Von Willebrand Disease (VWD) Subtypes: Type 1, 2A, 2B, 2M, 2N, and 3

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Medicosis Perfectionalis

Medicosis Perfectionalis

Күн бұрын

Von Willebrand Disease (VWD) Subtypes: Type 1, 2A, 2B, 2M, 2N, and Type 3 VWD.
Von-Willebrand factor (vWF) comes from the meagakaryocytes (bone marrow) as well as the Weibel-Palade bodies (endothelial cells). Von Willebrand factor (vWF) helps the platelets adhere to the sub endothelial collagen via GP1b receptor on the platelet.
Von Willebrand disease (vWD) is the most common inherited bleeding disorder worldwide.
Primary hemostasis involves forming a platelet plug (thrombocytes) whereas secondary hemostasis involves making a fibrin thrombus (coagulation factors).
Ionized calcium helps blood coagulate.
We test for primary hemostasis via platelet count, bleeding time, and platelet aggregometry.
We test secondary Hemostasis via prothrombin time (PT), activated partial thromboplastin time (aPTT), and the old coagulation time (or clotting time, or thrombin time).
Von Willebrand Dusease (vWD) affects primary as well as secondary Hemostasis.
Hemolytic Uremic Syndrome (HUS), atypical Hemolytic Uremic Syndrome (aHUS), and Thrombotic Thrombocytopenic Syndrome (TTP).
Atypical hemolytic uremic syndrome (aHUS) is similar to thrombotic thrombocytopenic Purpura (TTP).
Typical Hemolytic Uremic Syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure.
Thrombotic thrombocytopenic purpura (TTP) is a pentad of microangiopathic hemolytic anemia, thrombocytopenia, acute renal failure, fever, and neurological abnormalities.
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Bernard-Soulier Syndrome (BSS) is an autosomal recessive (AR) disorder characterized by a defective GP1b platelet receptor leading to a problematic platelet adhesion process (primary hemostasis).
Glanzmann Thrombasthenia (GT) is an autosomal recessive defect in the GPIIb/IIIa (GP2b/3a) receptor on the platelet which leads to defective platelet aggregation.
Ristocetin Cofactor Assay (RIPA) is usually abnormal in cases of Bernard-Soulier Syndrome (BSS).
Bleeding time is prolonged, platelets are big in size, but few in number (Macrothrombocytopenia).
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Platelet count is one of the lab tests used by a doctor or a hematologist to assess whether your platelet number is normal, low (thrombocytopenia) or high (thrombocytosis).
Bleeding 🩸 time (BT) is another test for platelet function.
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Пікірлер: 243
@dvf4550
@dvf4550 3 жыл бұрын
This guy teaches a technical, dry topic with wisdom and hilarity! :-D
@MedicosisPerfectionalis
@MedicosisPerfectionalis 3 жыл бұрын
Thank you so much ☺️
@jimmypk1353
@jimmypk1353 16 күн бұрын
Yeah! Like, "We gotta keep the blood stream PG-13." How can you forget an analogy like that? 😁
@kollywogs3342
@kollywogs3342 7 ай бұрын
Great video. Thanks! I have VWD Type 2M with severe symptoms. The standard of care for this type is Humate-P as this type is only variably responsive to DDAVP. I even keep Humate-P at home and take it with me when I travel because hospitals don't always have it on hand. Surgeries are particularly difficult because DDAVP requires a strict fluid intake which is often impossible to adhere to when you have to get IV fluids and/or blood transfusions during and after the procedure. Everyone who has VWD should work closely with a benign hematologist who specializes in bleeding disorders because surgeons and anesthesiologists often don't understand the nuances and you need to have a plan going into the surgery. Oncologist/hematologists are often focused on cancer and they don't have a lot of experience with the VWD subtypes. Also, fun fact... type 2M gets misdiagnosed more often as Type 1 than it gets correctly diagnosed as Type 2M. This type doesn't necessarily have missing multimers, just abnormal ones. There is a large variabilty in bleeding patterns and laboratory phenotypes. Genetic testing can help.
@Yippieyiyo
@Yippieyiyo Жыл бұрын
I'm not a med student but I have type 2b and I love learning about my condition
@MedicosisPerfectionalis
@MedicosisPerfectionalis Жыл бұрын
Thank you so much for watching! I wish you all the best!
@shahabuddin6149
@shahabuddin6149 3 жыл бұрын
You are the best teacher in KZbin as well as in globe,we are only depending on you're lovely and precise notes,not college professors,keep it up be happy and healthy forever
@MedicosisPerfectionalis
@MedicosisPerfectionalis 3 жыл бұрын
Thank you so much, brother! I appreciate you!
@shahabuddin6149
@shahabuddin6149 3 жыл бұрын
@@MedicosisPerfectionalis most welcome Sir,,I note you're 2 lectures daily base thanks,achieve first position from day first up to 4th years MBBS
@theafricanesther5795
@theafricanesther5795 Жыл бұрын
I'm still a med student but I thoroughly enjoyed this video. You made it so easy, thank you
@MedicosisPerfectionalis
@MedicosisPerfectionalis Жыл бұрын
Glad it was helpful!
