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Inherited Bleeding Disorders Flashcards

1
Q

what is the timescale of the coagulation cascade

A

Continuous process happens all the time in everyone

Fibrin lysis is happening at same rate of coagulation

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2
Q

what is the relationship between clot build up and break down in a coagulopathy pt

A

Clotting and clot breakdown are not matched

- Imbalance in ability to clot or not clot

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3
Q

what is the role of proteins in the coagulation cascade

A

Areas of problems if deficient
Breaks on coagulation system
- Missing not behave the way they should
- Defined control is upset

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4
Q

how should you treat a bleeding disorder pt in general

A

Treat as a normal person
- Depend on individual and disease level

Treatment planning

Be aware of potential problems

  • Hygiene phase therapy
  • Local anaesthetic
  • Extractions and surgery
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5
Q

what is the main technique to employ for dental treatment of blood disorder pt

A

PREVENTION

Avoid having to do dentistry - then not a risk as problem cannot arise (manage with prevention)

  • Oral Hygiene
  • Regular dental care
  • Fluoride supplements
  • Fissure sealant
  • Dietary advice
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6
Q

what are dental treatments that special care would be required for treating a bleeding disorder pt (4)

A

Extraction

Minor oral surgery

Periodontal surgery

Soft tissue Biopsies

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7
Q

what are dental treatments that no additional problems would be incurred for a bleeding disorder pt

A

Hygiene therapy

Removable prosthodontics

Restorative dentistry inc crowns and bridges

Endodontics

Orthodontic treatment

No blood being released from mouth or blood getting into mouth
No need for ID block

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8
Q

what do you and the pt haematologist need to do prior to dental extractions and surgery

A

Appropriate monitoring +/- treatment prior to the procedure

Preparation work is necessary to get pt properly ready so more likely to have successful treatment

Liaise with haematologist so they work out coagulation issue for the dental tx plan
- Communicate well

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9
Q

what are the 3 possible areas of defect for the inherited bleeding disorders

A

coagulation cascade

platelets

a combined deficiency

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10
Q

what could be a coagulation cascade issue

A

a reduction in one, or more, of the coagulation factors

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11
Q

what could be issues with the platelets

A

number or function

- both are key to platelet role

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12
Q

Factor VIII deficiency

A

haemophilia

haemophilia A

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13
Q

factor IX deficiency

A

Christmas disease

haemophilia B

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14
Q

Von Willebrand’s disease

A

Reduced factor VIII level

Reduced platelet aggregation

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15
Q

factor XI deficiency effects

A

common in Ashkenazy Jew population

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16
Q

what are examples of types of rare bleeding disorders

A

Inherited defects of other factors in the coagulation pathway

Inherited defect of either the number or function of the platelets

Numbers in each group are small
- Large number of different conditions registered in the UK (Over 50)

Management is complex as the bleeding does not always relate to the factor levels

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17
Q

what is the representation of bleeding disorders like in small gene pools

A

disproportionately represented

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18
Q

what are carriers of Haemophilia

A

More than enough
- Large amount of redundancy in clotting factors in normal life

Serious bleeding problem where coagulation severely tested then can be an issue

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19
Q

what type of inheritance is HA and HB

A

sex linked recessive gene
- One X chromosome that is defective

Rare for girls
Boy with will have

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20
Q

what is the CF that is effected in haemophilia

A

CF8

Make some but inadequate if slightly broken
Completely defective – no F8 made

spectrum of activity depending on how defective the gene is

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21
Q

what are haemophilia centres

A

Manages all coagulopathies
Few in Scotland

Practically - rural have distance to travel to get to major support

General plan of action with GP and local hospital and then large problems at centre

Have a special dental care team attached

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22
Q

who are more prone to get rare coagulation disorders

A 
Autosomal recessive (?) inheritance
- Bad luck – 2 recessive genes

More common in racial groups where cousin marriage frequent
- Factor XI common in Ashkenazy Jews

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23
Q

why are rare coagulation disorders difficult to manage

A

Generally, lack of clear correlation between bleeding and level of factor, so more difficult to manage
- Doesn’t always translate into bleeding problems

