inherited bleeding disorders Flashcards
what do inherited bleeding disorders affect?
coagulation of blood
how can inherited bleeding disorders affect coagulation ?- 4 ways
haemophilia- too little coagulation
thrombophilia- too much coagulation
no of platelets
function of platelets
what chromosome does haemophilia affect?
x- affects males (females are carriers)
what factor deficiency affects haemophilia A?
factor VIII deficiency
what is required for severe deficiency of factor VIII?
recombinant factor VIII
what is required for mild/carrier deficiency for factor VIII?
DDAVP
tranexamic acid to reduce clot breakdown
what is the relationship between clot breakdown and clot formation in haemophilia?
clot breakdown is greater than clot formation
what is deficiency of haemophilia B also known as?
Christmas disease
what factor is deficient in haemophilia B?
factor IX
what is required for severe haemophilia B?
recombinant factor IX
what is required for mild/carrier haemophilia B?
recombinant factor IX
what are coagulation factor inhibitors?
ab’s- produced when given recombinant factor
what causes van willebrands disease?
factor VIII deficiency and reduced platelet function
how many types of willebrands disease are there?
type 1 and 2- mild (tranexamic acid)
3- severe DDVAP
in those with haemophilia, where should non bleeding procedures be carried out?
primary care
what does degree of bleeding in haemophiliac patients depend on?
factor activity and procedure
if severe haemophilia, where should dental care be carried out?
treatment at haemophiliac centre
where should mild/carier haemophilia procedures be carried out?
normally GDP
what LA procedures are safe?
buccal infiltration
intraligamentry
intrapapillary
what LA procedures are unsafe?
IDB
posterior superior alveolar nerve block
lingual infiltration
what is thrombophilia?
increase clotting
what is the relationship between clot formation and clot breakdown in thrombophilic patients?
clot formation is greater then clot breakdown
what other factors cause thrombophilia alongside genetics?
smoking
pregnancy
surgery
lupus anticoagulants
immobilisation
what is thrombocytopenia?
reduced no. of platelets
what is thrombocythemia?
increased no of platelets
what is qualitative platelet disorder ?
normal no of platelets but reduced platelet function
how does dental care affect platelet disorders?
if low or high platelets=primary care
if platelet dysfunction-speciality care