@fatimarezai5644
@fatimarezai5644 3 жыл бұрын
Sir thumbs up for you The best ever explaination All videos of you are a great gift for all the poor knowledged medical students for life Big fan of you❤❤❤❤👍👍👍👌👌👏👏👏 The one n only authentic source for me out of many others
@MedicosisPerfectionalis
@MedicosisPerfectionalis 3 жыл бұрын
Thank you so much 😊
@emanabdelghaffar1923
@emanabdelghaffar1923 3 жыл бұрын
Words can’t expess how much I’m grateful for you , you have made medical school much easier (: Much of love from your home country
@cherrrriii
@cherrrriii 3 ай бұрын
i can never stop admiring your work, thank you so much
@MedicosisPerfectionalis
@MedicosisPerfectionalis 3 ай бұрын
I am honored!
@Pathology92
@Pathology92 4 жыл бұрын
Thank you!❤❤,,,,,best explanation of subtypes,,,,,most abundant platelet surface receptor is Gp2b/3a complex(40,000-90,000 copies per cell with an additional pool that can be recruited by agonist-induced platelet activation) and qualitative or quantitative deficiency of it leads to a coagulopathy named glanzmann's thrombasthenia,,,,,,,
@MedicosisPerfectionalis
@MedicosisPerfectionalis 4 жыл бұрын
Thank you so much for your kind words! You’re absolutely correct
@flaviusmiron9171
@flaviusmiron9171 3 жыл бұрын
Can't believe that you used "The Good, the Bad and the Ugly" movie reference. Love your videos, they are great :D
@MedicosisPerfectionalis
@MedicosisPerfectionalis 3 жыл бұрын
Thank you so much ☺️
@DasEsa
@DasEsa Жыл бұрын
Ur style for lecturing are Amazing. Allah blessing u. From Kurdistan/iraq
@MedicosisPerfectionalis
@MedicosisPerfectionalis Жыл бұрын
Thank you so much! I appreciate you! Would you please help me by sharing?
@sissilguitare7919
@sissilguitare7919 4 жыл бұрын
Thank you so Much man best explanation ever love this playlist
@MedicosisPerfectionalis
@MedicosisPerfectionalis 4 жыл бұрын
My pleasure 😇
@jacksparrow-wq1pb
@jacksparrow-wq1pb 5 ай бұрын
Never skip the ads ❤ that's how I show my gratitude ❤
@MedicosisPerfectionalis
@MedicosisPerfectionalis 5 ай бұрын
I appreciate you so much!
@MohamedMohamed-uq7bn
@MohamedMohamed-uq7bn 2 жыл бұрын
Really exellent vedio .... bleeding disorders were always a nightmare for me... not any more
@MedicosisPerfectionalis
@MedicosisPerfectionalis 2 жыл бұрын
I am so delighted!
@manarmahmoud9872
@manarmahmoud9872 2 жыл бұрын
12:26 subtypes ❤️ Thanks for your efforts ❤️❤️
@MedicosisPerfectionalis
@MedicosisPerfectionalis 2 жыл бұрын
Thank you!
@nesmaessam9115
@nesmaessam9115 3 жыл бұрын
I love you, 😭😭❤️❤️❤️ I stady to my exam from here.. and I'm sure I'll passsssss 😭😭😭❤️❤️❤️❤️❤️❤️❤️❤️❤️❤️❤️
@MedicosisPerfectionalis
@MedicosisPerfectionalis 3 жыл бұрын
Thank you so much 😊 Good luck to you!
@leptoquark6278
@leptoquark6278 4 жыл бұрын
The best ever......and u don’t need to retire...lol...
@MedicosisPerfectionalis
@MedicosisPerfectionalis 4 жыл бұрын
Haha 😂 Thank you so much 😊
@rathodnehal2869
@rathodnehal2869 4 жыл бұрын
i am nursing student but after i watch your so many video iam happy and i have confidence tq so much 😘😊👍 and clap 🖐️🖐️🖐️tq again
@dominikabajguz1221
@dominikabajguz1221 Жыл бұрын
Nice video! Just one correction: Type 2N is also autosomal recessive. Carriers do not have symptoms
@Truth_Seeker1
@Truth_Seeker1 3 жыл бұрын
I have Von Willebrand’s disease type 3.
@MedicosisPerfectionalis
@MedicosisPerfectionalis 3 жыл бұрын
I am so sorry 😐
@Truth_Seeker1
@Truth_Seeker1 3 жыл бұрын
@@MedicosisPerfectionalis Thanks, so much but it’s not your fault. I don’t get bleeds too often so I’m fine. I did get injections everyday after surgery.
