Malignancy and Immune Disorders in Patients with Hereditary Angioedema
نویسندگان
چکیده
Hereditary angioedema (HAE) is well documented condition with inherited deficiency or dysfunction of C1 inhibitor. However, its association other immune disorders such as autoimmunity, malignancy and primary immunodeficiency not known.
منابع مشابه
Perioperative management for patients with hereditary angioedema
Hereditary angioedema (HAE) is a rare autosomal dominant disease that results from mutations in the C1-esterase inhibitor (C1-INH) gene. HAE is characterized by recurrent episodes of angioedema of the skin (face, extremities, genitalia, trunk), the gastrointestinal tract, and respiratory tract. Symptoms experienced can be debilitating, may impact quality of life, and can be life threatening. Pr...
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چکیده ندارد.
Hereditary and acquired angioedema: experience with patients in Puerto Rico.
Hereditary (HAE) and acquired (AAE) angioedema are vascular reactions involving the sub mucosal tissues, representing localized edema caused by dilatation and increased permeability of the capillaries. HAE and AAE are clinical disorders characterized by angioedema that require prompt differentiation from other causes of angioedema in order to receive the most pertinent and effective therapeutic...
متن کاملGenetic test indications and interpretations in patients with hereditary angioedema.
Patients with hereditary angioedema (HAE) present with recurrent, circumscribed, and self-limiting episodes of tissue or mucous membrane swelling caused by C1-inhibitor (CI-INH) deficiency. The estimated frequency of HAE is 1:50,000 persons. Distinguishing HAE from acquired angioedema (AAE) facilitates therapeutic interventions and family planning or testing. Patients with HAE benefit from trea...
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Background: Hereditary angioedema (HAE) is a rare autosomal dominant primary immunodeficiency with complement system defect characterized by recurrent episodes of angioedema involving the skin or mucosa of the upper respiratory and gastrointestinal tracts. Objective: To characterize the clinical and laboratory data of hereditary angioedema in Iran. Methods: Patients with probable diagnosis of a...
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ژورنال
عنوان ژورنال: The Journal of Allergy and Clinical Immunology
سال: 2021
ISSN: ['1097-6825', '0091-6749', '1085-8725']
DOI: https://doi.org/10.1016/j.jaci.2020.12.121