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Liz's Hemostasis/Coagulation > Inherited Diseases of Coagulation > Flashcards

Inherited Diseases of Coagulation Flashcards

1
Q

Importance of evaluating bleeding episodes in a patient’s history when a bleeding disorder is suspected

A

Is the bleeding out of proportion to the challenge/injury?

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2
Q

Importance of a physical examination in a patient’s history when a bleeding disorder is suspected

A

?

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3
Q

Importance of evaluating family history in a patient’s history when a bleeding disorder is suspected

A

Sex-linked vs. autosomal

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4
Q

Importance of evaluating drug history in a patient’s history when a bleeding disorder is suspected

A

?

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5
Q

Importance of evaluating the presence of existing diseases in a patient’s history when a bleeding disorder is suspected

A

?

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6
Q

Six types of coagulation disorders which may have normal screening tests

A
  • von Willebrand disease (many variations)
  • Mild hereditary factor deficiencies
  • Factor XIII deficiency
  • Dysfibrinogenemia
  • Mild qualitative platelet disorders
  • Mild acquired inhibitors
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7
Q

Factor VIII deficiency (Hemophilia A)

- How is it inherited?

A

Sex-linked recessive

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8
Q

Factor VIII deficiency (Hemophilia A)

- Treatment

A
  • Factor VIII concentrate

- DDAVP (vasopressin than stimulates teh production of VIII:C and VIII:vWF)

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9
Q

Factor IX deficiency (Hemophilia B)

- How is it inherited?

A

Sex-linked recessive

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10
Q

Factor IX deficiency (Hemophilia B)

- Treatment

A
  • Factor IX concentrates
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11
Q

von Willebrand disease

- How is it inherited?

A
  • Autosomal dominant

- Autosomal recessive

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12
Q

von Willebrand disease

- Bleeding episodes

A
  • Mucus membrane bleeding
  • Superficial wound bleeding
  • Bleeding w/ other hemostatic chanllenges (surgery, childbirth)
  • Menorrhagia
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13
Q

von Willebrand disease

- Coagulation test results

A
  • PLT: normal
  • BT: prolonged
  • PLT aggregation studies: absent w/ ristocetin, normal w/ other agonists
  • PT: normal
  • APTT: prolonged (usually)
  • VIII assay: decreased (usually)
  • VIII: vWF Ag decreased
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14
Q

von Willebrand disease

- Treatment

A
  • Humate
  • DDAVP
  • AMICAR
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15
Q

Hemophilia A vs. von Willebrand disease

- APTT

A
  • Hemophilia A: prolonged

- von Willebrand disease: prolonged (usually)

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16
Q

Hemophilia A vs. von Willebrand disease

- Bleeding time

A
  • Hemophilia A: normal

- von Willebrand disease: prolonged

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17
Q

Hemophilia A vs. von Willebrand disease

- Factor VIII activity

A
  • Hemophilia A: decreased

- von Willebrand disease: decreased (usually)

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18
Q

Hemophilia A vs. von Willebrand disease

- VIII: vWF Ag

A
  • Hemophlia A: ??

- von Willebrand disease: decreased

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19
Q

Hemophilia A vs. von Willebrand disease

- Platelet aggregation

A
  • Hemophilia A: ??

- von Willebrand disease: absent w/ ristocetin, normal w/ other agonists

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20
Q

Hemophilia A vs. von Willebrand disease

- PT

A
  • Hemophilia A:

- von Willebrand disease: normal

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21
Q

Congenital Afibrinogenemia

- Bleeding episodes

A

Bleeding w/ every hemostatic challenge

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22
Q

Congenital Afibrinogenemia

- Coagulation test results

A
  • PT: increased
  • APTT: increased
  • TCT: increased
  • Fibrinogen: absent
  • BT: increased
  • PLT aggregation: None (WHY??)
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23
Q

Congenital Afibrinogenemia

- Treatment

A

Cryoprecipitate

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24
Q

Congenital Hypofibrinogenemia

- Bleeding episodes

A

Mild to absent bleeding episodes

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25
Q

Congenital Hypofibrinogenemia

- Coagulation test results (PT, APTT, TCT, fibrinogen)

A
  • PT: increased
  • APTT: increased
  • TCT: increased
  • Fibrinogen: low (< 100 mg/dL)
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26
Q

Congenital Hypofibrinogenemia

- Treatment

A

Cryoprecipitate

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27
Q

Congenital Dysfibrinogenemia

- Bleeding episodes

A

Asymptomatic to mild bleeding episodes

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28
Q

Congenital Dysfibrinogenemia

- Coagulation test results (PT, APTT, TCT, reptilase)

A
  • PT: variable
  • APTT: variable
  • TCT: increased
  • Reptilase: increased
29
Q

Congenital Dysfibrinogenemia

- Treatment

A

Cryoprecipitate

30
Q

Factor II deficiency

- How is it inherited?

