C1 Inhibitor (c1 + inhibitor)

Distribution by Scientific Domains
Medical Sciences71%

Terms modified by C1 Inhibitor

  • c1 inhibitor deficiency
    		•

    Selected Abstracts

    Erratum: Detection of C1 inhibitor (SERPING1/C1NH) mutations in exon 8 in patients with hereditary angioedema: evidence for 10 novel mutations,,

    HUMAN MUTATION, Issue 1 2003
    Alvaro Blanch
    The original article to which this Erratum refers was published in Human Mutation 20:405,406 Human Mutation (2002) 20(5) 405,406 The authors regret that there was an error in Table 2 on Page 4 of the original article. In patient DS, the nucleotide change 16838C>T is not correct. It should be 16838G>A, since this was the mutation at the antisense change. [source]

    C1 inhibitor level on neonatal sepsis and its relations with clinical findings

    JOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 3 2010
    Anil Tapisiz
    Background: Generalised oedema is a frequent finding during neonatal sepsis, but its aetiology remains uncertain. Objective: The objective of this study was to measure functional C1 inhibitor (fC1 inh) levels in newborns with culture-proven sepsis, compare the results with age- and gestational age (GA)-matched controls and correlate the results with the clinical course of the patients during infection, with regard to vascular leak and oedema formation. Methods: Newborns with blood culture-proven sepsis were included and samples for C1 inh levels were obtained before the beginning of antibiotic therapy and on the 3rd day of treatment. Body weight, urine output and other treatment modalities including volume boluses were recorded. Oedema formation as a sign of vascular leak was determined by calculating percent weight change over time. Age- and GA-matched newborns without infection were used as controls. Results: No difference was observed between the patient and the control groups concerning fC1 inh levels. Percent weight change in the patient group was not correlated with the C1 inh levels. Conclusion: Despite studies suggesting the role of C1 inhibitor deficiency in vascular leak during sepsis in adults, there is no information in the literature regarding the C1 inh levels of healthy or septic newborns to date. In this study, fC1 inh levels were no different than controls, necessitating the consideration of other factors causing vascular leak and oedema during neonatal sepsis. [source]

    Angioedema from angiotensin-converting enzyme (ACE) inhibitor treated with complement 1 (C1) inhibitor concentrate

    ACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 1 2006
    E. W. Nielsen
    Background:, Up to seven in every 1000 patients experience angioedema from angiotensin-converting enzyme (ACE) inhibitors, even after many years of use. In 2003, every 20th Norwegian used an ACE inhibitor. Case report:, A 61-year-old woman with chronic obstructive pulmonary disease and a past acute myocardial infarction had used 7.5 mg of ramipril daily for the past 7 years. She also used acetylsalicylic acid, simvastatin, theophylline and salmeterol. One night she woke up with edema of the tongue. On hospital arrival, 250 mg of hydrocortisone and 5 mg of dexchlorpheniramine were given intravenously (i.v.) and 0.3 mg of epinephrine was given subcutaneously (s.c.). The edema of the tongue progressed over the next 8 h and made the tongue protrude. Fiberscopy revealed glassy edema of the arytenoids. Inspiratory stridor was heard and the patient could not speak. She became increasingly uneasy and restless. Berinert® complement 1 (C1) inhibitor concentrate (1500 units) was administered i.v. Over the following 20 min, stridor gradually subsided, the patient calmed and she was able to talk. Discussion:, ACE inhibitor-provoked angioedema shares many clinical features with hereditary angioedema (HAE), including a limited effect of steroids, antihistamines and epinephrine. HAE, caused by excess bradykinin formation as a result of C1 inhibitor deficiency, usually has its laryngeal edema effectively reversed by C1 inhibitor in less than 0.5 h. Although patients experiencing ACE inhibitor-provoked angioedema have normal C1 inhibitor values, as in our patient, excess bradykinin is probably important as ACE breaks down bradykinin. It is unknown why ACE inhibitor-provoked angioedema appears in some and sometimes after many years of use. Conclusion:, We believe that C1 inhibitor was effective in reversing the ACE inhibitor-induced angioedema in our patient. [source]

