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Inhibitor Deficiency (inhibitor + deficiency)
Kinds of Inhibitor Deficiency Selected AbstractsC1 inhibitor level on neonatal sepsis and its relations with clinical findingsJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 3 2010Anil 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] The risk of recurrent venous thromboembolism among patients with high factor IX levelsJOURNAL OF THROMBOSIS AND HAEMOSTASIS, Issue 1 2003A. Weltermann Summary., High factor IX (FIX) is a risk factor of deep vein thrombosis. The impact of high FIX on the risk of recurrent venous thrombosis is unknown. We prospectively followed 546 patients after anticoagulation for a first spontaneous venous thromboembolism. Patients with a natural coagulation inhibitor deficiency, lupus anticoagulant or cancer were excluded. At 3 years, the likelihood of recurrence was 23% among patients with high FIX (exceeding the 75th percentile) compared with 11% among patients with lower levels. Among patients with high FIX, the relative risk of recurrence was 2.2 (95% CI: 1.3,3.6) before and was 1.6 (95% CI: 1.0,2.8) after adjustment for age, gender, duration of anticoagulation, FV Leiden, FII G20210A, high FVIII and hyperhomocysteinemia. Compared with patients with low factor IX (< 138 IU dL,1) and low FVIII (, 234 IU dL,1), the relative risk of recurrence was 1.5 among patients with high FIX and low FVIII, 2.7 among patients with low FIX and high FVIII and 6.6 among patients with high FIX and high FVIII. High levels of FIX confer an increased risk of recurrent venous thromboembolism and enhance the risk of recurrence among patients with high FVIII. [source] Angioedema from angiotensin-converting enzyme (ACE) inhibitor treated with complement 1 (C1) inhibitor concentrateACTA ANAESTHESIOLOGICA SCANDINAVICA, Issue 1 2006E. 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] A regional audit of experience managing C1 esterase inhibitor deficiency , cause of a potential airway emergencyANAESTHESIA, Issue 1 2010C. S. Hawe No abstract is available for this article. [source] Is acquired C1 inhibitor deficiency associated with lupus a distinct disease entity?ARTHRITIS & RHEUMATISM, Issue 10 2002Comment on the article by Cacoub et al No abstract is available for this article. [source] New insights into hereditary angio-oedema: Molecular diagnosis and therapyAUSTRALASIAN JOURNAL OF DERMATOLOGY, Issue 3 2010Nikoletta Nagy ABSTRACT Hereditary angio-oedema (HAE) is a rare but potentially life-threatening condition. Three types are now recognized. Types I and II HAE involve mutations in the C1NH (SERPING1) gene, encoding the C1 inhibitor protein, whereas type III HAE involves mutations in the F12 gene, encoding coagulation factor XII (Hageman factor). They share a common final pathway leading to increased bradykinin formation. HAE must be distinguished from acquired angio-oedema with C1 esterase inhibitor deficiency, angiotensin-converting enzyme inhibitor-induced angio-oedema and the much more common histaminergic angio-oedema, occurring with or without weals. Understanding the pathogenesis of HAE is leading to the introduction of new therapies that target the bradykinin receptor or inhibit kallikrein activity, innovations that will hopefully reduce morbidity and mortality in this group of severe genetic disease. [source] C1 inhibitor deficiency: consensus documentCLINICAL & EXPERIMENTAL IMMUNOLOGY, Issue 3 2005M. 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] | ||||||||||