Physiology Flashcards

1
Q

What is a deficiency in GpIb called?

A

Bernard-Soulier syndrome

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2
Q

What is a deficiency in vWF called?

A

von Willebrand disease

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3
Q

Failure of aggregation with ristocetin assay occurs in what diseases?

A

von Willebrand disease and Bernard-Soulier syndrome

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4
Q

What activates vWF to bind GpIb

A

Ristocetin

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5
Q

What drugs inhibit GpIIb/IIIa directly

A

Tirofiban, Eptifibatide, Abciximab (TEA)

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6
Q

What drugs inhibit ADP-induced expression of GpIIb/IIIa via P2Y12 receptor?

A

Prasugrel, Clopidogrel, Ticlopidine (PCT)

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7
Q

What drug inhibits TXA2 synthesis?

A

Aspirin

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8
Q

What are the steps of primary hemostasis?

A

Injury, exposure, adhesion, activation, aggregation

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9
Q

What anti-aggregation factor is released by endothelial cells?

A

Nitrous oxide (NO)

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10
Q

What are the anti-aggregation factors?

A

PGI2 and NO, increased blood flow, and decreased platelet aggregation

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11
Q

What are the pro-aggregation factors?

A

TXA2, decreased blood flow, increased platelet aggregation

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12
Q

What binds GpIIb/IIIa allowing linking of platelets?

A

Fibrinogen

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13
Q

What induces GpIIb/IIIa expression at platelet surface?

A

ADP binding to P2Y12 receptor

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14
Q

What helps platelets adhere to endothelium?

A

ADP

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15
Q

What mediates vasoconstriction in a damaged endothelial cell?

A

Endothelin and neural stimulation reflex

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16
Q

What is released from damaged endothelial cell that causes vasoconstriction?

A

Endothelin

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17
Q

What initiates platelet plug formation?

A

Endothelial cell injury

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18
Q

What causes a conformational change in platelets to release ADP, calcium and TXA2?

A

Binding of GpIb to vWF

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19
Q

What does vWF bind to on the platelet?

A

GpIb

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20
Q

What does vWF bind to on the endothelial cell?

A

Exposed collagen

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21
Q

What is used clinically as a thrombolytic?

A

tPA

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22
Q

What is the function of Protein C and S?

A

Cleave factors Va and VIIa

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23
Q

What is factor V Leiden?

A

Factor V resistant to protein C

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24
Q

What are the principle targets of antithrombin?

A

Factors Xa and thrombin (IIa)

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25
Q

What does heparin enhance the activity of?

A

Antithrombin

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26
Q

What factors are inhibited by antithrombin?

A

Activated factors II, VII, IX, X, XI, XII

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27
Q

What enzyme converts factors II, VII, IX, X, proteins C and S into their active forms?

A

y-glutamyl carboxylase

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28
Q

What enzyme reduces vitamin K?

A

Epoxide reductase

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29
Q

What factor carries or protects factor VIII

A

vWF

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30
Q

What coagulation factors will be low in vitamin K deficiency?

A

Factors II, VII, IX, X, protein C and S

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31
Q

What inhibits vitamin K and its enzyme, epoxide reductase?

A

Warfarin

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32
Q

What is the inheritance pattern of hemophilia C?

A

Autosomal recessive

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33
Q

What is the inheritance pattern of hemophilia A and B?

A

X-linked recessive

34
Q

What is a deficiency in factor XI called?

A

Hemophilia C

35
Q

What is a deficiency in factor IX called?

A

Hemophilia B

36
Q

What is a deficiency in factor VIII called?

A

Hemophilia A

37
Q

What is the function of plasmin?

A

Degrade the fibrin mesh and destroy coagulation factors

38
Q

What is the action of thrombolytics like alteplase, reteplase, streptokinase, and tenecteplase?

A

Promotes conversion of plasminogen to plasmin

39
Q

Which coagulation factors are part of the common pathway?

