Accepted
at 6:49 p.m. Dec, 08, 2024
by
Ahmed7
Author:
mbaroody
Type of change:
New Tags
Rationale for change
Additional context on hereditary angioedema, as well as C1 esterase inhibitor deficiency could help answer this question.
Here is a snippet from the answer explanation:
This patient's recurrent facial swelling is most likely due to hereditary angioedema, which is characterized by a deficiency or dysfunction of C1 inhibitor... Initial evaluation of hereditary angioedema is with complement testing. Low C4 is characteristic because, in the absence of C1 inhibitor, unregulated activation of C1 leads to excess activated C1 and, in turn, unchecked cleavage of C4.
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Text
{{c2::C1 esterase inhibitor}} deficiency results in {{c1::hereditary angioedema}}
Extra

Photo credit: James Heilman, MD, CC BY-SA 3.0, via Wikimedia Commons
Angioedema of the lips:

Image(s) licensed by DermNet and used with permission, CC-BY-NC-ND 3.0 NZ

- C1 inhibitor (AKA C1-esterase inhibitor) normally inhibits kallikrein, which is responsible for conversion of HMWK to bradykinin
- Deficiency leads to high levels of serum bradykinin → episodes of swelling and edema
- Can treat with icatibant (bradykinin B2 receptor antagonist), C1-inhibitor concentrate, or ecallantide (kallikrein inhibitor)

Photo credit: James Heilman, MD, CC BY-SA 3.0, via Wikimedia Commons
Angioedema of the lips:

Image(s) licensed by DermNet and used with permission, CC-BY-NC-ND 3.0 NZ

Lecture Notes
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Missed Questions
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Pathoma
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Boards and Beyond
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First Aid




Sketchy 2
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Sketchy Extra
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Picmonic
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Physeo
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OME
Additional Resources

characterized by rapid-onset edema of the face, genitals, limbs, larynx, and intestines without urticaria (helps distinguish hereditary angioedema from type 1 hypersensitivity reactions); treatment options include with fresh frozen plasma (contains C1 inhibitor), icatibant (bradykinin antagonist), and/or ecallantide (kallikrein inhibitor)

characterized by rapid-onset edema of the face, genitals, limbs, larynx, and intestines without urticaria (helps distinguish hereditary angioedema from type 1 hypersensitivity reactions); treatment options include with fresh frozen plasma (contains C1 inhibitor), icatibant (bradykinin antagonist), and/or ecallantide (kallikrein inhibitor)
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