A

?

31
Q

Factor II deficiency

- Bleeding episodes

A
  • Postoperative bleeding
  • Epistaxis
  • Menorrhagia
  • Easy bruising
32
Q

Factor II deficiency

- Coagulation test results (PT, APTT)

A
  • PT: increased

- APTT: increased

33
Q

Factor II deficiency

- Treatment

A

PCC, raise to 75%

34
Q

Factor V deficiency

- How is it inherited?

A

?

35
Q

Factor V deficiency

- Bleeding episodes

A

Mild bleeding episodes

36
Q

Factor V deficiency

- Coagulation test results (PT, APTT)

A
  • PT: increased

- APTT: increased

37
Q

Factor V deficiency

- Treatment

A

FFP, raise level to 75%

38
Q

Factor VII deficiency

- How is it inherited?

A

?

39
Q

Factor VII deficiency

- Bleeding episodes

A

Moderate to severe hemorrhagic symptoms including spontaneous hemorrhage, hemarthroses, postoperative bleeding

40
Q

Factor VII deficiency

- Coagulation test results (PT, APTT)

A
  • PT: increased

- APTT: normal

41
Q

Factor VII deficiency

- Treatment

A

PPC*, FFP

* PCC not effective by itself, level too low in product

42
Q

Factor X deficiency

- How is it inherited?

A

?

43
Q

Factor X deficiency

- Bleeding episodes

A

Moderate to severe hemorrhagic symptoms including ecchymoses, CNS bleeding, excessive bleeding after childbirth

44
Q

Factor X deficiency

- Coagulation test results (PT, APTT)

A
  • PT: increased

- APTT: increased

45
Q

Factor X deficiency

- Treatment

A

PCC, raise level to 75%

46
Q

Factor XI deficiency

- How is it inherited?

A

?

47
Q

Factor XI deficiency

- Bleeding episodes

A

Mild to moderate bleeding episodes

48
Q

Factor XI deficiency

- Coagulation test results (PT, APTT)

A
  • PT: normal

- APTT: increased

49
Q

Factor XI deficiency

- Treatment

A

FFP, raise level to 75%

50
Q

Factor XII deficiency

- How is it inherited?

A

?

51
Q

Factor XII deficiency

- Bleeding episodes

A

Asymptomatic

52
Q

Factor XII deficiency

- Coagulation test results (PT, APTT)

A
  • PT: normal

- APTT: increased

53
Q

Factor XII deficiency

- Treatment

A

None needed

54
Q

Factor XIII deficiency

- How is it inherited?

A

?

55
Q

Factor XIII deficiency

- Bleeding episodes

A

Bleeding w/ every hemostatic challenge including umbilical cord bleeding, delayed wound healing, intracranial hemorrhage

56
Q

Factor XIII deficiency

- Coagulation test results (PT, APTT, urea solubility)

A
  • PT: normal
  • APTT: normal
  • Abnormal urea solubility test
57
Q

Factor XIII deficiency

- Treatment

A

Cryoprecipitate or FFP every 3 weeks

58
Q

Fletcher Factor deficiency

- How is it inherited?

A

?

59
Q

Fletcher Factor deficiency

- Bleeding episodes

A

Asymptomatic

60
Q

Fletcher Factor deficiency

- Coagulation test results (PT, APTT)

A
  • PT: normal

- APTT: increased

61
Q

Fletcher Factor deficiency

- Treatment

A

None needed

62
Q

Fitzgerald Factor deficiency

- How is it inherited?

A

?

63
Q

Fitzgerald Factor deficiency

- Bleeding episodes

A

Asymptomatic

64
Q

Fitzgerald Factor deficiency

- Coagulation test results (PT, APTT)

A
  • PT: normal

- APTT: increased

65
Q

Fitzgerald Factor deficiency

- Treatment

A

None needed

66
Q

Types of bleeds for both Hemophilia A and B in infants

A

Excessive bleeding from circumcism

67
Q

Types of bleeds for both Hemophilia A and B in toddlers

A
  • Bleeding/large ecchymoses w/ teething, crawling, learning to walk/fall
  • Bleeding into mouth/pharynx → suffocation
  • Lifting may cause hematuria
68
Q

Hemophilia A and B

- Bleeding episodes

A

Major disability is from crippling hemarthrosis
- Bleeding into muscles/soft tissue
- Circulation is blocked
- Muscle atrophy/wasting
- Gangrene may set in
Major cause of death is intracranial hemorrhage

69
Q

Hemophilia A and B

- Coagulation test results

A
  • BT, PLT, PT: normal
  • APTT: prolonged
  • Mixing studies: PNP corrects
  • Confirm w/ Factor VIII or IX assay: decreased

Liz's Hemostasis/Coagulation (10 decks)

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