Study Findings Result in New Diagnostic Recommendations for Hereditary Angioedema in the ER

According to a story from Angioedema News, a team of researchers has used data from a recent study to provide up to date recommendations for diagnosing hereditary angioedema in the emergency room setting. Dozens of patients may be admitted to the ER with severe swelling, but there are certain signs and characteristics that doctors must know to make a quick and accurate diagnosis; it’s not like hereditary angioedema is the only cause of severe swelling around.

About Hereditary Angioedema (HAE)

Hereditary angioedema is a genetic disorder which is characterized by chronic episodes of swelling that can affect multiple areas of the body. The condition is caused by mutations affecting the HAE gene. Swelling attacks generally occur every two weeks or so; they can usually last for several days. Swelling may affect the limbs, digestive tract, face, and airway, with blockage of the airway being the most dangerous complication. Vomiting and abdominal pain may accompany attack as well if the digestive tract is involved. Treatment involves reducing the likelihood for attacks to appear and preventing them from worsening when they do. Hereditary angioedema is typically only life-threatening if left untreated. Prevalence of the condition is estimated to be around one in 10,000 to one in 50,000, at least in the US and Canada. To learn more about hereditary angioedema, click here.

Like many rare diseases, patients with this condition may experience a delayed diagnosis, primarily because the symptoms of the disorder so closely resemble those of a severe allergic reaction. The result can be potentially fatal delays in treatment or treatments that are inappropriate.

About The Guidelines

The team of scientists has developed the Hereditary Angioedema Rapid Triage (HAE-RT) Tool, which details four steps for rapidly diagnosing the disorder in the emergency setting.

  1. The first and absolutely most critical step is ensuring that the airway is free of obstruction. This is how a swelling attack can easily be fatal and must be addressed hastily.
  2. The second step is administering allergy medications. If the patient does not respond to these medications and also has a history of swelling events, hereditary angioedema could be the culprit.
  3. If the condition is suspected, rapid treatment with appropriate medications should occur immediately. Examples include the C1 inhibitors Cinryze and Berinert or the bradykinin inhibitor Firazyr.
  4. The final step should be a referral of the patient to a specialist (most likely an allergist) that can definitively confirm the diagnosis.


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