Congenital bleeding disorders Flashcards
What do the normal haemostatic system rely on?
Vessel wall
Platelets
Von willebrands factor
Coagulation factor
NO vasodilation or vasoconstriction?
Dilation
Heparin?
Decrease clotting ability
Primary platelet plug??
When there is exposure to the subendothelium
- it is not good to maintain haemostasis
- fibrin formation - fibrin mesh - stabilise patent plug
Extrinsic pathway?
VII binds to tissue factor
Together activate factor 10
Activated Xa converses prothrombin to thrombin
Thrombin converts fibrinogen to fibrin
What is tissue factor?
subendothelial tissue and leukocytes
Aka factor 3
Causes of bleeding disorders?
- Decreased number of platelets
- Abnormal platelet function
- Von Willebrand disease
- Coagulation factors – deficiency or
inhibition
History of bleeding disorder?
- Has the patient actually got a bleeding
disorder - How severe is the disorder?
- Pattern of Bleeding
- Congenital or Acquired
- Mode of inheritance
- Bruising
- Epistaxis
- Post-surgical bleeding
- Menorrhagia
- Post-partum haemorrhage
- Post-trauma
Epistaxis?
Nose bleed
Mucosal bleeding?
Gums
GI tracts
Platelet type patter of bleeding?
Mucosal
Epistaxis
Purpura
Menorrhagia
GI
Coagulation factor type bleeding?
Articular
Muscle Haematoma
CNS
When does Haemophelia appear in boys?
Falling over in first few months of walking
How to differentiate congenital or acquired bleeding disorder?
- Previous Episodes ?
- Age at first event
- Previous surgical challenges
- Associated History
How is Haemophelia passed on?
Women are carriers and pass on to men
X -liked
When is Haemophelia A/B severe, moderate or mild?
- <1% Severe - spontaneous bleeding
- 1-4% Moderate
- 5-30% Mild - go undetected until surgery or major injury
Clinical features of Haemophelia?
- Haemarthrosis
- Muscle haematoma
- CNS bleeding - bleeding onto to head. Children during birth or first few months of life
- Retroperitoneal bleeding
- Post surgical bleeding
Questions ofr a dental pt with regards to bleeding?
- Do you have a history of a bleeding
disorder? - Do you have a family history of bleeding?
- Have you had any previous operations?
- Are you taking any anticoagulant drugs?
Provision for mild Haemophelia with regards to dentist?
Enhanced preventative advice and tx GDP/community dentist
Many proceed at GDP
2 yearly review specialist dental care
How to manage mild Haemophelia?
Desmopressin acetate (DDAVP)/tranexamic acid
Management of moderate/mild hameophelia A?
Coagulation factor replacement FVIII
Management for Haemophelia B?
coagulation factor
replacement
Procedures that do not require factor replacement? (LA)
Buccal infil
Intrapapillar injection
Intra-ligament are injections
Procedures that require factor elevation? (LA)
IDB
Lingual infil
Local haemostatic measures?
include oxidised cellulose,
Surgicel®, resorbable gelatine sponge, Gelfoam®, cyanoacrylate tissue adhesives and surgical splints.
Novel haemostatic agents?
Lyostpt® and Ankaferd Blood Stopper may also be of use
Complications with Haemophelia tx?
Viral infection: HIV, HBV, HCV
Inhibitors (antibodies against regular tx e.g. DDAVP)
Who is affected by Von willebrands?
Both mean and woman
Autosomal inheritance
Bleeding type of Von willebrands disease?
Platelets type bleeding (mucosal)
Gums, GI etc
Where do patents bind?
Subendothelial collagen
Or von willebrands factor
Mutation in Von willebrands factors and platelet plug?
Can prevent form binding
Man agent of vWF?
Recumbenant vWF - wont be exposed to donor blood …
VWF concentrate or DDAVP
Tranexamic acid
Topical applications
Pt with VWF, what to do before dental procedure?
1g 3 times a day for 3-5 days
Swirl for 1 min an hour before dental procedure
Desmopressin?
Artificial for of vasopressin
= vasoconstriction