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24
Q

what are inhibitors to CFs

A

antibodies which develop to factor VIII and IX (clotting factor)

  • Body doesn’t make them usually
  • Binds to artificial clotting factor (not made in body) so removed from circulation so no clotting factor
  • — Slightly different to body’s – matches the receptor and triggers the sequences but tail end can be subtly different so recognised as foreign

The amount of antibody developed varies between patients

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25
when are inhibitors made
35-40% of patients develop an inhibitor when they first start treatment Usually disappear shortly after treatment - Use factor fast - fine as take a while for body to make response But if give artificial clotting factor often then need to factor in part removed by inhibitor - Give more to overcome the loss
26
how should pt be treated with artificial recombinant CFs to manage inhibitors
Need to have carefully Tx to allow inhibitors to drop most of the time so when given factors have a positive effect
27
what does a pt with a congenital bleeding disorder look like
Normal appearance, challenges with bleeding but tend to live a normal life
28
what sex is affected by haemophilia
males
29
what sex are the carriers of haemophilia
females
30
what is the severe level of haemophila
less than 0.02iu/ml | 2%
31
what are the ranges of severe to moderate to mild to carriers of haemophilia
Severe <0.02iu/ml Moderate 0.02-0.09 iu/ml Mild 0.1 – 0.4 iu/ml Carriers <0.5 iu/ml
32
why is there such a large decrease between normal CF8 level to severe haemophilia
Lots of reserved capacity for F8 - Take a while to see effect - Excessive capacity in kidneys
33
what is the clinical effect of haemophilia A
small clotting problem, more bleeding when cut themselves
34
treatment of severe and moderate haemophilia A
Require the use of recombinant factor VIII - No other option/ substitution Made by bacteria - Protein factor 8 purified and injected as needed - Depend on activity
35
treatment of mild and carriers of haemophilia a
Majority respond to DDAVP - Vasopressin, like ADH - Release stuck F8 from endothelial cells (like glucose to haemoglobin) - Released due to competitive binding - Deals with mild bleeding problems Very mild cases may only require oral tranexamic acid - Inhibitor of fibrinolysis - Slow down clot break down – important balance of 2 processes - Returns pt to normal - Slower and lower level of turnover of clots Occasionally require F8 - E.g. Challenge to vascular system e.g. extraction
36
treatment of haemophilia B
Do not response to DDAVP - F9 is not bound to endothelial cells unlike F8 Prophylactic cover requires recombinant factor IX - Only way to manage is with recombinant
37
what type of inheritance is von WIllebrand disease
Autosomal dominant - Both sexes equally affected - Normal gene and spontaneous mutation or inherited
38
what causes von Willebrand disease
Deficiency of von Willebrand factor with a reduction in factor VIII levels Platelet and coagulation F8 problem
39
what are the 3 types of von Willebrand disease
Type 1 - Dominant - Mild Type 2 - Dominant - Mild Type 3 - Recessive - Severe - less likely - 2 parents pass on recessive gene
40
what is the treatment for von willebrand disease
Majority of patients responds to DDAVP - F8 platelet aggregation Interacts with F8 in blood - Defective VW factor on platelet - Role of platelet in starting coagulation is affected - now ineffective Very mild cases may only require oral tranexamic acid
41
how are coagulation disorders treated throughout the UK
Common standardised treatment regimes for all patients. - National management of Tx Depend on level of risk that they pose Treatment should be available locally. - Mild haemophilia – dentist can do all (sometime extractions in haemophilia centre) - Hospital visits reduced. - ----More convenient and simpler for pt to have locally managed
42
in general how do severe and moderate coagulation disorder pt get treated
Majority of the treatment in the hospital except for prosthodontics. - Denture by local – small bleeding risk Treatment by GDP/PDS by arrangement
43
in general how do mild and carriers of coagulation disorders get treated
Treatment shared with GDP/PDS. - Extractions, LA Patient reviewed at the hospital every 2 years. - Check nothing needs changed, all fine
