کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
3204261 | 1587147 | 2013 | 6 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
Hereditary Angioedema with Normal C1-INH (HAE Type III)
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کلمات کلیدی
OCPHRTACEC1-INHC1 inhibitor - C1 بازدارندهHereditary angioedema - آنژیوادم ارثیAngiotensin-converting enzyme - آنژیوتانسین تبدیل آنزیمEstrogen - استروژنDiagnosis - تشخیصHAE - جستجوTreatment - درمانOral contraceptive pill - قرص ضد بارداری خوراکیType III - نوع IIIhormone replacement therapy - هورمون جایگزین درمان
موضوعات مرتبط
علوم زیستی و بیوفناوری
ایمنی شناسی و میکروب شناسی
ایمونولوژی
پیش نمایش صفحه اول مقاله
![عکس صفحه اول مقاله: Hereditary Angioedema with Normal C1-INH (HAE Type III) Hereditary Angioedema with Normal C1-INH (HAE Type III)](/preview/png/3204261.png)
چکیده انگلیسی
Hereditary angioedema (HAE) with normal C1 inhibitor (C1-INH), also known as HAE type III, is a familial condition only clinically recognized within the past three decades. Similar to HAE from C1-INH deficiency (HAE types I and II), affected individuals experience unpredictable angioedema episodes of the skin, gastrointestinal tract, and airway. Unique clinical features of HAE with normal C1-INH include the predominance of affected women, frequent exacerbation by estrogen, and a prominence of angioedema that involves the face and oropharynx. The underlying pathophysiology of HAE with normal C1-INH is poorly understood, but indirect evidence points to contact pathway dysregulation with bradykinin-mediated angioedema. Currently, evaluation is complicated by a lack of confirmatory laboratory testing such that clinical criteria must often be used to make the diagnosis of HAE with normal C1-INH. Factor XII mutations have been identified in only a minority of persons affected by HAE with normal C1-INH, limiting the utility of such analysis. To date, no controlled clinical studies have examined the efficacy of therapeutic agents for HAE with normal C1-INH, although published evidence supports frequent clinical benefit with medications shown effective in HAE due to C1-INH deficiency.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: The Journal of Allergy and Clinical Immunology: In Practice - Volume 1, Issue 5, SeptemberâOctober 2013, Pages 427-432
Journal: The Journal of Allergy and Clinical Immunology: In Practice - Volume 1, Issue 5, SeptemberâOctober 2013, Pages 427-432
نویسندگان
Marc A. MD, MS,