The Experts below are selected from a list of 282 Experts worldwide ranked by ideXlab platform
Tobias Suiter - One of the best experts on this subject based on the ideXlab platform.
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recombinant human c1 Esterase Inhibitor as short term prophylaxis in patients with hereditary angioedema
The Journal of Allergy and Clinical Immunology: In Practice, 2020Co-Authors: Anna Valerieva, Sladjana Andrejevic, Roman Hakl, Maria Staevska, Milos Jesenak, Katarina Hrubiskova, Marta Sobotkova, Radana Zachova, Tobias SuiterAbstract:Hereditary angioedema (HAE), an inherited deficiency offunctional C1 Esterase Inhibitor (C1-INH), is characterized byrecurrent episodes of disabling and often painful swelling insubcutaneous and/or submucosal tissues.1HAE attacks aregenerally unpredictable, but triggers for an attack can includehaving a dental or medical procedure (eg, surgery), other trauma,or stress. A preemptive management plan for patients under-going these types of situations may reduce the risk of HAE at-tacks. Recommendations include administration of short-termprophylaxis in patients with HAE before invasive medical pro-cedures, especially those involving the upper airways or digestivetract, with C1-INH concentrate typically the medication ofchoice.
Milos Jesenak - One of the best experts on this subject based on the ideXlab platform.
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recombinant human c1 Esterase Inhibitor as short term prophylaxis in patients with hereditary angioedema
The Journal of Allergy and Clinical Immunology: In Practice, 2020Co-Authors: Anna Valerieva, Sladjana Andrejevic, Roman Hakl, Maria Staevska, Milos Jesenak, Katarina Hrubiskova, Marta Sobotkova, Radana Zachova, Tobias SuiterAbstract:Hereditary angioedema (HAE), an inherited deficiency offunctional C1 Esterase Inhibitor (C1-INH), is characterized byrecurrent episodes of disabling and often painful swelling insubcutaneous and/or submucosal tissues.1HAE attacks aregenerally unpredictable, but triggers for an attack can includehaving a dental or medical procedure (eg, surgery), other trauma,or stress. A preemptive management plan for patients under-going these types of situations may reduce the risk of HAE at-tacks. Recommendations include administration of short-termprophylaxis in patients with HAE before invasive medical pro-cedures, especially those involving the upper airways or digestivetract, with C1-INH concentrate typically the medication ofchoice.
Anna Valerieva - One of the best experts on this subject based on the ideXlab platform.
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recombinant human c1 Esterase Inhibitor as short term prophylaxis in patients with hereditary angioedema
The Journal of Allergy and Clinical Immunology: In Practice, 2020Co-Authors: Anna Valerieva, Sladjana Andrejevic, Roman Hakl, Maria Staevska, Milos Jesenak, Katarina Hrubiskova, Marta Sobotkova, Radana Zachova, Tobias SuiterAbstract:Hereditary angioedema (HAE), an inherited deficiency offunctional C1 Esterase Inhibitor (C1-INH), is characterized byrecurrent episodes of disabling and often painful swelling insubcutaneous and/or submucosal tissues.1HAE attacks aregenerally unpredictable, but triggers for an attack can includehaving a dental or medical procedure (eg, surgery), other trauma,or stress. A preemptive management plan for patients under-going these types of situations may reduce the risk of HAE at-tacks. Recommendations include administration of short-termprophylaxis in patients with HAE before invasive medical pro-cedures, especially those involving the upper airways or digestivetract, with C1-INH concentrate typically the medication ofchoice.
Douglas T. Johnston - One of the best experts on this subject based on the ideXlab platform.
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Hereditary and Acquired Complement Component 1 Esterase Inhibitor Deficiency: A Review for the Hematologist
Acta haematologica, 2012Co-Authors: Marco Cicardi, Douglas T. JohnstonAbstract:Hereditary angioedema (HAE), a rare autosomal dominant disorder, was first described in the late 19th century. The disease remained poorly understood and without therapeutic options until the latter half of the 20th century. Advances in the understanding of immunologic and hematologic pathways have shed light on HAE, a disease characterized by painful and unpredictable recurrent attacks of nonpitting edema without urticaria. Recognition that a deficiency of complement component 1 (C1) Esterase Inhibitor leads to overproduction of vasoactive kinins that cause angioedema paved the way for the development of early treatments. Increased understanding of the role of bradykinin in hereditary and acquired forms of C1 Esterase Inhibitor deficiency has led to the development of more targeted treatments for this painful, debilitating and potentially life-threatening disease.
