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
Local haemostatic measures?
include oxidised cellulose, Surgicel®, resorbable gelatine sponge, Gelfoam®, cyanoacrylate tissue adhesives and surgical splints.
26
Novel haemostatic agents?
Lyostpt® and Ankaferd Blood Stopper may also be of use
27
Complications with Haemophelia tx?
Viral infection: HIV, HBV, HCV Inhibitors (antibodies against regular tx e.g. DDAVP)
28
Who is affected by Von willebrands?
Both mean and woman Autosomal inheritance
29
Bleeding type of Von willebrands disease?
Platelets type bleeding (mucosal) Gums, GI etc
30
Where do patents bind?
Subendothelial collagen Or von willebrands factor
31
Mutation in Von willebrands factors and platelet plug?
Can prevent form binding
32
Man agent of vWF?
Recumbenant vWF - wont be exposed to donor blood … VWF concentrate or DDAVP Tranexamic acid Topical applications
33
Pt with VWF, what to do before dental procedure?
1g 3 times a day for 3-5 days Swirl for 1 min an hour before dental procedure
34
Desmopressin?
Artificial for of vasopressin = vasoconstriction