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Topic: C1-inhibitor

Ads by Google      AADMC: Selected Articles 2001: C1 inhibitor therapy for laryngeal edema in HAE C1 inhibitor therapy for laryngeal edema in HAE AADMC: Selected Articles 2001: C1 inhibitor therapy for laryngeal edema in HAE Laryngeal edema is a prominent and potentially fatal manifestation of hereditary angioedema (HAE) due to a deficiency or dysfunction of the C1 inhibitor (C1INH). http://www.aaaai.org/AADMC/CURRENTLITERATURE/selectedarticles/2001archive/c1_inhibitor.html      Canadian Hereditary Angioedema Society Currently unlicensed in Canada, C1 inhibitor replacement therapy may be made available to HAE patients on a compassionate basis. The Society exists to enhance the quality of life for persons with Hereditary Angioedema or Acquired Angioedema due to C1 inhibitor deficiency. C1 inhibitor is a complement protein that regulates various pathways within the body important in the maintenance of health, for example - fighting infections or healing inflammation. http://calgaryallergy.ca/Articles/HereditaryAngioedema.htm      Angioedema C1 inhibitor is also called C1 esterase inhibitor, but the shorter name is better, because the esterase only refers to something which happens in lab tests, not in the body. It comes about if you are short of a normal blood protein, called C1 inhibitor, or if you have inherited genes which make a C1 inhibitor protein which does not work properly. But if the reason is C1 inhibitor deficiency, at most they will just find swelling, and an operation won't help. http://www.users.globalnet.co.uk/~aair/angioedema.htm      UCLA Department of Medicine - wfsection-Hereditary Angioedema For acute intervention the drug of choice is C1 inhibitor concentrate. Visentin DE, Yang WH, Karsh J. C1-esterase inhibitor transfusions in patients with hereditary angioedema. C1 inhibitor concentrate is used extensively in Europe where adverse reactions or incidents of transmission of hepatitis C or HIV have not been reported. http://www.med.ucla.edu/modules/wfsection/article.php?articleid=110      Lactoferrin, a Potent Tryptase Inhibitor, Abolishes Late-Phase Airway Responses in Allergic Sheep -- ELROD et al. 156 (2): 375 -- American Journal of Respiratory and Critical Care Medicine Lactoferrin appears to be a noncompetitive inhibitor of human tryptase (Figure 3) consistent with a mechanism of irreversible Our results show that lactoferrin is a potent, time-dependent inhibitor of human tryptase; the K we show that lactoferrin is a poor inhibitor of rat skin tryptase http://www.ajrccm.org/cgi/content/full/156/2/375      Primary Immunodeficiency Association - Understanding Hereditary Angioedema and acquired C1-Inhibitor Deficiency Only when this approach fails, or if the drugs are contraindicated, should reliance on the inhibitor be considered. This is unusual so experience so far with the inhibitor is scant. Although this would seem to be preferable to drug therapy, the concentrate has the disadvantage of lasting only for a short time in the body. http://www.pia.org.uk/publications/general_publications/hereditary_angioedema.htm      New Prevention, Treatment for Hereditary Angioedema Attacks, September 1996 "We wanted to show that C1 inhibitor prepared this way would be safe and efficacious, particularly in patients for whom other therapies were not effective," says Dr. Frank, who now chairs the Department of Pediatrics at Duke University Medical Center. "Of much greater importance, they add, is the use of C1 inhibitor as a safe and effective treatment for acute attacks of HAE." HAE is caused by a defect in the gene that encodes the C1 inhibitor protein, an important regulator of a group of immune system enzymes known collectively as complement. http://www.niaid.nih.gov/Publications/dateline/0996/page8.htm      HAEI - International Patient Organization for C1 Inhibitor Deficiencies Our purpose is to join the efforts and experience of the global HAE community to achieve optimal standards of care and treatment for all those patients affected by C1 inhibitor deficiencies. HAEI International Patient Organization for C1 Inhibitor Deficiencies. C1 inhibitor deficiencies comprise Hereditary (HAE) and Acquired Angioedema (AAE) both rare and potentially fatal disorders resulting from a deficiency of a plasma protein known as C1 esterase inhibitor (C1 Inhibitor). http://www.haei.org/index.php?language=en      ARUP: C1-Esterase Inhibitor For additional interpretive data see C-1-Esterase Inhibitor in "ARUP's Guide to Clinical Laboratory Testing" http://www.arupcs.com/guides/ug/tests/0050140.jsp      03-09-01 - Baxter and Pharming Start Clinical Study with Recombinant Human C1 Inhibitor for Hereditary Angioedema Recombinant human C1 inhibitor has been developed through our breakthrough transgenic technology and we hope it will prove to be a highly effective therapeutic modality in hereditary angioedema. Baxter News - Baxter and Pharming Start Clinical Study with Recombinant Human C1 Inhibitor for Hereditary Angioedema 03-09-01 - Baxter and Pharming Start Clinical Study with Recombinant Human C1 Inhibitor for Hereditary Angioedema http://www.quickfind.baxter.com/about_baxter/news_room/news_releases/2001/03-09pharming.html      Allergic Diseases Resource Center: Upper Airway Oedema It is unclear as to why only a few individuals on ACE inhibitor therapy develop this potentially serious complication. inhibitor level, followed by a slower increase in C If angioedema occurs in a patient on an ACE inhibitor it should be stopped and an alternative drug from a different group of anti-hypertensive drugs should be used for therapy. http://www.worldallergy.org/professional/allergic_diseases_center/upperairwayoedema/index.shtml      Biological effects of the C1 inhibitor Background – The inhibitor of the C1 esterase (C1 INH) is deficient or dysfunctional in the plasma of individuals with hereditary angioedema (HAE). This thoughtful review describes a number of recent findings about C1 INH that may be of practical therapeutic relevance, particularly when the C1 INH preparation now used in Europe becomes more readily available elsewhere. C1 INH may also exert beneficial effects in certain other disorders such as endotoxin (septic) shock, hyperacute rejection and increased vascular permeability associated with thermal injury, IL-2 therapy and cardiopulmonary bypass. http://www.aaaai.org/aadmc/currentliterature/selectedarticles/2005archive/biological_effects.html      Administration of C1 Inhibitor Reduces Neutrophil Activation in Patients with Sepsis -- Zeerleder et al. 10 (4): 529 -- Clinical and Diagnostic Laboratory Immunology of C1 inhibitor therapy on cytokine levels was observed (data As described in the previous paragraph, C1 inhibitor therapy The effect of C1 inhibitor therapy on neutrophils http://cdli.asm.org/cgi/content/full/10/4/529      Potentiation of C1 Esterase Inhibitor by StcE, a Metalloprotease Secreted by Escherichia coli O157:H7 -- Lathem et al. 199 (8): 1077 -- The Journal of Experimental Medicine Human plasma kallikrein and C1 inhibitor form a complex possessing an epitope that is not detectable on the parent molecules: demonstration using a monoclonal antibody. C1 inhibitor-C1s complexes are internalized and degraded by the low density lipoprotein receptor-related protein. C1-Esterase inhibitor: an anti-inflammatory agent and its potential use in the treatment of diseases other than hereditary angioedema. http://www.jem.org/cgi/content/full/199/8/1077      ZLB BEHRING ANNOUNCES A CLINICAL STUDY OF C1-INHIBITOR INVESTIGATIONAL THERAPY FOR HEREDITARY ANGIOEDEMA The study, “Human C1 Esterase Inhibitor (C1-INH) Substitution in Subjects With Congenital C1-INH Deficiency and Acute Abdominal or Facial HAE Attacks,” is expected to begin in the third quarter of this year under an FDA-approved protocol. ZLB Behring C1-INH concentrate is a pasteurized, plasma-derived, intravenous therapy that currently can be used to treat and prevent acute HAE attacks in parts of the world where it is licensed. It is thought that by replacing the missing physiological C1 protein, this treatment can address the underlying mechanism of the disease by controlling the involved mediator systems. http://www.zlbplasma.com/zb/n25124pr27389358/ZLBBEHRINGANNOUNCESA.htm      Indian Pediatrics - Case Reports Exact mode of action of androgens in HAE has been not clear, however it is