Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Diseases of the Blood BDS2 > Inherited Bleeding Disorders > Flashcards

Inherited Bleeding Disorders Flashcards

1
Q

what is an inherited bleeding disorders?

A

an acquired defect which affects the coagulation of the blood

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

what may an inherited bleeding disorder affect?

A

coagulation cascade, platelets, a combined deficiency

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

what is affected with coagulation cascade disorders?

A

reduction in one or more of the coagulation factors or control proteins

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

what is affected with platelet disorders?

A

number or function

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

what is factor VIII deficiency called?

A

haemophilia A

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

what is factor IX deficiency called?

A

christmas disease/haemophilia B

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

what is von willebrand’s disease?

A

reduced factor VIII level and reduced platelet aggregation

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

how does haemophilia A and B come about?

A

inheritance with a sex-linked recessive gene - defective gene on X chromosome

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

what is the severity of haemophilia due to?

A

the amount of factor produced where 1iu is normal

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

in relation to iu what is severe haemophilia?

A

less than 2% = <0.02iu/ml

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

in relation to iu what is moderate haemophilia?

A

2-9% = 0.02-0.09iu/ml

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

in relation to iu what is mild haemophilia?

A

10-40% = 0.1-0.4iu/ml

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

what is the iu of carriers of haemophilia?

A

> 50% = >0.5iu/ml

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

what is the treatment of severe and moderate haemophilia A?

A

require the use of recombinant factor VIII

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

what is the treatment of mild and carrier haemophilia A?

A

DDAVP and tranexamic acid

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

what does DDAVP do?

A

release factor VIII that has been bound to endothelial cells giving a temporary boost to factor VIII levels and clotting ability

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

what does tranexamic acid do?

A

inhibitor of fibrinolysis - keeps any clot that is formed

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

what is the treatment of severe and moderate haemophilia B?

A

recombinant factor IX

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

what is the treatment of mild and carriers of haemophilia B

A

recombinant factor IX

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

why does all severities of haemophilia B require recombinant factor IX

A

because factor IX does not bind to the endothelial cell wall so DDAVP will not work

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what are coagulation factor inhibitors?

A

antibodies which develop to factor VIII and IX

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

what is von willebrands disease caused by?

A

autosomal dominant (not X chromosome related)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

with von willebrands disease why is there poor clot activation by platelets?

A

because there is defective von Willebrand factor on platelets which interacts badly with factor VIII

24
Q

what is type 1 von willebrand disease?

A

dominant mild

25
Q

what is type 2 von willebrand disease?

A

dominant mild

26
Q

what is type 3 von willebrand disease?

A

recessive severe

27
Q

what is treatment for severe and moderate von willebrands disease?

A

DDAVP

28
Q

what is treatment for mild and carriers of von willebrands disease?

A

oral tranexamic acid

29
Q

what is the most common bleeding disorder in the UK?

A

von willebrands disease

30
Q

what are rarer bleeding disorders?

A

inherited defects of other factors in the coagulation pathway or of either the number or function of the platelets

31
Q

what procedures can be done in primary care for bleeding disorder patients?

A

non-bleeding procedures

32
Q

what does the degree of bleeding risk depend on?

A

baseline factor activity - higher factor number gives lower risk

33
Q

what must the patient treatment plan incorporate?

A

both patient and treatment risks

34
Q

where are the scottish haemophilia centres?

A

inverness, aberdeen, dundee, edinburgh, glasgow

35
Q

where does dental care for severe and moderate haemophiliacs take place?

A

in the dental treatment unit attached to haemophilia centre (apart from pros)

36
Q

where does dental care for mild and carriers of haemophilia take place?

A

treatment shared by GDP/PDS depending upon procedure and LA needs

37
Q

what dental procedures require special care?

A

administration of LA, extractions, minor oral surgery, periodontal surgery, biopsies

38
Q

what types of LA are safe in haemophilia?

A

buccal infiltration, intraligamentary, intra-papillary injections

39
Q

what types of LA are dangerous in haemophilia?

A

IDB, lingual infiltration, posterior superior nerve block

40
Q

what is the most important aspect of care for patients with medical issues?

A

prevention - dietary advice, oral hygiene, fluoride supplements, regular dental care, fissure sealant where appropriate

41
Q

what is thrombophilia?

A

increased risk of clots developing, clot formation greater than clot breakdown

42
Q

what happens if a blood clot embolises?

A

lead to blockage of major blood vessels in the heart and/or lungs - pulmonary thromboembolism

43
Q

what are the inherited hypercoagulation syndomes?

A

protein C deficiency, protein S deficiency, factor V lieden, antithrombin III deficiency

44
Q

what are the acquired hypercoagulation risks?

A

antiphospholipid syndrome, oral contraceptives, surgery, trauma, cancer, pregnancy, immobilisation

45
Q

what is thrombocytopenia?

A

reduced platelet numbers

46
Q

what is qualitative disorders?

A

normal platelet number but abnormal function

47
Q

what is thrombocythemia?

A

increased platelet numbers

48
Q

what is the cause of thrombocytopenia caused by?

A

idiopathic, drug related (alcohol, penicillin, heparin), secondary to lymphoproliferative disorder (leukaemia, myelodysplasia)

49
Q

what does platelet count need to be greater than in a primary care setting for treatment to proceed?

A

100 x 10^9/L

50
Q

what does platelet count need to be greater than in a hospital setting for treatment to proceed?

A

50 x 10^9/L

51
Q

what are thrombocythemia patients usually on?

A

aspirin to prevent clot formation

52
Q

what inherited issues cause qualitative platelet disorders?

A

Bernard Soulier syndrome, Hermansky Pudlak, Glanzmann’s thrombasthenia

53
Q

what acquired issues cause qualitative platelet disorders?

A

cirrhosis, drugs, alcohol, cardiopulmonary bypass

54
Q

what procedures can go ahead for platelet disorder patients with greater 100 x10^9/L platelets?

A

hygiene therapy, removable prosthodontics, restorative dentistry, endodontics, orthodontic treatment

55
Q

if platelet count is below 100 x 10^9/L or above 500 x10^9/L what procedures require special care?

A

extractions, minor oral surgery, periodontal surgery, biopsies

Diseases of the Blood BDS2 (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions