Von Willebrand Disease | Pathophysiology, Types, Symptoms and Treatment
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- čas přidán 27. 07. 2024
- Lesson on Von Willebrand Disease (VWD): Pathophysiology, Types of VWD, Symptoms and Treatment. Von Willebrand disease is the most common inherited bleeding disorder, due to either deficiency or deficits of activity of von willebrand factor. VWD is estimated to affect 1% of the general population and affects women and men equally. Symptoms of VWD include a history of mucocutaneous bleeding. There are 3 main types of VWD, all resulting in varying severities of symptoms that require different types of treatments.If you found this video helpful, please like and subscribe for more videos like this one :)
JJ
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JJ
I am pretty sure VWF binds unto the GP1B, not the GPIIB/IIIa, as that is a Fibrinogen receptor.
Yes, you are right! It seems that this guy confuses an adhesion and aggregation.
Von Willebrand factor does act as bridge between platelets by binding two GP IIb/IIIa receptor on diferent platelets. Sources: czcams.com/video/ANXn3pUc9CA/video.html, and articles www.ncbi.nlm.nih.gov/pmc/articles/PMC424474/, ashpublications.org/blood/article/105/3/1078/19658/Von-Willebrand-factor-accelerates-platelet. BUT that is aggregation, most sources agree that the platelet plug starts with adhesion which involves the platelet binding to the vWF (which is tied to collagen) via the GP Ib receptor. After that it rolls on the collagen and binds to it with GP VI and GP Ia/IIa strengthtening the bond. After this follow the GP IIb/IIIa story.
Yes, I believe it can bind both domains although 1b seems to be more heavily studied.
"VWF mediates adhesion of platelet to subendothelium by binding to platelet glycoprotein 1b, Gp2b/3a when activated and to subendothelium"
Source -class slides
Also missing most important concept in labs, like increased BT, PTT. Normal platelets and ristocetin test decrease agglutination.
Very helpful video.Crisp and many concepts covered.Thank you so much
Excellent presentation.Thank you so much .
Excellent lecture. Thanks. DDAVP is costly. Cyclokapron is effective. It is often prescribed for menorrhagia, possibly managing undiagnosed VWD, with great success.
Great video. Just as a quick side note in case it helps someone, Desmopressin (DDAVP) is generally *avoided* in vWD type 2B due to risk of causing thrombocytopenia (due to gain of function defect - i.e. increased affinity for platelet binding and aggregation - this is also evident in an *increased* RIPA [Ristocetin-induced platelet aggregation] at low doses, as opposed to all other types which are all typically normal or decreased).
Thank you!
Very interesting and helpful video. I think i may have Von Willebron disease. Recently had 2 teeth extracted and ended up in the hospital due to excessive bleeding from the extraction sites. First they tried topical meds and then DDAVP. I was admitted for observation and ultimately send home later the next day. Will be following up with Hematologist.
I had a similar experience. I was sent home after dentist was able to stop bleeding. Two days later I was feeling extremely fatigued went to doctor was told that I was in shock. Sent to hospital by ambulance. Received a transfusion. I had a history of dysfunctional menstrual bleeding, and nosebleeds with heavy clotting and frequent gum bleeds. I was not diagnosed until a retired army doctor referred me for testing. He had once treated a man with Von Willebrands. My fortune was being misdiagnosed with a sarcoma in my gluteus muscle. I had to return to the hematologist that had sent me to get the blood work with the National Hemophilia Foundation. I had a very expensive battery of tests was told that I had Factor VII. I was treated with Amicar. I was told sometimes stress can trigger episodes. Good Luck.
Excellent sir
When I was 12 they did a clotting test before I got my tonsils out. It took 25 minutes for me to stop bleeding. Tonsils weren't taken out and I got admitted to Vanderbilt's Children Hospital. I spent a month there due to the uncontrolled bleeding.
Thank you 💕
Great ❤!
thank u so much
Vwf binds to GP1b on platelets
They are testing me for it because ive had recurrent miscarriages, hemorraghed during childbirth and bled badly with dental and abdominal surgery. My menstrual cycles are awful. I do carry the Hemophilia B gene but my OB thinks VWD is the mkre likely culprit tho you can show symptoms as a hemophilia carrier.
You can have both hemophilia and VWD
I think vWF binds to GP1b receptor of platelets
tıp 2 b
Hi my family history all members have Von Willebrand disease factor 8. We can take Covid-19 vaccine or not.lot of confusion please help me
Can we give minirin tab
It's GP1B for vWF
When you have VWD, can you undergo major surgery?
Hi sir what is the treatment of VWD my kid's have VWD kindly help me to show me the right way 🙏
What symptoms does he have?
Can vwd people put covid vaccine?
I took it then profusely bled .. for 8 months .
Wrong - VWF binds to gpIb.
its gp1b complex for vwf !!!!!