44
5 types of dental treatment where special care would be required no matter the severity of coagulation disorder
administration of LA (esp ID nerve block) extractions minor oral surgery periodontal surgery biopsies risk of blood loss
45
safe LA injection sites for coagulopathy pt (3)
Buccal infiltration Intraligamentary injections (space between tooth and gum) Intra-papillary injections (space between the teeth)
46
dangerous LA injection sites for coagulopathy pt (3)
Inferior alveolar nerve block Lingual infiltration Posterior superior (maxilla) nerve block
47
can mild haemophiliac get LA injections by GDP
yes - if choice of LA and technique is correct
48
what is the concern with extractions and dental surgeries for haemophiliacs
stopping bleeding
49
how should a extraction/surgery be approached for a haemophiliac
Appropriate cover from Haemophilia Unit A - Activity of F8 and 9 and inhibitors at basal and post injection level - Work out the amount needed to be administered prior to extraction - Haematologist prepare pt traumatic treatment Consider antibiotics - Infection will disturb clit - Routinely not used unless there is an infection Observe for 2-3 hours after surgery (minimum requirement) - Initially bleeding is stopped by platelets - Takes a few hours for coagulation to build up fibrin clump over it Comprehensive post-operative instructions - If start to bleed again - --Local treatment and potential more clotting factors needed - --Communication between GDP and haematologist
50
how long should a severe and moderate coagulopathy pt be observed post surgery/extraction
Patients should be observed overnight following surgery Risk of problem is higher with more they do Take out of domestic environment to try and ensure low activity level - Less BP and trauma to trigger bleeding
51
how long should a mild and carrier pt be observed post surgery/extraction
2-3 hours after surgery
52
what 9 possible parts of a coagulopathy pt comprehensive care team
``` Patient haematologist physiotherapist nurses dental surgeon psycho-social workers laboratory technicians orthopaedic surgeon ``` A lot involved in managing these pt Manage well - should have good quality of life
53
what is thrombophilia
Increased risk of clots developing - Opposite to haemophilia Disproportionate clotting to fibrinolytic action - Over producing clot or not enough fibrinolytic products Can be relatively undetectable until make blood clot inappropriately - E.g. DVT leads to pulmonary embolism - Mild until certain set of circumstances causes them to become visible Often an acquired condition superimposed on a genetic condition Usually possible to find a cause for the clot
54
what are 4 inherited syndromes that can lead to thrombophilia
Protein C deficiency Protein S deficiency Factor V Leiden Antithrombin III deficiency
55
what are 7 acquired syndromes that can lead to thrombophilia
Antiphospholipid syndrome - Lupus anticoagulants (sub group of lupus) Oral contraceptives Surgery Trauma Cancer Pregnancy Immobilisation risk of having high levels of clots
56
how are acquired syndromes causing thrombophilia managed
managed by medications that will reduce the level of clots
57
how are inherited syndromes of thrombophilia managed
broken down into individual groups and managed from there
58
thrombocytopenia
redcued platelet numbers
59
qualitative platelet disorders
normal platelet number but abnormal function
60
thrombocytopenia
increased platelet numbers
61
what are 3 platelet abnormalities
Thrombocytopenia Qualitative disorders Thrombocythemia
62
what are causes of thrombocytopenia
Idiopathic ``` Drug related - Alcohol - Penicillins - Heparin secondary to lymphoproliferative disorders ```
63
what is the effect on dental treatment of thrombocytopenia
dental treatment can proceed safely | providing the platelet count > 50 x10^9
64
how can qualitative platelet disorders arise
inherited
65
what are 3 rare qualitative platelet disorders
Bernard Soulier Syndrome Hermansky Pudlak Glanzmann’s thrombasthenia
66
what are 4 reasons for acquired qualitative platelet disorders
Cirrhosis Drugs Alcohol Cardiopulmonary bypass
67
what is the treatment for thrombocythemia
Uncommon disease Usually on aspirin to prevent clot formation

BDS2 BAMS (54 decks)

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