Angioedema

What is the underlying genetic defect in Type 1 HAE?

• Mutation in the CI esterase gene

• Deficiency in Factor XII

• Hyperproduction of bradykinin

• Mutation in the C4 gene

Which complement protein is typically low in patients with Type 1 HAE?

• C3

• C4

• C1-INH

• C5

In a patient with suspected HAE, which of the following lab findings would support the diagnosis of Type II HAE?

• Low C4, low C1-INH activity, normal C1-INH levels

• Normal C4, normal C1-INH activity

• High C4, high C1-INH levels

• Low C4, normal C1-INH activity

What is the first-line treatment for an acute attack of Hereditary Angioedema affecting the airway?

• Antihistamines

• Oral corticosteroids

• C1-INH replacement therapy

• Epinephrine injection

Which of the following is a common precipitating factor for an attack of Hereditary Angioedema?

• Cold weather exposure

• Physical trauma or surgery

• Medications like antihistamines

• Infections with high fever

Which of the following medications is contraindicated in patients with HAE due to its potential to trigger an attack?

• Beta-blockers

• ACE inhibitors

•  Statins

• Antihistamines

In Type II Hereditary Angioedema (HAE), which of the following best describes the pathophysiological mechanism?

• Low levels of C1 esterase inhibitor (C1-INH)

• Defective C1-INH function, but normal C1-INH levels

• High levels of C4

• Overproduction of bradykinin due to factor XII mutation

Which of the following best explains why C1 esterase inhibitor (C1-INH) is crucial in Hereditary Angioedema?

• C1-INH regulates kallikrein-kinin system preventing excess bradykinin prod.

• C1-INH acts to degrade histamine, preventing allergic reactions

• C1-INH is responsible for controlling complement C5a production

• C1-INH prevents the activation of mast cells during immune responses

Which of the following mediators is directly responsible for the vascular permeability changes seen in HAE?

•  Histamine

• Leukotrienes

•  Bradykinin

• Prostaglandins

What is the role of Factor XII in the pathogenesis of Hereditary Angioedema?

• Factor XII initiates kallikrein-kinin system when C1-INH is deficient/ dys.

• Factor XII activates the complement system in response to immune triggers

• Factor XII inhibits the production of bradykinin

• Factor XII increases C1-INH levels in the blood