Haemophilia Flashcards
what is haemophilia
genetic disorder affecting clotting ability of blood
what is haemophilia A associated with
deficiency of factor VIII
what is haemophilia B associated with
deficiency of factor IX
when is haemophilia considered severe
plasma activity less than 1 IU/dL
when is haemophilia considered moderate
plasma activity between 2-5 IU/dL
when is haemophilia considered mild
plasma activity between 6-40 IU/dL
what is used to increase factor VIII in some patients with mild or moderate haemophilia
desmopressin
what is the role of DDAVP
releases bound factor VIII
what should not be used for pain management in people with haemophilia
aspirin due to the inhibitory effect on platelet aggregation
why is IDB contraindicated in patients with haemophilia
potential airway compromise due to haematoma in retromolar or pterygoid space
apart from IDB what other local anaesthetic injection requires haemostatic cover
lingual infiltration
what is Von Willebrand Disease
deficient or abnormal plasma proteins (vWF)
what is von willebrand factor
stabilises factor VIII and enables platelet interaction with blood vessel wall
what are four management strategies for haemophilia
coagulation factor replacement therapy
desmopressin to release endogenous stores
antifibrinolytic drugs
local haemostatic measures
what is coagulation factor replacement therapy
factor concentrate is administered via IV infusion and dental procedures should be performed within 30 mins of administration
what is desmopressin
synthetic antidiuretic hormone which stimulates release of endogenous factor VIII and vWF in patients with mild haemophilia
how long before dental procedure is DDAVP administered
1 hour
what is an example of an antifibrinolytic agent
tranexamic acid
how does tranexamic acid work
competitively inhibits activation of plasminogen to plasmin thereby inhibiting fibrin clot lysiss
how should tranexamic acid be administered
1mg two hours pre-operatively and continued for 7-10 days post op
give four examples of local haemostatic measures
sutures
oxidised cellulose
surgicel
surgical splints
