3. Medically Complex Patients

Question 1

Describe the difference between primary and secondary hemostasis

Answer 1

Primary Hemostasis

• Injury to Blood vessel –>Platelet plug formation
• Platelets and coagulation factors in plasma and vessel wall interact to form platelet plug at injury site

Secondary Hemostasis
-Coagulation and clotting factors lead to production of fibrin which is deposited to form blood clot

Question 2

What happens when primary or secondary hemostasis is disrupted

Answer 2

prolonged bleeding

Question 3

People with inherited coagulation disorders results in a decrease in what

Answer 3

factors in coagulation cascade

Question 4

Hemophilia A is a decrease in factor….

Answer 4

VIII

Question 5

Hemophilia B is a decrease in factor _

Answer 5

IX

Question 6

Hemophilia B is also called

Answer 6

christmas disease

Question 7

Von Willebrands Diseases is a decrease in

Answer 7

VW factor

Question 8

What is the role of VW factor

Answer 8

-Binds VIII then the complex binds platelets for activation of coaggulation cascade

Question 9

What happens to factor VIII in the absence of VW factor

Answer 9

it is degraded

Question 10

What cells produce factor VIII

Answer 10

endothelial cells

Question 11

Describe the two components of Factor VIII

Answer 11

Von- Willebrand component –> initial platelet aggregation

Factor VIII component –> activates factor X in the intrinsic coagulation pathway

Question 12

What is the role of factor IX

Answer 12

Also activates factor X in the intrinsic coaggulation pathway

Question 13

What is the inheritance pattern of hemophilia A

Answer 13

X-linked recessive (only seen in men- females are carriers )

Question 14

30% of Hemophilia A cases are a result of what

Answer 14

spontaneous mutation

Question 15

Which is more common hemophilia A or B

Answer 15

A (85% hemophiliacs are Hemophilia A)

Question 16

The classification of hemophilia is based on what

Answer 16

the % procoagulant present

Question 17

Severe, Moderate, Mild hemophilia is defined as…

Answer 17

• Severe= <1% factor VIII non detectable in plasma spontaneous bleeding in joints and muscles
• Moderate= 1-4% Factor VIII. Less severe bleeding after small trauma
• Mild (5-25% Factor VIII) may not manifest until after trauma or surgery

Question 18

What is the normal % of factor VIII in the blood (aka non-hemophiliacs)

Answer 18

55-100%

Question 19

IS there a cure for hemophilia

Answer 19

no

Question 20

What should be done to minimize the health consequences of hemophilia

Answer 20

• Avoid injury and meds that promote bleeding
• Good nutrition
• Good oral hygiene
• IV administration of deficient clotting agent

Question 21

What are signs of hemophilia

Answer 21

• Intracranial hemorrhage
• Prlonged nose bleeds
• Bruise easily
• Warm, Painful swollen joints with decreased movement
• GI hemorrhage

Question 22

Inheritance pattern for hemophilia B is

Answer 22

X-linked recessive (same as hemophilia A)

Question 23

What is the inheritance pattern of Von Willebrand’s disease

Answer 23

autosomal dominant (can also be aquired)

Question 24

How can von willebrand’s disease be acquired

Answer 24

• Via thrombocytopenia

- Via autoantibodies against VWF

Question 25

Classification of VW disease is based on

Answer 25

- Inherited v.s acquired - Depends on platelet and factor defects - As mild as nosebleeds, gingival bleeding and bruising to severe internal bleeding

Question 26

What bleeding disorders can only boys get

Answer 26

Hemophilia A and B

Question 27

Blood screening tests for these three bleeding disorders tests the ability of the _ clotting pathway with the _ test

Answer 27

Intrinsic pathway... Activated Partial thromboplastin time (APTT)

Question 28

What is the blood test that measures the function of the extrinsic clotting pathway

Answer 28

Prothombin time (PT)

Question 29

If coagulation test results are _ seconds more than the control this is considered abnormal

Answer 29

2 sec

Question 30

The time required to stop bleeding is a measure of what

Answer 30

the platelet function

Question 31

What is the normal APTT and PT times

Answer 31

APTT=24-38 sec | PT= 11-17 sec

Question 32

Which of the three bleeding disorders will have a prolonged APTT

Answer 32

All of them

Question 33

Which of the three bleeding disorders will have a prolonged PT

Answer 33

None of them

Question 34

What bleeding disorders will have a decreased factor VIII

Answer 34

Hemophilia A and Von willebrands

Question 35

Which diseases will have an increased bleeding time

Answer 35

only Von Willebrand's because of the impaired platelet function

Question 36

Platelet counts in Von Willebrands disease will be (decrease/normal)

Answer 36

Could be either depends on the type

Question 37

Platelet counts in hemophilia will be (decreased/normal)

Answer 37

normal

Question 38

What are the clinical hallmarks of hemophilia

Answer 38

- Easy bruising - Prolonged/fatal bleeding after surgery or trauma - Will NOT necessarily have excessive bleeding after minor cuts and abrasions - Joint/muscle hemorrhages (Hemarthroses and hematomas in muscle) - Painful arthritis (degenerative joint disease) - Pseudotumors (hemorrhagic pseudocysts may occur) in the jaw - Bloodborne viral infections more common from transfusions (HIV and Hepatitis)

Question 39

Possible sequelae of a hematoma in the muscle could be what

Answer 39

compression of nerves (paralysis) or lead to vascular/airway obstruction

Question 40

What are the oral manifestations of bleeding disorders

Answer 40

- Petechiae - Ecchymosis - Generalized gingival hemorrhage - Purpura

Question 41

As a dentist if you suspect a bleeding disorder what should you do

Answer 41

- Refer to pediatrician for blood screen - If pediatrician is suscpicious the patient is refered to a hematologist - Consultation with the hematologist must be obtained before performing invasive dental care

Question 42

(T/F) Patients with a bleeding disorder can often receive basic outpatient care

Answer 42

t

Question 43

Is premedication required for supragingival scaling in mild/moderate cases? Subgingival scaling?

Answer 43

no... MAYBE

Question 44

In order to avoid extractions _ should be done with the teeth to avoid a platelet transfusion

Answer 44

endodontics

Question 45

Patients should be treated with what before invasive dentistry

Answer 45

- their deficient factor | - Amicar (from the hematologist) 1 hr prior

Question 46

What meds should be avoided in people with bleeding disorders

Answer 46

aspirin and ibuprofen (these decrease platelet function- blood thinners) **Use acetaminophen**