Haemophilia Flashcards

1
Q

what is haemophilia

A

genetic disorder affecting clotting ability of blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

what is haemophilia A associated with

A

deficiency of factor VIII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

what is haemophilia B associated with

A

deficiency of factor IX

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

when is haemophilia considered severe

A

plasma activity less than 1 IU/dL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

when is haemophilia considered moderate

A

plasma activity between 2-5 IU/dL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

when is haemophilia considered mild

A

plasma activity between 6-40 IU/dL

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

what is used to increase factor VIII in some patients with mild or moderate haemophilia

A

desmopressin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

what is the role of DDAVP

A

releases bound factor VIII

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

what should not be used for pain management in people with haemophilia

A

aspirin due to the inhibitory effect on platelet aggregation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

why is IDB contraindicated in patients with haemophilia

A

potential airway compromise due to haematoma in retromolar or pterygoid space

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

apart from IDB what other local anaesthetic injection requires haemostatic cover

A

lingual infiltration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

what is Von Willebrand Disease

A

deficient or abnormal plasma proteins (vWF)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

what is von willebrand factor

A

stabilises factor VIII and enables platelet interaction with blood vessel wall

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

what are four management strategies for haemophilia

A

coagulation factor replacement therapy
desmopressin to release endogenous stores
antifibrinolytic drugs
local haemostatic measures

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

what is coagulation factor replacement therapy

A

factor concentrate is administered via IV infusion and dental procedures should be performed within 30 mins of administration

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

what is desmopressin

A

synthetic antidiuretic hormone which stimulates release of endogenous factor VIII and vWF in patients with mild haemophilia

17
Q

how long before dental procedure is DDAVP administered

A

1 hour

18
Q

what is an example of an antifibrinolytic agent

A

tranexamic acid

19
Q

how does tranexamic acid work

A

competitively inhibits activation of plasminogen to plasmin thereby inhibiting fibrin clot lysiss

20
Q

how should tranexamic acid be administered

A

1mg two hours pre-operatively and continued for 7-10 days post op

21
Q

give four examples of local haemostatic measures

A

sutures
oxidised cellulose
surgicel
surgical splints

22
Q
A