Thanks for the lecture. I am a South African medical doctor. I have typed what I hear and a few more points to help the students - and myself - hope this helps.
von Willwbrands Disease - Most Important Points Most common congenital bleeding disorder, affecting 1-2 percent of the population.
vWF - is synthesized by the vascular endothelium, and a cofactor for platelet adhesion and a co factor for factor VIII
Forms the clot for the vascular injury site - and attaches
3 types- I,II,III
mild quantitative, 90 % present - most common - less serious quantitative defect - less vWF - more serious Absent - vWF is almost absent - most serious Clinical features: Bleeding particularly in children Mild - asymptomatic Mucosal or cutaneoous bleeding - easy bruising, gingival bleeding, epistaxis, menorrhagia Moderate to severe Soft-tissue hematomas, petechiae (rare), GI Bleed and hemarthroses Investigations: Coagulation profile - increased bleeding time and PTT low factor VIII (5 to 50%) (PT and factor VIII is normal - also blood group O has 30% less vWF) platelet count normal reduced ristocetin cofactor activity (normally causes vWF to bind platelets tightly) analysis of vWF multimeres Treatment Avoid Asprin and Heparin Desmopressin (DDAVP) for type I vWD causes the release of vWF and factor VIII from endothelial cells (after 4 doses patients develop tachyphylaxis) Highly purified factor VIII concentrate containing vWF (HEMATE) in selected cases and Type II conjugated estrogens (increase vWF - oral contraceptives) Platelet transfusions cyroprecipitates Control the bleeding - epistaxis - surgical strips Prognosis fluctuates and improves during pregnancy and with age http://www.youtube.com/watch?v=q5r7UFiRPh8&feature=player_embedded
POSTED BY DR SLEEP - DR YACOOB OMAR CARRIM AT 7:24
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Thanks for the lecture. I am a South African medical doctor. I have typed what I hear and a few more points to help the students - and myself - hope this helps.
von Willwbrands Disease - Most Important Points
Most common congenital bleeding disorder, affecting 1-2 percent of the population.
vWF - is synthesized by the vascular endothelium, and a cofactor for platelet adhesion and a co factor for factor VIII
Forms the clot for the vascular injury site - and attaches
3 types- I,II,III
mild quantitative, 90 % present - most common - less serious
quantitative defect - less vWF - more serious
Absent - vWF is almost absent - most serious
Clinical features: Bleeding particularly in children
Mild -
asymptomatic
Mucosal or cutaneoous bleeding - easy bruising, gingival bleeding, epistaxis, menorrhagia
Moderate to severe
Soft-tissue hematomas, petechiae (rare), GI Bleed and hemarthroses
Investigations:
Coagulation profile - increased bleeding time and PTT
low factor VIII (5 to 50%) (PT and factor VIII is normal - also blood group O has 30% less vWF)
platelet count normal
reduced ristocetin cofactor activity (normally causes vWF to bind platelets tightly)
analysis of vWF multimeres
Treatment
Avoid Asprin and Heparin
Desmopressin (DDAVP) for type I vWD
causes the release of vWF and factor VIII from endothelial cells
(after 4 doses patients develop tachyphylaxis)
Highly purified factor VIII concentrate containing vWF (HEMATE) in selected cases and Type II
conjugated estrogens (increase vWF - oral contraceptives)
Platelet transfusions cyroprecipitates
Control the bleeding - epistaxis - surgical strips
Prognosis
fluctuates and improves during pregnancy and with age
http://www.youtube.com/watch?v=q5r7UFiRPh8&feature=player_embedded
POSTED BY DR SLEEP - DR YACOOB OMAR CARRIM AT 7:24
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