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BDS2 PHYSIOLOGY BAMS > Inherited Bleeding Disorders > Flashcards

Inherited Bleeding Disorders Flashcards

1
Q

what is an inherited bleeding disorder?

A

an acquired defect which affects the coagulation of the blood

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2
Q

what are the different ways in which an inherited bleeding disorder can prevent the coagulation of blood?

A
  • affects the coagulation cascade
  • affects the platelets
  • affects both of the above
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3
Q

what occurs in an inherited bleeding disorder that affects the coagulation cascade?

A
  • a reduction in one or more of the coagulation factors or control proteins
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4
Q

what is Haemophilia?

A

too LITTLE clot formed

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5
Q

what is Thrombophilia?

A

too MUCH clot formed

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6
Q

what occurs when an inherited bleeding disorder affects the platelets?

A

affects the number and function of platelets

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7
Q

what disorder results in the deficiency of clotting factor VIII?

A

Haemophilia / Haemophilia A

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8
Q

what clotting factor is affected in patients suffering with Christmas disease / Haemophilia B?

A

factor IX deficiency

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9
Q

what occurs in patients suffering with von Willebrand’s disease?

A
  • reduced factor VIII level

- reduced platelet aggression

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10
Q

what coagulation factor deficiency is common in the Ashkenazy Jew population?

A

factor XI definitely

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11
Q

what type of disease are Haemophilia A & B?

A

Inherited sex-linked recessive diseases

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12
Q

what chromosome is affected in Haemophilia A & B?

A
  • defective gene on the X chromosome

- therefor males are affected & females are carriers

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13
Q

what is the severity of Haemophilia A/B dependent on?

A

depends upon the amount of factor produced: 1iu (international unit) is ‘normal’

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14
Q

describe the concentration of coagulation factor in a person with severe haemophilia A/B?

A

< 0.02in/ml (less than 2% activity)

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15
Q

describe the concentration of coagulation factor in a patient with moderate haemophilia?

A

0.02-0.09 iu/ml (2-9% activity)

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16
Q

describe the concentration of coagulation factor in a patient with mild haemophilia?

A

0.1-0.4 iu/ml (10-40%)

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17
Q

what would the concentration of coagulation factors look like in a carrier of haemophilia?

A

<0.5 in/ml (more than 50% activity)

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18
Q

what is the ‘normal’ clotting factor international unit value in healthy patients?

A

1iu

19
Q

how is severe Haemophilia A managed?

A
  • requires the use of recombinant factor VIII (clotting factor 8 that is produced in a library)
20
Q

how are patients with mild haemophilia/carriers of the disease managed?

A
  • occasional use of DDAVP

- very mild cases may only require oral tranexamic acid

21
Q

what is DDAVP?

A

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

22
Q

what is tranexamic acid?

A
  • inhibitor of fibrinolysis

- keeps any clot that is formed

23
Q

how is Haemophilia B managed in severe and moderate cases?

A

requires the use of recombinant factor IX

24
Q

how is Haemophilia B managed in mild cases and carriers?

A

ALSO requires the use of recombinant factor IX (no alternate treatment & DDAVP useless as factor IX doesnt bind to cell walls)

25
Q

what are coagulation factor inhibitors?

A

these are antibodies which develop to factor VIII and IX (very important in patients that regularly require recombinant factor VIII & IX)

26
Q

what type of disease is von Willebrand’s disease?

A 
autosomal dominant (NOT transmitted by the X chromosome) 
- therefor both sexes equally affected
27
Q

what are the different types of von Willebrand’s disease?

A

Type One - dominant mild
Type Two - dominant mild
Type Three - recessive severe

28
Q

why does insufficient clotting occur in von Willebrand’s patients?

A

defective vW factor on platelets which interacts badly with factor VIII so poor clot activation by platelets

29
Q

how is severe & moderate von Willebrand’s disease managed?

A

DDAVP enough for most

30
Q

how is mild von Willebrand’s disease / carriers of the disease managed?

A

mild cases may only require oral tranexamic acid

31
Q

what is the most common inherited bleeding disorder in the UK?

A

von Willebrand’s disease

32
Q

where are non-bleeding dental procedures carried out for a patient with a bleeding disorder?

A
  • these can be done in primary care
33
Q

in moderate/severe Haemophilia patients, where is majority of the treatment carried out?

A

the haemophilia centre (dental treatment uni)

34
Q

what would an example of a ‘no-risk dental treatment’ be in relation to a patient with haemophilia?

A

prosthodontics care

35
Q

when is SPECIAL CARE required for patients with haemophilia?

A
  • administration of local anaesthetics (ID blocks***)
  • extractions
  • minor oral surgery
  • periodontal surgery
  • biopsies
36
Q

in terms of local anaesthesia, what types are SAFE to do on patients with Haemophilia?

A
  • buccal infiltration
  • intraligamentary injections
  • intra-papillary injections
37
Q

what types of LA are considered DANGEROUS for patient suffering with HAemophilia?

A
  • inferior alveolar nerve block
  • lingual infiltration
  • posterior superior nerve block
38
Q

what is the post-operative treatment like for patients suffering from severe/moderate haemophilia when undergoing an extraction?

A

patients should be observed overnight following surgery

39
Q

what post-operative care occurs for patients suffering with mild/carrier of haemophilia after a tooth extraction?

A

patients must be observed for 2-3 hours after surgery

40
Q

what occurs in patients with Thrombophilia?

A
  • increased risk of clots developing (clot formation ability greater than clot breakdown ability)
41
Q

what are some thrombophilia inherited hypercoagulation syndromes?

A
  • protein C deficiency
  • protein S deficiency
  • factor V Leiden
  • Antithrombin III deficiency
42
Q

how can Thrombophilia be acquired?

A
  • oral contraceptives
  • antiphospholipid syndrome
  • surgery
  • trauma
  • cancer
  • pregnancy
  • immobilisation
43
Q

what is the disorder called that causes reduced platelet numbers?

A

thrombocytopenia

44
Q

what occurs in platelet qualitative disorders?

A

normal platelet number but abnormal function

BDS2 PHYSIOLOGY BAMS (39 decks)

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