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Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies
World Allergy Organization Journal, 2011Co-Authors: Richard G Gower, Emel Aygören-pürsün, Henriette Farkas, Paula J Busse, Amin J Barakat, Teresa Caballero, Mark Davis-lorton, David S Hurewitz, Joshua S Jacobs, Douglas T. JohnstonAbstract:Hereditary angioedema (HAE) caused by C1-Esterase Inhibitor deficiency is an autosomal-dominant disease resulting from a mutation in the C1-Inhibitor gene. HAE is characterized by recurrent attacks of intense, massive, localized subcutaneous edema involving the extremities, genitalia, face, or trunk, or submucosal edema of upper airway or bowels. These symptoms may be disabling, have a dramatic impact on quality of life, and can be life-threatening when affecting the upper airways. Because the manifestations and severity of HAE are highly variable and unpredictable, patients need individualized care to reduce the burden of HAE on daily life. Although effective therapy for the treatment of HAE attacks has been available in many countries for more than 30 years, until recently, there were no agents approved in the United States to treat HAE acutely. Therefore, prophylactic therapy is an integral part of HAE treatment in the United States and for selected patients worldwide. Routine long-term prophylaxis with either attenuated androgens or C1-Esterase Inhibitor has been shown to reduce the frequency and severity of HAE attacks. Therapy with attenuated androgens, a mainstay of treatment in the past, has been marked by concern about potential adverse effects. C1-Esterase Inhibitor works directly on the complement and contact plasma cascades to reduce bradykinin release, which is the primary pathologic mechanism in HAE. Different approaches to long-term prophylactic therapy can be used to successfully manage HAE when tailored to meet the needs of the individual patient.
Henriette Farkas - One of the best experts on this subject based on the ideXlab platform.
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hereditary angioedema c1 Esterase Inhibitor replacement therapy and coexisting autoimmune disorders findings from a claims database
Allergy Asthma & Clinical Immunology, 2020Co-Authors: Henriette Farkas, Donald Levy, Dylan Supina, Melvin Berger, Subhransu Prusty, Moshe FridmanAbstract:In this letter to the editor, we present results of claims data analysis. This claims data analysis supports a hypothesis that in patients with hereditary angioedema due to C1-Esterase Inhibitor (C1-INH) deficiency, the occurrence and/or symptomatology of coexisting autoimmune disease may be positively influenced by a replacement therapy with plasma derived C1-INH.
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Hereditary Angioedema Caused By C1-Esterase Inhibitor Deficiency: A Literature-Based Analysis and Clinical Commentary on Prophylaxis Treatment Strategies
World Allergy Organization Journal, 2011Co-Authors: Richard G Gower, Emel Aygören-pürsün, Henriette Farkas, Paula J Busse, Amin J Barakat, Teresa Caballero, Mark Davis-lorton, David S Hurewitz, Joshua S Jacobs, Douglas T. JohnstonAbstract:Hereditary angioedema (HAE) caused by C1-Esterase Inhibitor deficiency is an autosomal-dominant disease resulting from a mutation in the C1-Inhibitor gene. HAE is characterized by recurrent attacks of intense, massive, localized subcutaneous edema involving the extremities, genitalia, face, or trunk, or submucosal edema of upper airway or bowels. These symptoms may be disabling, have a dramatic impact on quality of life, and can be life-threatening when affecting the upper airways. Because the manifestations and severity of HAE are highly variable and unpredictable, patients need individualized care to reduce the burden of HAE on daily life. Although effective therapy for the treatment of HAE attacks has been available in many countries for more than 30 years, until recently, there were no agents approved in the United States to treat HAE acutely. Therefore, prophylactic therapy is an integral part of HAE treatment in the United States and for selected patients worldwide. Routine long-term prophylaxis with either attenuated androgens or C1-Esterase Inhibitor has been shown to reduce the frequency and severity of HAE attacks. Therapy with attenuated androgens, a mainstay of treatment in the past, has been marked by concern about potential adverse effects. C1-Esterase Inhibitor works directly on the complement and contact plasma cascades to reduce bradykinin release, which is the primary pathologic mechanism in HAE. Different approaches to long-term prophylactic therapy can be used to successfully manage HAE when tailored to meet the needs of the individual patient.
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Angiooedema due to acquired deficiency of C1-Esterase Inhibitor associated with leucocytoclastic vasculitis.
Acta dermato-venereologica, 2001Co-Authors: Henriette Farkas, Mariann Szongoth, Miklos Bely, Lilian Varga, Béla Fekete, István Karádi, George FüstAbstract:A hereditary and an acquired type of C1-Esterase Inhibitor deficiency have been described. Manifestations characteristic of both forms include recurrent subcutaneous and submucosal angiooedema. Acquired C1-Esterase Inhibitor deficiency has been observed in association with lymphoproliferative disorders, malignancy, autoimmune diseases and infections. We report on a case with the acquired form of the disease accompanied by leucocytoclastic vasculitis. Treatment with antimalarial agents resulted in complete resolution of symptoms and signs. Furthermore, C1-Esterase Inhibitor concentration and activity, as well as C1 levels, all returned to normal.