Transcription
Hey guys, another day, another lecture about bleeding and coagulation disorders. In the previous video, we have talked about hereditary angioedema. Today, let's talk about crazy mnemonics about it, and let's get started. [Music]
So here is a quick summary of hereditary angioedema: autosomal dominant disease leading to deficiency of C1 inhibitor, which increases bradykinin. When you have increased bradykinin, you have contraction of nonvascular smooth muscles such as your bronchioles, increased vessel permeability, natriuresis, and vasodilation, together leading into hypotension and increased pain due to inflammation. We have episodic attacks of angioedema and pain; the attacks last between two to three days and they resolve in one to two days. We start with a trigger, then a prodrome leading to symptoms. The trigger could be anything, specifically dental procedures. Prodrome including tingling, nausea, etc., and then the symptoms. All of these symptoms quoting angioedema, abdominal pain, but there is no urticaria, no itching, and no pitting, which distinguishes hereditary angioedema from allergic angioedema or allergic urticaria.
Diagnosis: You need the family history; you need this trigger, prodrome, symptoms; and you need the labs. C1 is normalcy too, and for our decreased C1 inhibitor is decreased. That's the whole definition. Bradykinin is high, prekallikrein is low, high-molecular-weight kininogen is low, C1 esterase inhibitor is low; it has been converted into kallikrein. High-molecular-weight kininogen is low because it has been converted into bradykinin.
Treatment: Do's and don'ts. Do give C1 inhibitor; do give Icatibant; and don't give steroids, antihistamine, and epinephrine. And heaven forbid, never ever ever give ACE inhibitors.
Now to the mnemonic. The etiology: the three subtypes. Type one is a decreased amount of the enzyme. So in type one, C1INH is none. Type two: the C1INH is too weak; normal amount, decreased function. Type three: we have a problem with factor XII. 12 has three digits in Roman numerals. Thank God for the Roman Empire because it doesn't work in English. Another mnemonic: 3 sounds like tree, and tree; remember the story of Adam and Eve. Eve; type 3 is estrogen-dependent, officially crazy.
Another mnemonic: Hereditary angioedema. H: high-molecular-weight kininogen is low; H: hypotension; I: episodic attacks lasting one to three days; the autosomal dominant; the deficiency of C1 esterase inhibitor; the dental works or dental procedures trigger the problem. Cool. A theory: it sounds like inhibitor because the C1 inhibitor is deficient. Angioedema; ACE inhibitors are contraindicated. Oh ouch; abdominal pain; the dry cough; airway obstruction could be an emergency; and if you don't have the available medications, you should endotracheal intubate.
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The B mnemonic of bradykinin: Bradykinin: prekallikrein, B or P: pain, perpetual, which means chronic, pro-inflammatory, big time, increased vessel permeability, to pus, and angioedema, which is perilous, meaning dangerous. Upper airway obstruction due to angioedema can lead to hypoxia. Bradykinin bronchoconstriction leading to dry cough; big vessel vasodilation; big-time vessel vasodilation, because when the vessel dilates it becomes big. Bradykinin hyponatremia and hypotension. Some more ecology mnemonics.
To drugs that can treat the acute attacks of angioedema: Icatibant and Ecallantide. Icatibant and Icatibant, and I'm a bradykinin receptor blocker; bradykinin receptor blocker. And by the way, it blocks B2 receptor, which is a bradykinin receptor. And again, ecallantide: it eliminates kallikrein; it's a kallikrein inhibitor.
We're done with hereditary angioedema. Next time we'll talk about acquired angioedema; the patient is older, if you notice.
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