Complement C1 Inhibitor Protein (C1 Esterase Inhibitor)

An endogenous 105-kDa plasma glycoprotein produced primarily by the LIVER and MONOCYTES. It inhibits a broad spectrum of proteases, including the COMPLEMENT C1R and the COMPLEMENT C1S proteases of the CLASSICAL COMPLEMENT PATHWAY, and the MANNOSE-BINDING PROTEIN-ASSOCIATED SERINE PROTEASES. C1-INH-deficient individuals suffer from HEREDITARY ANGIOEDEMA TYPES I AND II.

Also Known As:

Networked: 277 relevant articles (13 outcomes, 22 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Craig, Timothy J: 14 articles (05/2015 - 10/2008)
2.	Bernstein, Jonathan A: 12 articles (05/2015 - 10/2009)
3.	Bork, Konrad: 7 articles (05/2015 - 05/2003)
4.	Machnig, Thomas: 6 articles (05/2015 - 07/2012)
5.	Keinecke, Heinz-Otto: 6 articles (04/2014 - 10/2009)
6.	Fitts, David: 6 articles (01/2014 - 01/2012)
7.	Li, H Henry: 5 articles (11/2015 - 07/2012)
8.	Bewtra, Againdra K: 5 articles (07/2012 - 10/2009)
9.	Wasserman, Richard L: 5 articles (07/2012 - 10/2009)
10.	Riedl, Marc: 4 articles (07/2015 - 05/2009)

Related Diseases

1.	Hereditary Angioedemas 10/01/2009 - "C1 esterase inhibitor concentrate given intravenously at a dose of 20 U/kg is an effective and safe treatment for acute abdominal and facial attacks in patients with hereditary angioedema, with a rapid onset of relief." 01/01/2011 - "One such product, C1 esterase inhibitor, has now been shown in a rigorous double-blind study to be effective in prevention of hereditary angioedema attacks and has been approved by the US Federal Drug Administration for prophylaxis of the disease. " 07/01/2012 - "Placebo-controlled studies established the efficacy of replacement therapy with C1 esterase inhibitor (C1-INH) concentrate for treating single acute hereditary angioedema (HAE) attacks, but only limited data from prospective studies are available on repeated treatment of successive HAE attacks. " 05/01/2015 - "Although treatment with C1 esterase inhibitor (C1-INH) concentrate is well established for hereditary angioedema (HAE) attacks in general, data that assess its efficacy for cutaneous attack treatment are sparse. " 11/01/2015 - "Safety and efficacy of C1 esterase inhibitor for acute attacks in children with hereditary angioedema."
2.	Angioedema 01/01/2011 - "In addition to a detailed comprehensive medical history, laboratory data and imaging studies are required to confirm a diagnosis of angioedema due to C1 esterase inhibitor deficiency." 09/01/2014 - "A detailed drug and family history, screening blood work for C1 esterase inhibitor deficiency when indicated, and prompt management of angioedema based on presumed mechanism of swelling are crucial steps in managing undifferentiated angioedema in ED. " 06/01/2013 - "To evaluate the cost-utility of Ruconest(®) (conestat alfa) financed from public funds within the newly introduced therapeutic health program compared with Berinert(®) P (human C1 esterase inhibitor) in the treatment of acute angioedema attacks in adults with HAE. " 03/01/2011 - "Recombinant human C1 esterase inhibitor (rhC1INH) (Ruconest(®), Pharming) is a new drug developed for the relief of symptoms occurring in patients with angioedema due to C1-inhibitor deficiency. " 01/01/2011 - "Isolated angioedema of the bowel due to C1 esterase inhibitor deficiency: a case report and review of literature."
3.	Urticaria (Hives) 04/01/2000 - "She had recurrent urticaria, C1' esterase inhibitor deficiency and radiographic studies showed Crohn's disease of the ileum. " 01/01/2014 - "Angioedema is the swelling of the mucosal membranes as a variant of urticaria induced by hereditary C1 esterase inhibitor enzyme deficiency, certain foods, or drugs. " 03/01/2006 - "It has been reported to occur in association with urticaria in the context of Schnitzler's syndrome and also has been observed to occur in angioedema with acquired C1 esterase inhibitor deficiency. " 09/01/1995 - "The aim of the study was to evaluate the concentration and activity of C1 esterase inhibitor (C1 INH) in patients with aspirin-sensitive urticaria. " 01/01/1993 - "The aim of the study was to evaluate concentration and activity of C1 esterase inhibitor (C1 INH) in patients with aspirin-sensitive urticaria. "
4.	Myocardial Reperfusion Injury 08/01/2007 - "The inflammatory cascade has been hypothesized to be an important mechanism of post-ischaemic myocardial reperfusion injury and several studies demonstrated that C1 esterase inhibitor (C1-INH) is effective in post-ischaemia myocardial protection. "
5.	Lymphoma (Lymphomas) 10/01/2002 - "Regression of acquired C1 esterase inhibitor deficiency associated with spontaneous partial remission of lymphoma has not been reported previously." 10/01/2002 - "A 52 year old woman with marginal zone lymphoma developed recurrent episodes of angio-oedema and was found to have C1 esterase inhibitor deficiency. " 10/01/2002 - "Spontaneous regression of acquired C1 esterase inhibitor deficiency associated with splenic marginal zone lymphoma presenting with recurrent angio-oedema." 05/01/1989 - "[Acquired C1 esterase inhibitor deficiency, Gougerot-Sjögren syndrome and B lymphoma]." 06/01/1985 - "About 20 cases of acquired C1 esterase inhibitor deficiency have been reported in association with malignant lymphomas. "

Related Drugs and Biologics

1.	Danazol (Azol)
2.	icatibant
3.	Deficiency of C1 esterase inhibitor
4.	Complement System Proteins (Complement)
5.	Antithrombin III
6.	Bradykinin B2 Receptor
7.	Histamine Antagonists (Antihistamines)
8.	Fibrin
9.	Adrenal Cortex Hormones (Corticosteroids)
10.	omalizumab (Xolair)

Related Therapies and Procedures

1.	Premedication
2.	Stem Cell Transplantation
3.	Self Administration (Administration, Self)
4.	Intravenous Infusions
5.	Airway Management