von willibrand's disease

Question 1

what are the two types of factors in VWD?

Answer 1

tubular vWF

filamentous vWF

Question 2

where is the tubular vWF found ?

Answer 2

sub endothelium

Question 3

what is the function of the filamentous vWF?

Answer 3

trapping mesh

Question 4

what is the pattern of inheritance inn VWD ?

Answer 4

can be autosomal recessive or dominant and can affect both males and females

Question 5

what are the types of VWD ?

Answer 5

type 1
type 2
type 3

Question 6

what is the defect in type 1?

Answer 6

mild quantitive

Question 7

what is the method of inheritance in type 1 ?

Answer 7

autosomal dominant with incomplete penetrance

Question 8

what are the lab abnormalities in type 1 ?

Answer 8

increased APTT
decreased VWF-ag
decreased VWF- ac
equal Ag and Ac

Question 9

what is the treatment for type 1 VWD?

Answer 9

• DDAVP

- Tranexamic acid

Question 10

what is the defect inn type 2 VWD?

Answer 10

qualitative

Question 11

what is the inheritance pattern in VWD type 2 ?

Answer 11

autosomal dominant/recessive

Question 12

what are the lab abnormalities in type 2?

Answer 12

increased APTT
decreased VWF-ag
extremely low VWF-Ac
Ag>Ac

Question 13

what is the treatment for type 2 ?

Answer 13

• DDAVP (desmopressin)
• tranexamic acid
• concentrate

Question 14

what is the defect inn type 3 VWD?

Answer 14

severe quantitative

Question 15

what is the method of inheritance of Type 3 VWD ?

Answer 15

autosomal recessive

Question 16

what are the lab abnormalities in type 3 VWD?

Answer 16

increased APTT
extremely low VWF-Ag
extremely low VWF-AC
Ag=Ac

Question 17

what is the treatment for type 3 VWD ?

Answer 17

concentrate

Question 18

which of the three types is the most common ?

Answer 18

type 1

Question 19

which of the three types is the most serious and the rarest ?

Answer 19

type 3

Question 20

what investigations are required to make a diagnosis of VWD?

Answer 20

initial hemostasis tests

• CBC and platelete count
• PT and PTT
• fibrinogen or TT

Question 21

when do we give packed red blood cell ?

Answer 21

in cases of severe bleeding or in extremely low haemoglobin

Question 22

what are the three main treatment options of VWD ?

Answer 22

desmopressin (DVAPP)
tranexamic acid
fibrin sealant

Question 23

if the VWD is non responsive to desmopressin what is the next best step in management ?

Answer 23

give concentrates along with prophylaxis treatment for bleeding

Question 24

what is DIC ?

Answer 24

disseminated intravascular coagulation

theres a problem with both clotting and bleeding

Question 25

what are the common causes of DIC ?

Answer 25

``` sepsis trauma malignancy obstetric complications vascular malformation reaction to toxins immunological disorders ```

Question 26

what are the lab abnormalities in DIC ?

Answer 26

``` increased APTT increased PT increased TT increased FDP decreased fibrinogen decreased platelets ```

Question 27

what are the clinical manifestations of DIC?

Answer 27

petechia purpura arterial line oozing venipuncture site bleeding

Question 28

what are the significant lab abnormalities/findings ?

Answer 28

d-dimer is high fibrinogen is low platelet count is low

Question 29

what is the treatment for DIC ?

Answer 29

treat the underlying disorder platelet transfusion fresh frozen plasma and cryoprecipitate coagulation inhibitor concentrate

Question 30

what are the causes of bleeding in liver disorders ?

Answer 30

portal hypertension | peptic ulceration

Question 31

how do we make a lab diagnosis of bleeding that has been caused by liver disorders ?

Answer 31

``` prolonged PTT prolonged PTTK thrombocytopenia normal fibrinogen level decreased hepatic stores of vitamin K ```

Question 32

what is the treatment for prolonged PT/APTT?

Answer 32

fresh frozen plasma | prothrombin complex concentrate

Question 33

what is the treatment for low fibrinogen ?

Answer 33

cryoprecipitate /fibrinogen concentrate

Question 34

which lab results are an indicator of liver function ?

Answer 34

INR/PT

Question 35

what are the vitamin K dependent factors synthesized in the liver ?

Answer 35

``` factor 2 7 9 10 protein C and Protein S ```

Question 36

what are the causes of vitamin K deficiency ?

Answer 36

obstructive jaundice chronic diarrhea liver disease heamorrhagic states in infants