C1 INH deficiency Flashcards
What causes hereditary angioedema?
It is caused by a deficiency in C1 esterase inhibitor, leading to unregulated bradykinin production.
Which medication class is a well-known trigger for angioedema?
ACE inhibitors, which can cause angioedema even years after initiating therapy.
What is the most likely mechanism behind a patient’s symptoms of non-puritic facial swelling, generalized edema, nausea, abdominal pain, cramping, and non-bloody diarrhea in the absence of infectious causes or fever, shortly after a dental procedure?
C1 esterase inhibitor deficiency.
Increased bradykinin production.
(hereditary angioedema).
How does hereditary angioedema present in patients?
Recurrent abdominal pain, swelling of extremities or face, and normal inflammatory markers
What key factor helps differentiate hereditary angioedema from anaphylaxis?
Absence of urticaria and pruritus
What is the diagnostic test to confirm hereditary angioedema (C1 esterase inhibitor deficiency)?
Measurement of C1 esterase inhibitor (C1-INH) levels and C4 levels
What is the treatment for hereditary angioedema (C1 esterase inhibitor deficiency)?
C1-INH concentrate
Icatibant (bradykinin receptor antagonist)
Ecallatide (Kallikrenin inhibitor)
Lanadelumab (antibody against kallikrenin)
How is the acute and chronic treatment characterized for the treatment of hereditary angioedema?
1) Manage Airway
2) Give FFP/Ecallantide/Icantibant for initial therapy acutely
3) Manage chonically with angrogens
How is hereditary angioedema with airway compromise treated?
With C1 esterase inhibitor concentrate or recombinant C1 inhibitor.
Are epinephrine and antihistamines effective for hereditary angioedema?
No, they are ineffective because hereditary angioedema is bradykinin-mediated, not histamine-mediated.
Name prophylactic strategies for hereditary angioedema.
Antifibrinolytics (tranexamic acid).
Androgens (danazol or Stanozolol).
Regular infusions of C1 esterase inhibitor through plasma or recombinant forms.
