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Hereditary angioedema (HAE) VS allergic angioedema - Facial swelling, Difficulty Breathing

Medicosis Perfectionalis4:47

Transcription

Hey guys, it's Medicosis Perfectional. We're continuing our bleeding and coagulation disorder playlist. We have talked about hereditary angioedema before. Today, we'll compare between hereditary angioedema and allergic angioedema. Allergic angioedema is a type 1 hypersensitivity reaction. See the swelling with angioedema: swelling of the lips, the eyelids, structures in the face, and the neck. They can compress those airways, causing an emergency. Laryngeal edema is really bad for you. With that being said, let's get started. [Music]

Here is a very, very quick summary of hereditary angioedema. Autosomal dominant disease; deficiency or dysfunction of C1 inhibitor, leading to the release of, and inhibition of, plasma kallikreins. So, lots of kallikrein now leads to lots of bradykinin, because kallikrein converts high-molecular-weight kininogen into this nice—not nice, ugly—bradykinin, which will lead to contraction of nonvascular smooth muscle, such as your airway; increased vessel permeability, causing angioedema; nausea; pain, especially abdominal pain; vasodilation; and hypotension. Episodic attacks—they come in episodes of angioedema and pain. Sometimes there is a trigger, such as dental procedures. You go to the dentist, and, several hours, or like two hours later, you get those attacks of pain and angioedema. This is horrible. Sometimes there is a prodrome of sometimes tingling; could be nausea, etc. Then the symptoms—the symptoms are all of this crazy, bad stuff. But there is no urticaria; meaning no rash, no itching, no fitting. Because angioedema is non-pitting edema. Why? Because it's pus—it's an exudate and not a transudate.

How to diagnose hereditary angioedema? You need a family history. It doesn't have to be there, but it's possible. C1 level and function are normal. C2 and C4 decrease because they are consumed. C1 inhibitor is decreased because this is the definition of the whole freakin' thing. Bradykinin is high—this is what's causing the symptoms. Prekallikrein is low because all of it has been converted into plasma kallikrein. High-molecular-weight kininogen is low because all of it has been converted into bradykinin. Treatment: Do give C1 inhibitor by infusion. Do use Icatibant as well as lanadelumab. Okay, cool. Don't give steroids, antihistamine, or epinephrine. Why? Because this is a problem of bradykinin, not histamine. Cool. And don't give ACE inhibitors; they will make it worse. ACE inhibitors will inhibit the ACE, leading to lots of bradykinin, causing more of these symptoms. You'll be an idiot if you did that. The video is not over yet, but these cases can be over. Go to patreon.com/scishowsis and get them before they're gone.

Hereditary angioedema vs. allergic angioedema. Hereditary angioedema: Autosomal dominant, decreased C1 inhibitor. Now there is no inhibition of kallikrein. Now we have bradykinin. On the other hand, allergic angioedema: Type 1 hypersensitivity. We start with the allergen, B-cells, and we have IgE. The next exposure is going to be faster and stronger. Mast cell degranulation has histamine. So, hereditary angioedema: The problem is with increased bradykinin. Here we have increased histamine. Big difference. Can be life-threatening—why? Laryngeal edema. This can also be life-threatening: to drop hypotension. No urticaria, no itching. It has urticaria, meaning the rash and itching. Triggered by dental procedures, stress, emotions, menstruation, etc. This is triggered by exposure to allergen. Hereditary angioedema: The IgE level is normal. Here the IgE level could be high. Hereditary angioedema treatment: Do not give epinephrine, antihistamine, steroids, or ACE inhibitors. Here, please give epinephrine, antihistamines, and steroids. Hereditary angioedema: You can give C1 inhibitor, Icatibant, and lanadelumab.

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