نتایج جستجو برای: hereditary angioedema

تعداد نتایج: 89742  

بهاری, امیر, رضایی, محمد صادق, غفاری, جواد,

Hereditary angioedema is a rare disorder of complement system which is often seen with autosomal dominant hereditary. Clinical characteristics include non- pruritic and non-pitting mucocutaneous edema that could involve all parts of the body. This study reports seven cases of hereditary angioedema with classical manifestations accompanied by low function of C1INH (type 2). One death occurred du...

Journal: :Journal of investigational allergology & clinical immunology 2016
M Cicardi C Suffritti F Perego S Caccia

Angioedema is defined as local, noninflammatory, self-limiting edema that is circumscribed owing to increased leakage of plasma from the capillaries located in the deep layers of the skin and the mucosae. Two mediators, histamine and bradykinin, account for most cases of angioedema. Angioedema can occur with wheals as a manifestation of urticaria, and this form is frequently allergic. In the pr...

Journal: :Anais brasileiros de dermatologia 2012
Sergio Duarte Dortas Junior Solange Oliveira Rodrigues Valle Soloni Afra Pires Levy Rosangela P Tortora Augusto Tiaqui Abe Gisele Viana Pires José Angelo de Souza Papi Alfeu Tavares França

Hereditary Angioedema is a dominantly inherited disease. Routine screening of autoantibodies (AAB) is not recommended for individuals with Hereditary Angioedema; however, prevalence of these antibodies in Hereditary Angioedema patients is not well documented. We aim to determine the prevalence of AAB so that individuals at risk of developing autoimmune diseases can be identified. Fifteen patien...

Journal: :iranian journal of immunology 0
shervin shahinpour research center for immunod eficiencies, pediatrics ce nter of excel lence, children's medical c enter, tehran university of medical sciences, tehran marzieh tavakol department of allergy and clinical immunology, shahid bahonar hospital, alborz u niversity of medical sciences, karaj, iran hassan abolhass ani research center for immunod eficiencies, pediatrics ce nter of excel lence, children's medical c enter, tehran university of medical sciences, tehran payam mohammadinejad research center for immunod eficiencies, pediatrics ce nter of excel lence, children's medical c enter, tehran university of medical sciences, tehran masoud movahedi research center for immunod eficiencies, pediatrics ce nter of excel lence, children's medical c enter, tehran university of medical sciences, tehran saba arshi allergy and clinical immunology department of rasole-akram hospital, iran university of medical sciences, tehran, iran asghar aghamohammadi

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...

2017
Gulsen Akoglu Belgin Kesim Gokhan Yildiz Ahmet Metin

BACKGROUND Hereditary angioedema is a rare autosomal dominantly inherited immunodeficiency disorder characterized by potentially life-threatening angioedema attacks. OBJECTIVE We aimed to investigate the clinical and genetic features of a family with angioedema attacks. METHODS The medical history, clinical features and C1-INH gene mutation of a Turkish family were investigated and outcomes...

2015
Michelle Fog Andersen Anette Bygum

Hereditary angioedema is a rare, but potentially life-threatening genetic disorder that results from an autosomal dominant trait. It is characterized by acute, recurrent attacks of severe local edema, most commonly affecting the skin and mucosa. Swelling in hereditary angioedema patients does however not always have to be caused by angioedema but can relate to other concomitant disorders. In th...

Journal: :Acta dermatovenerologica Croatica : ADC 2010
Lea Salamon Jadranka Morović-Vergles

Hereditary angioedema is a rare genetic disorder resulting from an inherited deficiency or dysfunction of the C1 inhibitor. It is characterized by recurrent, circumscribed, and self-limiting episodes of cutaneous and mucous membrane swelling involving different organs. Hereditary angioedema may present with diverse clinical pictures, even within families with the same mutation. We present a fir...

Journal: :Cardiovascular Therapy and Prevention 2023

Hereditary angioedema is a rare (1:50,000) hereditary chronic disease from the group of primary immunodeficiencies, which manifests in form recurrent skin and mucous membrane edema respiratory tract gastrointestinal tract. Pathogenesis this are associated with an increased risk number cardiovascular pathologies, complexity management choosing optimal treatment methods. This case report demonstr...

Journal: :General dentistry 2002
Nathaniel D Tricker Kay M Malone Michael M Ellis

Hereditary angioedema is an autosomal dominant condition with a typical presentation of diffuse edematous, painless, and nonpitting swelling of the soft tissues. The disease manifests itself primarily in the extremities, face, airway, and abdominal viscera. Proper diagnosis and treatment are essential as this condition has the capacity of becoming life-threatening due to potential airway embarr...

Journal: :iranian journal of allergy, asthma and immunology 0
eray atalay department of internal medicine, kafkas university practice and research hospital, kars, turkey mehmet tamer özdemir department of internal medicine, kafkas university practice and research hospital, kars, turkey gülşen çiğsar kafkas university practice and reserch hospital emergency department kars, turkey ferhat omurca department of internal medicine, kafkas university practice and research hospital, kars, turkey nurullah aslan department of internal medicine, kafkas university practice and research hospital, kars, turkey mehmet yildiz department of internal medicine, kafkas university practice and research hospital, kars, turkey

angioedema is an asymmetric non-pitting oedema on face, lips, tongue and mucous membranes; any delay in diagnosis and treatment can be fatal. treatment with lisinopril as an angiotensin converting enzyme (ace) inhibitor, can be a reason of angioedema. here we report a case who developed oral-facial edema four years after using lisinopril/hydrochlorothiazide. laryngeal oedema is a main cause of ...

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