@madhureddy6483
@madhureddy6483 3 жыл бұрын
Plz Send me your mobile no I have type 2
@dvf4550
@dvf4550 16 күн бұрын
I and most of my family have type 2N. Our doctors have us use Wilate, not Desmopressin.
@ravitejasamudrala4
@ravitejasamudrala4 3 жыл бұрын
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
@abelsaju5155
@abelsaju5155 7 күн бұрын
the most abundant receptor on the surface of PLT is Gp2b/3a receptor
@knblaster5448
@knblaster5448 4 жыл бұрын
Thanks prof! Really enjoyed the video.
@MedicosisPerfectionalis
@MedicosisPerfectionalis 4 жыл бұрын
Thank you so much 😊
@itssomeone4407
@itssomeone4407 Жыл бұрын
Thanks alooooooooooot
@MedicosisPerfectionalis
@MedicosisPerfectionalis Жыл бұрын
You’re very welcome! Would you please help me by sharing my videos with others?
@ayecii
@ayecii 4 ай бұрын
You are amazing.
@kaptankancik9816
@kaptankancik9816 3 жыл бұрын
2N is autosomal recessive
@alraheemt47bc12
@alraheemt47bc12 Жыл бұрын
i watch your videos whenever i feel low.....
@MedicosisPerfectionalis
@MedicosisPerfectionalis Жыл бұрын
:)
@LEW_Aleha
@LEW_Aleha 4 жыл бұрын
You are the best!
@MedicosisPerfectionalis
@MedicosisPerfectionalis 4 жыл бұрын
Thank you so much 😊
@mahmed5021
@mahmed5021 4 жыл бұрын
U made it all so easy! Thank u 💌 just one request, can u please explain the vwf:Rco/vwf:Ag ratio a bit more please? Why its increased in type 1 and type 2N and decreased in type 2A , 2B, 2M? thanks!
@MedicosisPerfectionalis
@MedicosisPerfectionalis 4 жыл бұрын
Just divide vWf: Rco by vWF: Ag, and you get the answer. There is no scientific explanation here, it's just a mathematical ratio
@mahmed5021
@mahmed5021 4 жыл бұрын
Medicosis Perfectionalis thanku ! Didnt notice it before ☺
@orbetayedamarie6213
@orbetayedamarie6213 5 ай бұрын
Hii, i really love your videos :)) can i ask what books is your references for this? I am a medtech student and we have a case study concerning this. Thank you so much!
@ayaaljarrah6359
@ayaaljarrah6359 2 жыл бұрын
What's is the answer
@patrickmwai6514
@patrickmwai6514 3 жыл бұрын
On the type 2N, why did you not mention about the effect of abnormal vwf on platelet adhesion?
@mohammedalshubbar5273
@mohammedalshubbar5273 Жыл бұрын
But since type 1 is a quantitative defect, why will there be decrease in vwf activity, should it be only in antigen?
@rathodnehal2869
@rathodnehal2869 4 жыл бұрын
hey. 🖐️🖐️ doctor do you please make video of DIC please 🙏🙏
@MedicosisPerfectionalis
@MedicosisPerfectionalis 4 жыл бұрын
Coming soon
@rathodnehal2869
@rathodnehal2869 4 жыл бұрын
@@MedicosisPerfectionalis thank you doctor🙏🙏 i wait 🙏🙏
@MedicosisPerfectionalis
@MedicosisPerfectionalis 4 жыл бұрын
My pleasure 😇
@beequinn5204
@beequinn5204 3 жыл бұрын
Why would you have low factor eight and factor seven lacking in vWD, I always end up having to get plasma & blood transitions??? What happens when you can't have DDAVP
@yalda3339
@yalda3339 2 жыл бұрын
amazing sir!thank you👏💖
@MedicosisPerfectionalis
@MedicosisPerfectionalis 2 жыл бұрын
My pleasure 😇
@meredith1581
@meredith1581 Жыл бұрын
How about if you VWB factor is super high (over 500) and factor VIII is normal. Which type would that be?
@dominique573n9
@dominique573n9 2 жыл бұрын
i have been diagnosed with VWD type 3 it is very severe and need to get humate p infusions for the 7-10 days I get my menstrual cycle. My parents have had genetic testing and only my mother has the gene for VWD. My dad does not. My question is, don’t both parents need to pass this in order for their child to have VWD type 3? How does it work? Thank you!
@ifrahakbar2878
@ifrahakbar2878 3 жыл бұрын
please make videos on cancers of all the systems!
@TorieEzernack-s2w
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@hannahlutz2382
@hannahlutz2382 9 ай бұрын
Why does it say to give desmopressin with type three???
@hannahlutz2382
@hannahlutz2382 9 ай бұрын
This is stated in the table. It is wrong and should be corrected. Does repression should never be given in type three.
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@sarahlaila4379
@sarahlaila4379 3 жыл бұрын
Which tests should be done to distinguish vWD 2b from pseudo vWD?
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2M has normal multimers
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