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Hereditary Angioedemas

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Inherited disorders that are characterized by subcutaneous and submucosal EDEMA in the upper RESPIRATORY TRACT and GASTROINTESTINAL TRACT.

Also Known As:

Angioedemas, Hereditary; Hereditary Angioedema; Hereditary Angioneurotic Edema; Angioneurotic Edema, Hereditary; Angioneurotic Edemas, Hereditary; Edema, Hereditary Angioneurotic; Edemas, Hereditary Angioneurotic; Hereditary Angioneurotic Edemas; Angioedema, Hereditary

Networked: 694 relevant articles (42 outcomes, 49 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Angioedema
2.	Edema
3.	Urticaria (Hives)
4.	Rare Diseases (Rare Disease)
5.	Systemic Lupus Erythematosus (Libman-Sacks Disease)

Experts

1.	Farkas, Henriette: 30 articles (10/2015 - 12/2003)
2.	Bork, Konrad: 22 articles (05/2015 - 05/2003)
3.	Craig, Timothy J: 22 articles (05/2015 - 05/2008)
4.	Varga, Lilian: 20 articles (10/2015 - 12/2003)
5.	Bernstein, Jonathan A: 20 articles (05/2015 - 10/2009)
6.	Li, H Henry: 13 articles (11/2015 - 06/2010)
7.	Csuka, Dorottya: 13 articles (10/2015 - 03/2010)
8.	Füst, George: 13 articles (07/2013 - 04/2005)
9.	Bouillet, Laurence: 12 articles (09/2015 - 07/2006)
10.	Riedl, Marc: 11 articles (07/2015 - 05/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hereditary Angioedemas:

