inherited bleeding disorders

Question 1

what do inherited bleeding disorders affect?

Answer 1

coagulation of blood

Question 2

how can inherited bleeding disorders affect coagulation ?- 4 ways

Answer 2

haemophilia- too little coagulation
thrombophilia- too much coagulation
no of platelets
function of platelets

Question 3

what chromosome does haemophilia affect?

Answer 3

x- affects males (females are carriers)

Question 4

what factor deficiency affects haemophilia A?

Answer 4

factor VIII deficiency

Question 5

what is required for severe deficiency of factor VIII?

Answer 5

recombinant factor VIII

Question 6

what is required for mild/carrier deficiency for factor VIII?

Answer 6

DDAVP
tranexamic acid to reduce clot breakdown

Question 7

what is the relationship between clot breakdown and clot formation in haemophilia?

Answer 7

clot breakdown is greater than clot formation

Question 8

what is deficiency of haemophilia B also known as?

Answer 8

Christmas disease

Question 9

what factor is deficient in haemophilia B?

Answer 9

factor IX

Question 10

what is required for severe haemophilia B?

Answer 10

recombinant factor IX

Question 11

what is required for mild/carrier haemophilia B?

Answer 11

recombinant factor IX

Question 12

what are coagulation factor inhibitors?

Answer 12

ab’s- produced when given recombinant factor

Question 13

what causes van willebrands disease?

Answer 13

factor VIII deficiency and reduced platelet function

Question 14

how many types of willebrands disease are there?

Answer 14

type 1 and 2- mild (tranexamic acid)
3- severe DDVAP

Question 15

in those with haemophilia, where should non bleeding procedures be carried out?

Answer 15

primary care

Question 16

what does degree of bleeding in haemophiliac patients depend on?

Answer 16

factor activity and procedure

Question 17

if severe haemophilia, where should dental care be carried out?

Answer 17

treatment at haemophiliac centre

Question 18

where should mild/carier haemophilia procedures be carried out?

Answer 18

normally GDP

Question 19

what LA procedures are safe?

Answer 19

buccal infiltration
intraligamentry
intrapapillary

Question 20

what LA procedures are unsafe?

Answer 20

IDB
posterior superior alveolar nerve block
lingual infiltration

Question 21

what is thrombophilia?

Answer 21

increase clotting

Question 22

what is the relationship between clot formation and clot breakdown in thrombophilic patients?

Answer 22

clot formation is greater then clot breakdown

Question 23

what other factors cause thrombophilia alongside genetics?

Answer 23

smoking
pregnancy
surgery
lupus anticoagulants
immobilisation

Question 24

what is thrombocytopenia?

Answer 24

reduced no. of platelets

Question 25

what is thrombocythemia?

Answer 25

increased no of platelets

Question 26

what is qualitative platelet disorder ?

Answer 26

normal no of platelets but reduced platelet function

Question 27

how does dental care affect platelet disorders?

Answer 27

if low or high platelets=primary care
if platelet dysfunction-speciality care