postulated that these drugs exert their therapeutic effect through increase in the hepatic synthesis of C1 inhibitor protein. It is further classified into Type I (lower production of C1 inhibitor proteins) and Type II (functional defect of C1 inhibitor with normal plasma levels)(1,2). Although mother had infrequent and very mild attacks which did not require any active intervention at the time of presentation, her serum C4 was decreased to 0.06 g/L and C1 esterase inhibitor was decreased to 29%. http://www.indianpediatrics.net/april2000/april-431-432.htm      HAEI - International Patient Organization for C1 Inhibitor Deficiencies Indeed, it was the failure of a poorly designed C1 inhibitor concentrate clinical trial that provided the impetus for the HAE scientific and patient communities to explore ways to open better lines of communication regarding future trial designs. HAEI International Patient Organization for C1 Inhibitor Deficiencies. By the time the C1 Inhibitor Deficiency Workshop was held in Budapest in May, 2003 there were proposals on the table of how to do this, and a final charter and mission statement were signed in a meeting held in Milan in October, 2003. http://www.haei.org/about-history.php?language=en§ion=2      C1 ESTERASE INHIBITOR (Antigenic) Patients who present with angioedema may have C1 inhibitor deficiency. Acquired C1 inhibitor deficiency can be distinguished from the hereditary form by measuring C1 q levels which are low in the acquired form but normal in the inherited. Some people synthesise an abnormal C1 inhibitor protein that is reactive in immunoassays, but devoid of the ability to inhibit the C1 enzyme, so that direct immunoassays for C1 inhibitor may not detect all cases. http://www.waikatodhb.co.nz/Laboratory/tests/immunology_tests/c1_esterase_inhibitor.htm      Frontiers in Bioscience 1, e15-25 March 1, 1996 C.E. Hack, A.C. Ogilia, B. Eisele, P.M. Jansen, J. Wagstaff and L.G Thijs: Initial studies on administration of C1-esterase inhibitor to patients with septic shock or with a vascular leakage syndrome induced by interleukin-2 therapy. The regulation of complement at C1 stage is abrogated in patients with genetic deficiency of C1-INH (hereditary angioneurotic edema; HANE), patients with cancer undergoing treatment with IL-2 and septic patients. From this point of view, some laboratories are developing low molecular weight synthetic inhibitors whereas others are focusing on the development of high molecular weight plasma or cell surface complement inhibitors in their natural or recombinant forms for therapeutic purposes. http://www.bioscience.org/1996/v1/e/asghar1/htmls/15-25.htm      MedlinePlus Medical Encyclopedia: C1 inhibiting factor Patients with hereditary angioedema are often depleted of C1 esterase inhibitor. This is a test that measures the concentration of an inhibitor of complement in the blood. The sample is then sent to the laboratory where the quantity of C1 inhibiting factor is measured. http://www.nlm.nih.gov/medlineplus/ency/article/003353.htm      A multicentre evaluation of the diagnostic efficiency of serological investigations for C1 inhibitor deficiency -- Gompels et al. 55 (2): 145 -- Journal of Clinical Pathology Behavior in vivo of normal and dysfunctional C1 inhibitor in normal subjects and patients with hereditary angioneurotic edema. for analysis from 907 patients suspected of C1 inhibitor deficiency. C1 inhibitor is a serine protease inhibitor that is involved http://jcp.bmjjournals.com/cgi/content/full/55/2/145      critical care Full text C1-Inhibitor substitution as ultima ratio therapy in septic shock: experience with 15 patients Plasma C1-Inhibitor (C1-INH) is the major inhibitor of both the classical pathway of complement system and contact activation. Vangerow B, et al.: Clinical improvement after administration of C1 -Inhibitor in two patients with SIRS and capillary leak syndrome. Hack CE, et al.: C1 -inhibitor substitution therapy in septic shock and in vascular leak syndrome induced by high doses of interleukin-2. http://ccforum.com/content/2/S1/P019      The effect of sequence variations within the coding region of the C1 inhibitor gene on disease expression and protein function