Hereditary Angioedema

Question 1

Hereditary angioedema (HAE) is an

Answer 1

Autosomal dominant

Question 2

Physiology of heriditary angio edema

Answer 2

uncontrolled release of bradykinin resulting in oedema of tissues

Question 3

Clinical features of hereditary angio edema

Answer 3

Multiple hospital admissions in the last year with lip and tongue swelling and shortness of breath

hydrocortisone, chlorphenamine, and adrenaline may show little to no improvement

no evidence of lip or tongue swelling

Question 4

Diagnosis of hereditary angio edema

Answer 4

Serum C4 is the most reliable and widely used screening tool

may be proceeded by painful macular rash

painless, non-pruritic swelling of subcutaneous/submucosal tissues
may affect upper airways, skin or abdominal organs (can occasionally present as abdominal pain due to visceral oedema)

Question 5

Hereditary angio edema does not respond to ?

Answer 5

adrenaline, antihistamines, or glucocorticoids

Question 6

Mx of angio edema

Answer 6

IV C1-inhibitor concentrate,
fresh frozen plasma (FFP) if this is not available

Question 7

Prophylaxis of angio edema

Answer 7

anabolic steroid Danazol may help