1.	icatibantIBA 08/01/2011 - "A short-cut review was carried out to establish whether icatibant is effective in the treatment of hereditary angioedema. " 06/01/2007 - "Icatibant was effective in treating acute attacks of hereditary angioedema. " 11/01/2012 - "Clinical efficacy of icatibant in the treatment of acute hereditary angioedema during the FAST-3 trial." 01/01/2010 - "Across both FAST-1 and -2, the efficacy of subcutaneous icatibant 30 mg in the treatment of laryngeal episodes of hereditary angioedema was generally consistent with that seen for abdominal and cutaneous episodes, with a median time to first symptom improvement of 0.6-1.0 hours. " 01/01/2004 - "Positive results from an icatibant formulation comparative study, in patients with acute attacks of hereditary angioedema, were announced in August 2004; IV and SC formulations showed no difference in efficacy and safety. "
2.	Danazol (Azol)FDA LinkGeneric 01/01/1988 - "A 24-year-old woman, having familial hereditary angioneurotic edema (HAE) treated with danazol, developed an anti-Ro-positive lupus featured by a mild erythematous eruption that seemed initially to decrease and then worsened under danazol, and dramatically improved after danazol was withdrawn. " 05/01/1980 - "Danazol is an effective agent for the prophylaxis of the recurrent attacks of mucocutaneous and visceral swelling which occur in patients with hereditary angioedema (HAE). " 04/01/1979 - "Danazol, an attentuated androgen, has been suggested as an effective agent for the prophylaxis of attacks of hereditary angioedema. " 04/01/1999 - "This study evaluated the efficacy of short-term danazol prophylaxis in hereditary angioedema patients undergoing maxillofacial or dental procedures. " 03/01/2011 - "Long-term efficacy of danazol treatment in hereditary angioedema."
3.	Complement C1 Inhibitor Protein (C1 Esterase Inhibitor)IBA 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."
4.	Complement System Proteins (Complement)IBA 03/01/1999 - "After C1 inhibitor has for many years been proven beneficial in acute treatment of hereditary angioedema, a growing number of reports now suggests that C1 inhibitor provides an effective means to protect against complement-mediated inflammatory tissue damage. " 09/01/2007 - "Based on the presence of the typical clinical features and the positive results on the complement tests, we diagnosed hereditary angioedema. " 03/01/1992 - "Complement study of the patient's family members revealed that he was one of 5 patients in 3 generations with hereditary angioneurotic edema (HANE). " 07/01/1984 - "Studies of complement autoactivatability in hereditary angioedema: direct relationship to functional C-1-INA and the effect of classical pathway activators." 05/01/1978 - "Since the genes for several disorders of the complement system have been found to be linked to the HLA loci on chromosome 6, studies of the inhibitor of the activated first component of complement (Cl INH) and HLA in two families with hereditary angioedema (HAE) were undertaken. "
5.	BradykininIBA 01/01/2016 - "Emerging evidence suggests that FFP and medications approved for management of acute attacks of hereditary angioedema, another bradykinin-mediated event, may be effective for use in ACEI-IA. " 09/01/2008 - "Studies of the mechanisms of bradykinin generation in hereditary angioedema plasma." 10/15/2015 - "Deficiency of C1-INH is associated with hereditary angioedema (HAE), an autosomal inherited disease characterized by swelling attacks caused by elevated levels of bradykinin. " 01/01/2015 - "Bradykinin, secondary to kallikrein-kinin system activation is the key mediator of hereditary angioedema. " 01/01/2015 - "Bradykinin-mediated subtypes are more closely related to hereditary angioedema than the other forms. "
6.	Stanozolol (Winstrol)FDA Link 12/01/1987 - "Thus, stanozolol appears to be a safe and effective agent for management of hereditary angioedema when patients are continually monitored to define the minimal effective dose or the feasibility of stopping the drug." 09/01/2007 - "Stanozolol is a safe and effective drug for the long-term management of hereditary angioedema. " 09/01/2007 - "Stanozolol may be used in the long-term treatment of patients with hereditary angioedema provided such patients are closely supervised with routine clinical, biochemical, and radiologic assessments." 09/01/2007 - "To assess the frequencies of various side effects of the attenuated androgen stanozolol in a population of patients with hereditary angioedema treated for 20 to 40 years. " 09/01/2007 - "Hereditary angioedema: Safety of long-term stanozolol therapy."
7.	Bradykinin Receptors (Bradykinin Receptor)IBA 01/01/2014 - "Icatibant, an inhibitor of bradykinin receptor 2, for hereditary angioedema attacks: prospective experimental single-cohort study." 01/01/2012 - "Icatibant, a first-in-class B2 bradykinin receptor antagonist, appears to have a favorable efficacy and safety profile for the treatment of acute attacks of hereditary angioedema in adults. " 08/01/2011 - "The clinical bottom line is there is promising evidence for the use of the bradykinin receptor antagonist icatibant for the treatment of acute attacks of hereditary angioedema." 09/01/2010 - "Hereditary angioedema therapy: kallikrein inhibition and bradykinin receptor antagonism." 08/05/2010 - "Icatibant, a new bradykinin-receptor antagonist, in hereditary angioedema."
8.	SteroidsIBA 02/01/1986 - "[Prolonged preventive treatment of hereditary angioneurotic edema with anabolic androgenic steroids]." 01/01/1980 - "Prophylactic treatment of hereditary angioneurotic edema with anabolic steroids." 07/01/1991 - "However, several additional diagnostic possibilities should be pursued in patients with angioedema, such as hereditary angioedema caused by C1-esterase inhibitor deficiency, because anabolic steroids are effective in the treatment of these conditions." 11/01/1994 - "Serial laboratory examinations revealed: [table: see text] We believe that the occurrence of abdominal pain, facial swelling, and difficulty swallowing suggests that this patient may have experienced a mild, generalized flare of hereditary angioedema during the postoperative period in spite of prophylactic therapy with both anabolic steroids and C1 INH concentrate. " 03/01/1989 - "Recognition of their side-effects and complications, comparison of their efficacy with other forms of therapy and more critical evaluation of their value has reduced the range of their definitive indications to a limited number of relatively uncommon situations; these comprise primary hyperplasminaemia, menorrhagia in women in whom oestrogens are contraindicated or in those with von Willebrand's disease, severe traumatic hyphaema, dental extraction in haemophiliacs and hereditary angioedema in patients in whom treatment with anabolic steroids is contraindicated. "
9.	ecallantide FDA Link 01/01/2011 - "Ecallantide is a novel treatment for attacks of hereditary angioedema due to C1 inhibitor deficiency." 03/01/2013 - "Efficacy and safety of ecallantide in treatment of recurrent attacks of hereditary angioedema: open-label continuation study." 01/01/2012 - "Prospective, double-blind, placebo-controlled trials of ecallantide for acute attacks of hereditary angioedema." 07/01/2011 - "Ecallantide (DX-88) for acute hereditary angioedema attacks: integrated analysis of 2 double-blind, phase 3 studies." 08/05/2010 - "In this double-blind, placebo-controlled trial, patients with hereditary angioedema presenting with an acute attack were randomly assigned, in a 1:1 ratio, to receive subcutaneous ecallantide, at a dose of 30 mg, or placebo. "
10.	Acquired angioedemaIBA 05/01/1997 - "We have studied 19 patients with hereditary angioedema (HAE) during remission, 5 HAE patients during acute attacks, and 6 patients with acquired angioedema (AAE) during remission and during seven attacks. " 04/01/2015 - "We aimed to study the subgroups of hereditary angioedema with FXII mutation (FXII-HAE), unknown genetic defect (U-HAE) and idiopathic non-histaminergic acquired angioedema (InH-AAE). " 03/01/1995 - "The study included 85 patients with hereditary angioedema and 4 with acquired angioedema. " 03/01/2014 - "In contrast to hereditary angioedema, which usually manifests in childhood, acquired angioedema is broadly recognized to affect people older than 40 years. " 01/01/2014 - "Hereditary angioedema (HAE) and acquired angioedema (AAE) are rare life-threatening conditions caused by deficiency of C1 inhibitor (C1INH). "