in families with hereditary angio-oedema -- Cumming et al. 40 (10): 114 -- Journal of Medical Genetics Behavior in vivo of normal and dysfunctional C1 inhibitor in normal subjects and patients with hereditary angioneurotic edema. Complex formation was assessed by incubating dilutions of C1 inhibitor with an excess of C1s at 37°C for 5 min. Table 2 Summary of patient and family member details including results of clinical evaluation, measurement of plasma levels of C1 inhibitor, C4 and C3, symptoms, and score indicating level of clinical severity: 0 is unaffected and 10 is fatal. http://jmg.bmjjournals.com/cgi/content/full/40/10/e114      Recurrent abdominal pain{---}the forgotten cause -- 77 (904): 128 -- Postgraduate Medical Journal A biochemical abnormality in hereditary angioedema: absence of serum inhibitor of C1 esterase. The diagnosis of C1 INH deficiency is frequently missed, as happened in our patient over a period of 14 years. C1 INH deficiency is not associated with urticaria but the angio-oedema may be preceded by a serpiginous erythematous rash http://www.postgradmedj.com/cgi/content/full/77/904/128      Prevention of Polymerization of M and Z alpha 1-Antitrypsin (alpha 1-AT) with Trimethylamine N-Oxide . Implications for the Treatment of alpha 1-AT Deficiency -- Devlin et al. 24 (6): 727 -- American Journal of Respiratory Cell and Molecular Biology Crucial residues in the carboxy-terminal end of C1 inhibitor revealed by pathogenic mutants impaired in secretion or function. COOH-terminal substitutions in the serpin C1 inhibitor that cause loop overinsertion and subsequent multimerization. the serpin as a proteinase inhibitor and so is not an ideal therapy http://ajrcmb.atsjournals.org/cgi/content/full/24/6/727      {alpha}1-Proteinase inhibitor mutants with specificity for plasma kallikrein and C1s but not C1 -- Sulikowski et al. 11 (9): 2230 -- Protein Science {alpha}1-Proteinase inhibitor mutants with specificity for plasma kallikrein and C1s but not C1 -- Sulikowski et al. and one approach to therapy is to develop proteinase inhibitors of C1 in a hemolytic assay was observed. http://www.proteinscience.org/cgi/content/abstract/11/9/2230      Pharming outsources production of recombinant C1 inhibitor for HAE to DSM Attacks of angioedema can be effectively treated with intravenous administration of C1 inhibitor purified from human blood. HAE is a genetic disorder, which is caused by C1 inhibitor deficiency. Pharming Group N.V. based in Leiden has signed the agreement with DSM Biologics for purification of its lead product, recombinant human C1 inhibitor, rhC1INH, with a view to launching the product on the market. http://www.advancesinlifescience.com/news_110.htm      Modern Complement Analysis -- Kirschfink and Mollnes 10 (6): 982 -- Clinical and Diagnostic Laboratory Immunology the classical pathway (C1rs-C1 inhibitor, C4d, and C4bc), the Pathogenetic and clinical aspects of C1 inhibitor deficiency. Fifteen percent of patients with HAE have a dysfunctional protein http://cdli.asm.org/cgi/content/full/10/6/982      Oxandrin Paper Long term treatment of C1 inhibitor deficiency with epsilon-aminocaproic acid in two patients. Hereditary angioedema (HAE) is a rare autosomal dominant disorder typified by a deficiency or dysfunction of C1-esterase inhibitor (C1-INH), and characterized clinically by swelling of the extremities, severe episodic abdominal pain, and sometimes upper airway obstruction. Her father was shown to have a defect on his C1-INH gene consisting of a single nucleotide insertion at 4460 (S198) leading to a stop signal 3 codons downstream, and resulting in a truncated non-functional C1 inhibitor protein. http://www.hereditaryangioedema.com/oxscott.htm      The Functional Integrity of the Serpin Domain of C1-inhibitor Depends on the Unique N-terminal Domain, as Revealed by a Pathological Mutant -- Bos et al. 278 (32): 29463 -- Journal of Biological Chemistry C1-Inh is the major inhibitor of C1s and C1r of the classical C1-inhibitor (C1-Inh) is a serine protease inhibitor (serpin) pathway of complement and also an inhibitor of the contact http://www.jbc.org/cgi/content/full/278/32/29463

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