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BDS3 Human Disease > Congenital bleeding disorders > Flashcards

Congenital bleeding disorders Flashcards

1
Q

What do the normal haemostatic system rely on?

A

Vessel wall

Platelets

Von willebrands factor

Coagulation factor

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2
Q

NO vasodilation or vasoconstriction?

A

Dilation

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3
Q

Heparin?

A

Decrease clotting ability

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4
Q

Primary platelet plug??

A

When there is exposure to the subendothelium

  • it is not good to maintain haemostasis
  • fibrin formation - fibrin mesh - stabilise patent plug
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5
Q

Extrinsic pathway?

A

VII binds to tissue factor

Together activate factor 10

Activated Xa converses prothrombin to thrombin

Thrombin converts fibrinogen to fibrin

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6
Q

What is tissue factor?

A

subendothelial tissue and leukocytes

Aka factor 3

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7
Q

Causes of bleeding disorders?

A
  • Decreased number of platelets
  • Abnormal platelet function
  • Von Willebrand disease
  • Coagulation factors – deficiency or
    inhibition
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8
Q

History of bleeding disorder?

A
  • Has the patient actually got a bleeding
    disorder
  • How severe is the disorder?
  • Pattern of Bleeding
  • Congenital or Acquired
  • Mode of inheritance
  • Bruising
  • Epistaxis
  • Post-surgical bleeding
  • Menorrhagia
  • Post-partum haemorrhage
  • Post-trauma
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9
Q

Epistaxis?

A

Nose bleed

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10
Q

Mucosal bleeding?

A

Gums
GI tracts

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11
Q

Platelet type patter of bleeding?

A

Mucosal
Epistaxis
Purpura
Menorrhagia
GI

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12
Q

Coagulation factor type bleeding?

A

Articular
Muscle Haematoma
CNS

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13
Q

When does Haemophelia appear in boys?

A

Falling over in first few months of walking

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14
Q

How to differentiate congenital or acquired bleeding disorder?

A
  • Previous Episodes ?
  • Age at first event
  • Previous surgical challenges
  • Associated History
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15
Q

How is Haemophelia passed on?

A

Women are carriers and pass on to men

X -liked

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16
Q

When is Haemophelia A/B severe, moderate or mild?

A
  • <1% Severe - spontaneous bleeding
  • 1-4% Moderate
  • 5-30% Mild - go undetected until surgery or major injury
17
Q

Clinical features of Haemophelia?

A
  • Haemarthrosis
  • Muscle haematoma
  • CNS bleeding - bleeding onto to head. Children during birth or first few months of life
  • Retroperitoneal bleeding
  • Post surgical bleeding
18
Q

Questions ofr a dental pt with regards to bleeding?

A
  • Do you have a history of a bleeding
    disorder?
  • Do you have a family history of bleeding?
  • Have you had any previous operations?
  • Are you taking any anticoagulant drugs?
19
Q

Provision for mild Haemophelia with regards to dentist?

A

Enhanced preventative advice and tx GDP/community dentist

Many proceed at GDP

2 yearly review specialist dental care

20
Q

How to manage mild Haemophelia?

A

Desmopressin acetate (DDAVP)/tranexamic acid

21
Q

Management of moderate/mild hameophelia A?

A

Coagulation factor replacement FVIII

22
Q

Management for Haemophelia B?

A

coagulation factor
replacement

23
Q

Procedures that do not require factor replacement? (LA)

A

Buccal infil

Intrapapillar injection

Intra-ligament are injections

24
Q

Procedures that require factor elevation? (LA)

A

IDB

Lingual infil

25
Q

Local haemostatic measures?

A

include oxidised cellulose,
Surgicel®, resorbable gelatine sponge, Gelfoam®, cyanoacrylate tissue adhesives and surgical splints.

26
Q

Novel haemostatic agents?

A

Lyostpt® and Ankaferd Blood Stopper may also be of use

27
Q

Complications with Haemophelia tx?

A

Viral infection: HIV, HBV, HCV

Inhibitors (antibodies against regular tx e.g. DDAVP)

28
Q

Who is affected by Von willebrands?

A

Both mean and woman

Autosomal inheritance

29
Q

Bleeding type of Von willebrands disease?

A

Platelets type bleeding (mucosal)

Gums, GI etc

30
Q

Where do patents bind?

A

Subendothelial collagen

Or von willebrands factor

31
Q

Mutation in Von willebrands factors and platelet plug?

A

Can prevent form binding

32
Q

Man agent of vWF?

A

Recumbenant vWF - wont be exposed to donor blood …

VWF concentrate or DDAVP

Tranexamic acid

Topical applications

33
Q

Pt with VWF, what to do before dental procedure?

A

1g 3 times a day for 3-5 days

Swirl for 1 min an hour before dental procedure

34
Q

Desmopressin?

A

Artificial for of vasopressin

= vasoconstriction

BDS3 Human Disease (33 decks)

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