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Review of hereditary angioedema
LymphoSign Journal, 2016Hereditary angioedema (HAE) is a rare disease caused by deficiency of C1 esterase inhibitor (C1-INH). It is an autosomal dominant disease caused by a variety of mutations in the C1-INH gene. C1-INH is an important regulator of several pathways. One pathway it affects is the kallikrein–kinin pathway, which results in the generation of bradykinin ...
Lisa W. Fu +3 more
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Management of hereditary angioedema
Nursing Standard, 2013Hereditary angioedema is characterised by unpredictable, painful and potentially life-threatening oedema. Recently, some C1 inhibitors have been approved for self-administration and/or routine prevention, enabling patients to be proactive in managing their disease and reducing the burden of illness.
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The management of hereditary angioedema
La Ricerca in Clinica e in Laboratorio, 1983Our experience in managing 120 cases of hereditary angioedema is reported. Forty-two severe episodes of mucous or subcutaneous edema were successfully managed using CI-INH concentrate. A prophylactic treatment was done with two classes of drugs: antifibrinolytic agents (tranexamic acid) and 17α-alkylated androgen derivatives.
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The pathogenesis of hereditary angioedema
Transfusion and Apheresis Science, 2003Hereditary angioedema (HAE), which is characterized by episodic localized angioedema of the skin or mucosa, results from heterozygous deficiency of the plasma protease inhibitor, C1 inhibitor (C1INH). The most obvious biologic role of C1INH, therefore, is prevention of excessive vascular permeability.
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Journal of Allergy and Clinical Immunology, 2008
Hereditary angioedema is an episodic swelling disorder with autosomal dominant inheritance. Attacks are characterized by brawny, self-limited, nonpruritic edema of the deep dermal layers of the skin that most often involve the hands and feet. They usually begin in childhood and become more severe after puberty.
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Hereditary angioedema is an episodic swelling disorder with autosomal dominant inheritance. Attacks are characterized by brawny, self-limited, nonpruritic edema of the deep dermal layers of the skin that most often involve the hands and feet. They usually begin in childhood and become more severe after puberty.
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New England Journal of Medicine, 2020
Paula J, Busse, Sandra C, Christiansen
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Paula J, Busse, Sandra C, Christiansen
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Treatment of Hereditary Angioedema
JAMA: The Journal of the American Medical Association, 1979To the Editor.— In his letter "Hereditary Angioedema" (240:2155, 1978), Dr Reimann does not mention the use of androgenic steroids for prophylaxis of attacks. Methyltestosterone was first effectively used by Spaulding 1 in 1960. This was followed by reports of effectiveness of less virilizing analogues, fluoxymesterone, oxymetholone, 2 and danazol.
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Contraception in hereditary angioedema
Fertility and Sterility, 2008To report a case of successful use of a transdermal contraceptive in a patient with hereditary angioedema.Case report.University medical center.A patient who had used oral contraceptives contracted SC and submucosal tumefaction every 1 or 2 months. She was diagnosed with type I hereditary angioedema.
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The pathophysiology of hereditary angioedema
Clinical Immunology, 2005Hereditary angioedema (HAE), characterized by recurrent episodes of angioedema involving the skin, or the mucosa of the upper respiratory or the gastrointestinal tracts, results from heterozygosity for deficiency of the serine proteinase inhibitor (serpin), C1 inhibitor (C1INH).
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