What is the inheritance pattern of Hereditary angioedema?

autosomal dominant

What are the main clinical features of Hereditary angioedema?

• attacks 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)
• urticaria is not usually a feature

What is the management of Acute Hereditary angioedema?

• does not respond to adrenaline, antihistamines, or glucocorticoids
• IV C1-inhibitor concentrate, fresh frozen plasma (FFP) if this is not available

What is the pathophysiology of Hereditary angioedema?

associated with low plasma levels of the C1 inhibitor protein. C1-INH is a multifunctional serine protease inhibitor - the probable mechanism behind attacks is uncontrolled release of bradykinin resulting in oedema of tissues

What is the management of prophylaxis Hereditary angioedema?

anabolic steroid Danazol may help

What is seen in bloods during an acute attack of Hereditary angioedema?

low C1-INH level

What abnormalities are seen in bloods in Hereditary angioedema?

low C2 and C4 levels are seen, even between attacks. Serum C4 is the most reliable and widely used screening tool