    Mining biomarkers in human sera using proteomic tools

    PROTEINS: STRUCTURE, FUNCTION AND BIOINFORMATICS, Issue 1 2004
    Rulin Zhang
    Abstract One of the major difficulties in mining low abundance biomarkers from serum or plasma is due to the fact that a small number of proteins such as albumin, ,2-macroglobulin, transferrin, and immunoglobulins, may represent as much as 80% of the total serum protein. The large quantity of these proteins makes it difficult to identify low abundance proteins in serum using traditional 2-dimensional electrophoresis. We recently used a combination of multidimensional liquid chromatography and gel electrophoresis coupled to matrix-assisted laser desorption/ionization-quadrupole-time of flight and Ion Trap liquid chromatography-tandem mass spectrometry to identify protein markers in sera of Alzheimer's disease (AD), insulin resistance/type-2 diabetes (IR/D2), and congestive heart failure (CHF) patients. We identified 8 proteins that exhibit higher levels in control sera and 36 proteins that exhibit higher levels in disease sera. For example, haptoglobin and hemoglobin are elevated in sera of AD, IR/D2, and CHF patients. The levels of several other proteins including fibrinogen and its fragments, alpha 2-macroglobulin, transthyretin, pro-platelet basic protein, protease inhibitors clade A and C, as well as proteins involved in the classical complement pathway such as complement C3, C4, and C1 inhibitor, were found to differ between IR/D2 and control sera. The sera levels of proteins, such as the 10 kDa subunit of vitronectin, alpha 1-acid glycoprotein, apolipoprotein B100, fragment of factor H, and histidine-rich glycoprotein were observed to be different between AD and controls. The differences observed in these biomarker candidates were confirmed by Western blot and the enzyme-linked immunosorbent assay. The biological meaning of the proteomic changes in the disease states and the potential use of these changes as diagnostic tools or for therapeutic intervention will be discussed. [source]

    Recombinant C1 inhibitor in brain ischemic injury,

    ANNALS OF NEUROLOGY, Issue 3 2009
    Raffaella Gesuete BD
    Objective C1 inhibitor (C1-INH) is an endogenous inhibitor of complement and kinin systems. We have explored the efficacy and the therapeutic window of the recently available human recombinant (rh) C1-INH on ischemic brain injury and investigated its mechanism of action in comparison with that of plasma-derived (pd) C1-INH. Methods rhC1-INH was administered intravenously to C57Bl/6 mice undergoing transient or permanent ischemia, and its protective effects were evaluated by measuring infarct volume and neurodegeneration. The binding profiles of rhC1-INH and pdC1-INH were assessed in vitro using surface plasmon resonance. Their localization in the ischemic brain tissue was determined by immunohistochemistry and confocal analysis. The functional consequences of rhC1-INH and pdC1-INH administration on complement activation were analyzed by enzyme-linked immunosorbent assay on plasma samples. Results rhC1-INH markedly reduced cerebral damage when administered up to 18 hours after transient ischemia and up to 6 hours after permanent ischemia, thus showing a surprisingly wide therapeutic window. In vitro rhC1-INH bound mannose-binding lectin (MBL), a key protein in the lectin complement pathway, with high affinity, whereas pdC1-INH, which has a different glycosylation pattern, did not. In the ischemic brain, rhC1-INH was confined to cerebral vessels, where it colocalized with MBL, whereas pdC1-INH diffused into the brain parenchyma. In addition, rhC1-INH was more active than pdC1-INH in inhibiting MBL-induced complement activation. Interpretation rhC1-INH showed a surprisingly wider time window of efficacy compared with the corresponding plasmatic protein. We propose that the superiority of rhC1-INH is due to its selective binding to MBL, which emerged as a novel target for stroke treatment. Ann Neurol 2009;66:332,342 [source]

    C1 inhibitor deficiency: consensus document

    CLINICAL & EXPERIMENTAL IMMUNOLOGY, Issue 3 2005
    M. M. Gompels
    Summary We present a consensus document on the diagnosis and management of C1 inhibitor deficiency, a syndrome characterized clinically by recurrent episodes of angio-oedema. In hereditary angio-oedema, a rare autosomal dominant condition, C1 inhibitor function is reduced due to impaired transcription or production of non-functional protein. The diagnosis is confirmed by the presence of a low serum C4 and absent or greatly reduced C1 inhibitor level or function. The condition can cause fatal laryngeal oedema and features indistinguishable from gastrointestinal tract obstruction. Attacks can be precipitated by trauma, infection and other stimulants. Treatment is graded according to response and the clinical site of swelling. Acute treatment for severe attack is by infusion of C1 inhibitor concentrate and for minor attack attenuated androgens and/or tranexamic acid. Prophylactic treatment is by attenuated androgens and/or tranexamic acid. There are a number of new products in trial, including genetically engineered C1 esterase inhibitor, kallikrein inhibitor and bradykinin B2 receptor antagonist. Individual sections provide special advice with respect to diagnosis, management (prophylaxis and emergency care), special situations (childhood, pregnancy, contraception, travel and dental care) and service specification. [source]