Therapies and Procedures

1.	Self Administration (Administration, Self) 11/01/2014 - "Hereditary angioedema: children should be considered for training in self-administration." 01/01/2013 - "Self-administration of therapy can help hereditary angioedema (HAE) patients regain control of their disease or reduce its impact and improve the quality of their lives. " 01/01/2013 - "Recent advances in hereditary angioedema self-administration treatment: summary of an International Hereditary Angioedema Expert Meeting." 01/01/2013 - "Results from a 16-question survey about self-administration of hereditary angioedema (HAE) therapy, administered in Europe, Canada and the USA, were used to guide discussion at an international HAE expert meeting. " 01/01/2013 - "Current status of implementation of self-administration training in various regions of Europe, Canada and the USA in the management of hereditary angioedema."
2.	Transplants (Transplant) 07/20/2007 - "These results may help to improve therapeutic C1 inhibitor preparations used in the treatment of hereditary angioedema, organ transplant rejection, and heart attack." 06/01/2010 - "Perioperative management of a patient with hereditary angioedema during off-pump coronary artery bypass graft surgery." 08/01/2002 - "Successful off-pump coronary artery bypass graft surgery in a patient with hereditary angioedema." 06/01/2010 - "The case of a patient with hereditary angioedema (HAE), a rare, life-threatening disorder caused by reduced activity of the C1 esterase inhibitor, and requiring off-pump coronary artery bypass graft (OP-CABG) surgery, is presented. " 09/01/2007 - "Important directions are the search for complement inhibitors that (a) interfere with the rejection of transplants; (b) can replace C1 inhibitor in hereditary angioedema, and (c) have a high anti-inflammatory activity in the therapy of rheumatic diseases, diabetes, and other autoimmune disorders. "
3.	Therapeutics 09/01/2010 - "The recent approvals of anti-C5 for the treatment of paroxysmal nocturnal hemoglobinuria as well as of C1 inh for the treatment of hereditary angioedema beyond European countries have provided a resurgence of interest in the potential of complement therapeutics for the treatment of disease." 12/01/2015 - "In view of the clinical experience acquired with drugs licensed for the treatment of rare diseases such as hereditary angioedema and paroxysmal nocturnal hemoglobinuria, growing evidence supports the safety and efficacy of complement therapeutics in restoring immune balance and preventing aggravation of clinical outcomes. "
4.	Injections 08/05/2010 - "The second study was a crossover trial involving 22 subjects with hereditary angioedema that compared prophylactic twice-weekly injections of nanofiltered C1 inhibitor concentrate (1000 units) with placebo during two 12-week periods. " 01/01/2011 - "Hereditary angioedema: long-term treatment with one or more injections of C1 inhibitor concentrate per week." 11/01/2013 - "The For Angioedema Subcutaneous Treatment (FAST)-2, a phase III, double-blind, randomized, multicenter, placebo-controlled study (ClinicalTrials.gov identifier: NCT00500656), established the efficacy and safety of single injections of icatibant, a bradykinin B₂ receptor antagonist, in the treatment of hereditary angioedema (HAE) attacks. "
5.	Oral Surgery (Maxillofacial Surgery) 05/01/2015 - "Hereditary angioedema and anxiety in oral surgery: a case series report." 09/01/2010 - "Hereditary angioedema in oral surgery: overview of the clinical picture and report of a case." 01/01/1993 - "Hereditary angioedema: uncomplicated maxillofacial surgery using short-term C1 inhibitor replacement therapy." 09/01/1988 - "[Hereditary angioneurotic edema in oral surgery]." 12/01/1979 - "A case report in which the management of a patient with hereditary angioneurotic edema requiring oral surgery is presented. "