A

Factors I, II, V, X

40
Q

Which coagulation factors are part of the intrinsic pathway?

A

Factors XII, XI, IX, VIII

41
Q

Which coagulation factors are part of the extrinsic pathway?

A

Factor VII

42
Q

What inactivates bradykinin?

A

ACE

43
Q

What activates bradykinin?

A

Kallikrein

44
Q

What converts plasminogen to plasmin?

A

tPA

45
Q

Aminocaproic acid targets what part of the coagulation pathway?

A

tPA (fibrinolytic system)

46
Q

What is the target of Fondaparinux?

A

Factor Xa in the common pathway

47
Q

Apixaban and rivaroxaban target which part of the coagulation pathway?

A

Common pathway (Xa)

48
Q

Which anticoagulant has the greatest efficacy on factor Xa?

A

LMWH

49
Q

What is the function of bradykinin in the coagulation cascade?

A

Increase pain, permeability and vasodilation

50
Q

Why does the HbS molecule not migrate as far as an HbA molecule on gel electrophoresis?

A

The negative glutamic acid is replaced by neutral valine

51
Q

Why does the HbC molecule migrate the least on gel electrophoresis?

A

The negative glutamic acid is replaced by positive lysine

52
Q

On a gel electrophoresis, which Hb molecule migrates the least?

A

HbC

53
Q

On a gel electrophoresis, which Hb molecule migrates the furthest?

A

HbA

54
Q

On a gel electrophoresis, in which direction does Hb migrate?

A

Negative cathode to positive anode

55
Q

What is the presentation of ABO hemolytic disease of the newborn?

A

Mild jaundice within 24 hours of birth

56
Q

What is the treatment for ABO hemolytic disease of the newborn?

A

Phototherapy or exchange transfusion

57
Q

Who is usually susceptible to ABO hemolytic disease of the newborn?

A

Type A or B fetuses born to type O mothers

58
Q

What type of immunoglobulin is anti-D seen in Rh negative mothers?

A

IgG

59
Q

Rh type considered universal recipient of RBCs

A

Rh positive

60
Q

What type of immunoglobulins are anti-B and anti-A?

A

IgM

61
Q

Why are O blood types considered universal recipients of plasma?

A

They have both anti-A and anti-B

62
Q

Blood type considered universal recipient of plasma

A

O

63
Q

Why are O blood types considered universal donors of RBCs?

A

They do not express any A or B receptors

64
Q

Blood type that is the universal donor of RBCs

A

O

65
Q

Why are AB blood types considered universal donors of plasma?

A

Their plasma does not contain any anti-A or anti-B antibodies

66
Q

Why are AB blood types considered universal recipients of RBCs?

A

They have receptors for both A and B blood

67
Q

Blood type that is the universal recipient of RBCs

A

AB

68
Q

Blood type that is the universal donor of plasma

A

AB

69
Q

When is RhoGAM given?

A

3rd trimester and early postpartum period

70
Q

Who is given RhoGAM?

A

Rh negative mothers with an Rh positive fetus

71
Q

When do Rh negative mothers develop anti-D IgG antibodies?

A

If exposed to Rh positive blood

72
Q

What chains make up HbA2?

A

2-alpha and 2-delta chains

73
Q

Small form of adult hemoglobin present in small amounts?

A

HbA2

74
Q

Chains that make up adult Hb?

A

2-alpha and 2-beta

75
Q

Chains that make up fetal Hb?

A

2-alpha and 2-gamma chains

76
Q

Chains that make up embryonic Hb?

A

2-zeta and 2-epsilon chains

77
Q

Site of fetal erythropoiesis which occurs in 18 weeks to adult and lifelong

A

Bone marrow

78
Q

Site of fetal erythropoiesis which occurs in 10-28 weeks

A

Spleen

79
Q

Site of fetal erythropoiesis which occurs in 6 weeks to birth

A

Liver

80
Q

Site of fetal erythropoiesis which occurs in 3-8 weeks

A

Yolk sac