Copyright © 2008 Massachusetts Medical Society. A 19-year-old woman presents to the emergency department with light-headedness, severe abdominal pain, and intractable nausea and vomiting that began 12 hours earlier. The patient reports previous episodes of abdominal pain and swelling of her hands and feet that have been attributed possibly to food allergies, which have recently become more freq...

Hereditary angioedema (HAE) is a rare autosomal dominant disorder caused by reduced activity of the C1 esterase inhibitor. Clinically, angioedema most often involves the upper extremities, face, neck and larynx. The most common cause of death is asphyxia related to laryngeal oedema. Attacks are triggered by many factors such as trauma, stress, infections and hormonal fluctuations. C1 esterase i...

Hereditary angioedema (HEA) is a disease characterized by decreased levels or function of C1 esterase inhibitor (C1-INH). The symptoms of HEA in pediatric age group generally consist of recurrent episodes of soft tissue swelling. These symptoms can be transient, subtle, and varied in severity. Genitourinary system is rarely affected in this disease. Here, a three-year-old girl who presented wit...

BACKGROUND Hereditary angioedema caused by mutations in the factor XII gene is a recently described disease entity that occurs mainly in women. It differs from hereditary angioedema caused by C1 inhibitor deficiency. OBJECTIVE To assess the clinical symptoms, factors triggering acute attacks, and treatments of this disease. METHODS Thirty-five female patients with hereditary angioedema and ...

DOI 10.2146/news100005 Dyax Corp. on December 1 announced that FDA approved the marketing of the company’s ecallantide injection, or Kalbitor, for the treatment of acute attacks of hereditary angioedema in patients age 16 years or older. Ecallantide, the company said, binds to plasma kallikrein and blocks its binding site, resulting in less production of bradykinin, a vasodilator. According to ...

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...

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.

Hereditary angioedema is characterised by recurrent episodes of peri-orbital and peri-oral swelling which can cause an upper airway obstruction, abdominal pain, vomiting, diarrhoea, and even hypotensive collapse. This potentially fatal condition is frequently misdiagnosed; its early recognition and appropriate treatment are thus important. We report a familial cluster of hereditary angioedema i...

The prevalence of non-hereditary angioedema was investigated in a general population sample (n = 7,931) and in a sample of Danish patients (n = 7,433) tested for deficiency of functional complement C(1) esterase inhibitor protein (functional C(1) INH). The general population sample (44% response rate) reported a lifetime prevalence of 7.4% for angioedema. In both groups symptoms were most frequ...

Hereditary angioedema is a rare autosomal dominant inherited disease which is characterized by an episodic, self-limiting increase in vascular permeability. Symptoms commonly involve in nonpitting, nonpruritic skin swellings. We present a case of hereditary angioedema. The patinets complained of a recurrent abdominal pain without accompanying skin swelling whose diagnosis was delayed nearly 20 ...