ölschläger, Meltem Avci-Adali, Bernd Neumann, Ernst Schweizer, Christian Schlensak, Hans-Peter Wendel, Sandra Stoppelkamp

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#32553246   // To Up

Complement-Based Therapy in the Management of Antibody-Mediated Rejection.

Antibody-mediated rejection (AMR) is one of the leading causes of kidney allograft failure and is usually mediated by anti-human leukocyte antigen donor-specific antibodies (DSAs). Activation of classical pathway of the complement system is responsible for downstream effects of DSA and account for significant manifestations of AMR. Currently, the treatment of AMR is based on strategies to remove preformed antibodies or to prevent their production; however, these strategies are often unsuccessful. It is theoretically possible to inhibit complement activity to prevent the effect of DSA on kidney allograft function. Complement inhibitors such as eculizumab, a complement 5 monoclonal antibody, and complement 1 esterase inhibitors (C1 INHs) have been used in prevention and treatment of AMR with variable success. Eculizumab and C1 INH seem to reduce the incidence of early AMR and allow transplantation in highly sensitized kidney transplant recipients, but data on their long-term effect on kidney allograft function are limited. Several case reports described the successful use of eculizumab in the treatment of AMR, but there are no randomized controlled studies that showed efficacy. Treatment of AMR with C1 INH, in addition to standard of care, did not change short-term outcome but long-term studies are underway.
Anshul Bhalla, Nada Alachkar, Sami Alasfar

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#32163480   2020/03/12 To Up

Short-term prophylaxis in patients with angioedema due to C1-inhibitor deficiency undergoing dental procedures: An observational study.

Patients affected by angioedema due to hereditary and acquired C1-inhibitor (C1-INH) deficiency (HAE and AAE, respectively) report trouble accessing dental care, due to the risk of a life-threatening oropharyngeal and laryngeal attack triggered by dental procedures. The aim of this study was to assess the identification of hurdles in receiving dental care, and the effectiveness of short-term prophylaxis (STP) in preventing angioedema attacks. In addition, the study evaluated the impact of dental care in angioedema disease. All patients affected by angioedema due to C1-INH deficiency who were treated in the dentistry outpatient department of ASST Fatebenefratelli Sacco hospital (Milan, Italy) between 2009 and 2017 were considered for the analysis. Data were collected from patients' records.
Andrea Zanichelli, Mario Ghezzi, Ivan Santicchia, Romualdo Vacchini, Marco Cicardi, Antonella Sparaco, Girolamo Donati, Vito Ranìa, Alberto Busa

1798 related Products with: Short-term prophylaxis in patients with angioedema due to C1-inhibitor deficiency undergoing dental procedures: An observational study.

100 μg100 μg100 μg 0.1 mg 100 μg100ug Lyophilized100 μg100 μg100uL50 mg100 μg 100ul

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#31841366   2019/12/16 To Up

Modeling Cost-Effectiveness of On-Demand Treatment for Hereditary Angioedema Attacks.

Hereditary angioedema (HAE) is a rare C1-inhibitor (C1-INH) deficiency disease. Low levels of functional C1-INH can lead to recurrent attacks of severe swelling occurring in areas such as the limbs, face, gastrointestinal tract, and throat. These attacks are both painful and disabling and, if not treated promptly and effectively, can result in hospitalization or death. Agents targeting the specific physiologic pathway of HAE attacks can offer improved outcomes with limited side effects compared with nonspecific therapies. However, these treatments display varying efficacy in HAE patients, including the need to redose or seek additional care if the treatment does not resolve symptoms effectively.
Jonathan A Bernstein, Christopher Tyson, Anurag Relan, Philippe Adams, Raf Magar

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