How Common Is Hereditary Angioedema?

Discussion Angioedema is edema that is non-pitting, self-limited occurring in non-dependent areas usually in an asymmetric distribution usually on the lips, face, hands, feet, genitals and also in the bowel. It usually develops over minutes to hours (often 1-2 hours) with resolution usually within 24-48 hours. Angioedema often occurs with urticaria but 20% of patients may have isolated angioedema. Acute allergic angioedema is often caused by drugs (including antibiotics and non-steroidal anti-inflammatory drugs), foods, infections, insects, various organic substances (i.e. latex, preservatives, formaldehyde, etc.), and other allergens such as animal danders, dust mites, pollens and molds. Common triggers in children are infections including the viruses Coxsackie A, Epstein-Barr virus, Hepatitis B, and Herpes simplex. Bacterial causes include otitis media, pharyngitis, sinusitis and urinary tract infections. Parasitic infections causing angioedema include filariasis, strongyloides and toxocara. Physical factors can cause mast cell release causing angioedema. These include exposure to cold, heat, pressure, the sun and vibration. Hereditary angioedema (HAE) is a rare genetic disorder. HAE is caused by decreased amount of C1 inhibitor (Type 1, 85% of cases), normal amount of C1 inhibitor but decreased functionality (Type II, 15%) and normal C1 inhibitor and functionality but with angioedema. Type I and Type II HAE are autosomal dominant diseases but up to 25% may have a de novo ...
Source: PediatricEducation.org - Category: Pediatrics Authors: Tags: Uncategorized Source Type: news

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