Hereditary angioedema type ΙΙ; report of a case
نویسندگان
چکیده
منابع مشابه
A Case of Type 2 Hereditary Angioedema With SERPING1 Mutation
Hereditary angioedema is a disease of congenital deficiency or functional defect in the C1 esterase inhibitor (C1-INH) consequent to mutation in the SERPING1 gene, which encodes C1-INH. This disease manifests as recurrent, non-pitting, non-pruritic subcutaneous, or submucosal edema as well as an erythematous rash in some cases. These symptoms result from the uncontrolled localized production of...
متن کاملHereditary angioedema: a case report and literature review.
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...
متن کاملReport on the First Survey of Iranian Patients with Hereditary Angioedema
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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Background In classic hereditary angioedema, inadequate C1-inhibitor (C1-INH) failing to restrict factor-XII activity leads to increased production of bradykinin, a potent vasodilator and mediator of angioedema. Hereditary angioedema with normal C1-INH (Type-III) manifests with sporadic recurrent angioedema but normal C1-INH concentration and activity. Here, bradykinin accumulation appears depe...
متن کاملHereditary angioedema: report of a family in Malaysia.
The most likely cause for PDPH is leakage of cerebrospinal fluid from a hole in the dura, the remarkable feature being that such a trivial injury can cause such severe symptoms. Conservative management with bed rest, analgesics and fluids is helpful in those with mild symptoms. Relief however, is not dramatic and sealing that hole in the dura is the only way to achieve rapid relief Gromley (196...
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ژورنال
عنوان ژورنال: Cumhuriyet Medical Journal
سال: 2012
ISSN: 1305-0028
DOI: 10.7197/1305-0028.1427