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My BDS2 > Congenital/Inherited Bleeding Disorders > Flashcards

Congenital/Inherited Bleeding Disorders Flashcards

1
Q

What can the bleeding disorder have an effect on?

A

Coagulation cascade

Platelets: number and function

Combination of both

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2
Q

Name common bleeding disorders.

A

Haemophilia A
Haemophilia B/Christmas disease
Von Willebrand’s disease
Thrombophilia

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3
Q

What causes rare congenital bleeding i.e factor 11 deficiency in Ashkenazy Jews (at gene level)?

A

Autosomal recessive inheritance

Common in cousin marriage

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4
Q

What are inhibitors?

A

Antibodies that develop against artificial/recombinant factor 8 and 9

Usually disappears quickly after treatment is started.

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5
Q

What must be done to counteract the problem of inhibitors?

A

Give more recombinant factor (since the immune system will attack some of it)

Just has to be done initially as the immune system stops attacking shortly after treatment starts.

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6
Q

How is haemophilia A inherited?

A

Sex linked recessive

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7
Q

What cause haemophilia A?

A

Deficiency in factor 8 (clotting factor)

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8
Q

Who is most affected by haemophilia A?

A

Males - females are carriers

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9
Q

What is crucial to note about haemophilia?

A

There is a spectrum of activity.

Could have a small deficiency, a large deficiency or factor 8 could be completely inactive.

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10
Q

How do you treat mild haemophilia A?

A

DDAVP - drug that releases factor 8 from endothelial cells

Transexamic acid - slows down clot breakdown

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11
Q

How do you treat severe haemophilia A?

A

Recombinant factor 8

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12
Q

How is haemophilia B inherited?

A

Sex linked recessive

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13
Q

What causes Haemophilia B?

A

Deficiency in factor 9 (clotting factor)

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14
Q

Who’s is most affected by haemophilia B?

A

Males - females carry

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15
Q

What is the treatment for haemophilia B?

A

Recombinant factor 9

Occasionally transexamic acid can be used.

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16
Q

What is haemophilia?

A

Tendency to bleed excessively - inability to clot

17
Q

How is Von Willebrand’s disease inherited?

A

Autosomal dominant

18
Q

Who is most affected by VW disease?

A

Both males and females equally

19
Q

What causes Von Willebrand’s disease?

A

Defect in the VW protein attached to platelets and a deficiency in factor 8.

20
Q

What determines how severe the Von Willebrand’s disease is?

A

How the gene is inherited
Type 1 = mild : dominant
Type 2 = mild : dominant
Type 3 = severe : recessive

21
Q

How do you treat Von Willebrand’s disease?

A

DDAVP - release factor 8 from endothelial cells

very mild cases - tansexamic acid can be used.

22
Q

In terms of dental treatment; how should a mild/carrier patient be treated?

A

Shared role between GDP and PDS

Patient must be reviewed every 2 years to ensure there are no changes to their condition.

23
Q

In terms of dental treatment; how should a patient with a severe bleeding disorder be treated?

A

Not by a general dentist - treated in hospital

24
Q

In those with congenital bleeding disorders; For what treatments must special care be taken?

A 
Extractions 
Some LA: blocks and lingual infiltration 
Biopsies
Periodontal treatment 
Minor ral surgery
25
Q

In those with congenital bleeding disorders; what treatments Are Ok To Carry out ?

A

Prosthodontics

Restorative procedures where safe local anaesthetic is required. (buccal, intraligamentary and intrapapillary infiltrations)

Treatment minus the use of LA

26
Q

In those with congenital bleeding disorders; What are safe types of injection?

A

Buccal infiltration
Intraligamentary infiltrations
Intrapapillary infiltrations

27
Q

In those with congenital bleeding disorders; What are unsafe types of injection?

A

Inferior alveolar nerve block
Superior posterior alveolar nerve block
lingual infiltration

28
Q

What post operative measures are taken after an extraction in those with mild congenital bleeding disorders?

A

observed for 2-3 hours after

Antibiotics may be required

Ensure clear post op instructions are given: patient knows what to do do if the bleeding doesn’t stop/starts again.

29
Q

How long must patients with severe congenital bleeding disorders be observed for after an extraction?

A

Overnight in the hospital

30
Q

List the 3 platelet abnormalities.

A

Thrombocytopenia: Reduction in platelet numbers.

Qualitative disorder: Correct number of platelets, reduced function.

Thrombocythaemia: increased platelet numbers

31
Q

*** not related to congenital disorder

What are the ACQUIRED causes Thrombocytopenia?

A

Can be Idiopathic

Some drugs can cause it;
Alcohol
Heparins
Penicillin

32
Q

*** not related to congenital disorder

What are the ACQUIRED causes qualitative platelet disorders?

A

Cirrhosis
drugs
Alcohol
cardiopulmonary bypass

33
Q

In terms of platelet numbers; treatment is safe to carry out when the number is…?

A

> 50* 10 (power of) 9

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