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Pediatrics > Bleeding Disorder > Flashcards

Bleeding Disorder Flashcards

1
Q

Define hemostasis:

A

Balance between (procoagulant: blood in fluid) - (anticoagulant)

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2
Q

diseases associated w\ Increased procoagulant factors vs increased in anticoagulant proteins

A
  • procoagulant: bleeding disorder

- anticoagulant: thrombotic disorder

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3
Q

Hemostasis is an interplay between

A
  • vascular endothelium
  • platelets
  • coagulation factors
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4
Q

Name the 4 phases for hemostasis:

A

1- vascular phase
2- platelet phase
3- coagulation factors
4- fibrinolytic system

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5
Q

Vwf is released in which phase of hemostasis?

A

In the vascular phase

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6
Q

Describe the vascular phase of hemostasis:

A
  • tissue injury
  • Vasoconstriction
  • CT exposure (sub-endothelium)
  • VwF release
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7
Q

What is primary and secondary hemostasis & what’s the end result?

A

Primary: platelet phase (platelet plug)
Secondary: coagulation factors (fibrin thrombus(

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8
Q

Describe the platelet phase of hemostasis:

A
  • adhesion: attach to endothelium
  • activation: activated & release granules (ADP, thromboxane A2, cofactor)
  • aggregation: w\VwF + w\platelets

[platelet plug]

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9
Q

Describe the coagulation phase of hemostasis:

A
  • initiation: making thrombin (extrinsic)
  • amplification: further thrombin (intrinsic)
  • propagation: (common)

[fibrin thrombus]

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10
Q

What are the factors for the intrinsic & extrinsic pathway?

A
  • intrinsic: 12, 11, 9, 8
  • extrinsic: 3,7
  • common: 5
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11
Q

What factor is responsible for stabilization of clot?

A

Factor 13

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12
Q

What is the role of protein C and protein S in hemostasis?

A

Inactivate factor 5 to inhibit thrombin formation

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13
Q

What is the role of antithrombin, heparin, A2 macroglobulin in hemostasis?

A

Neutralize the activity of thrombin

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14
Q

What is the role of TPA in hemostasis?

A

Converts plasminogen to plasmin to dissolve fibrin clot

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15
Q

Describe the extrinsic pathway

A
  • F7 > TF > F10 > F5
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16
Q

Describe the intrinsic pathway

A

F12 > F11 > F9 > to F10 by F8

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17
Q

Factors to consider when evaluating a bleeding disorder:

A
  • Gender
  • Age at 1st bleed
  • Site
  • Frequency
  • Severity
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18
Q

When is bleeding in a child considered normal?

A

If mild and over bony prominance like shaft (1-10yrs)

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19
Q

Usually the etiology of mucocutanous bleeding vs internal bleeding is caused by:

A
  • mucocutanous: primary

- internal: secondary

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20
Q

Delayed separation of umbilical stump is associated with which coagulation factor deficiency

A

Factor 13 deficiency

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21
Q

What are the history taking clues in the neonatal state that may indicate bleeding disorder

A

umbilical stump, veinipuncture site, cephalohematoma, ICH

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22
Q

What are the history taking clues in the vaccination and surgical procedure that may indicate bleeding disorder

A

Circumcision, hamatoma, prolonged oozing

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23
Q

It’s important to inquire about family history in bleeding disorder, such as:

A
  • bleeding in PPP in female relatives

- diagnosed bleeding disorder

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24
Q

What medications might be associated with bleeding in child

A

NSAID, warfarin, herbal (ginger, ginko, garlic)

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25
Q

Name the 3 bleeding disorder mimickers:

A
  • ehlers danlos
  • hemangioma
  • hemorrhagic talengectasia
  • child abusse
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26
Q

What are the symptoms of ehlers danlos syndrome

A
  • ecchymosis

- hyperextensible joints

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27
Q

What are the initial testing for bleeding child

A
  • CBC & film: HUS\TTP\leukemia
  • PT: F7
  • aPTT: F8, F9, F11
  • both: F10, F5, F2, F1
  • Fibrinogen: DIC & sepsis
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28
Q

In case of heparin contamination, what test is best to identify the problem?

A

Reptalise time

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29
Q

What is the mode of inheretance for hemophilia A, B, and C?

A

A & B: X-linked

C: autosomal recessive

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30
Q

In which group of population do we commonly see Hemophilia C?

A

Ashkenezi jews

31
Q

Hemophilia A is deficient in factor

A

8

32
Q

Hemophilia B is deficient in factor

A

9

33
Q

When can females be affected by hemophilia A & B?

A
  • symptomatic carrier
  • lyonization
  • homozygous recessive
  • turner syndrome
34
Q

Investigations for hemophilia:

A
  • aPTT: prolonged
  • factors: low “8 or 9 or11”
  • genetic & CVS
35
Q

What determines severity in hemophilia A & B patients?

A

Concentrations of factors

36
Q

Differentiate between mild, moderate and severe hemophilia

A

Activity of factors, frequncy, spontanous vs major trauma, and pattern

  • pattern: joint +- bleed after circumcision +- ICH [moderate]
    In severe: all of them w\out +-
    In mild: only till circumcision bleed
37
Q

What are the complications of hemophila A or B

A
  • arthropathy
  • viral transmission
  • inhibitors
38
Q

What is the aim of therapy in hemophilia treatment

A

Improve QOL, treat on demand for mild, treat prophylactically by factors for moderate to severe.

39
Q

What is the treatment for hemophilia patients

A
  • factor replacement
  • desmopressin
  • antifibrinolytic
40
Q

Name antifibrinolytics:

A

Tranxemic acid

Aminocarbonic acid

41
Q

What is the desired plasma factor level of mild hemophilia A & B

A

20-40%

42
Q

What is the desired plasma factor level of major hemophilia A & B

A

80-100%

43
Q

What is the FDA approved monoclonal AB used to treat hemophilia

A

Emicizumab

44
Q

What is the most common inherited bleeding disorder

A

VwF disease

45
Q

VwF is a carrier for which factor

A

Factor 8

46
Q

Differentiate between VwD types

A
  • type 1: mild - quantitative
  • type 2: qualitative
  • type 3: severe - absent
47
Q

What is the most common type in vwd?

A

Type 1

48
Q

In vWd, which one will be affected

Aptt or pt

A

Aptt

49
Q

In which type of vwd is riocetin reduced?

A

2B along with thrombocytopenia

50
Q

What is the defect in type 2b vWd

A

Increased affinity to vWF to GP1b

51
Q

In which types of vWb is desmopressin contraindicated and why?

A
  • type 2B: thrombocytopenia

- type 3: lack of responsne

52
Q

What are treatment options for vwd?

A
  • Desmopressin
  • antifibrinolytic
  • factor concentration (humate-P)
  • OCP
53
Q

What is the role of bleeding time in platelet disorders?

A

Abandoned

54
Q

Platelet testings include:

A
  • count
  • morphology
  • flow cytometery
  • PFA
  • electron microscopy
55
Q

What is the etiology of bernard soulier syndrome?

A

AR, Lack of GP1b-IX-V receptor

56
Q

What are the characterstics of bernard soulier?

A
  • prolonged PFA
  • thrombocytopenia
  • large platelet
57
Q

What is the etiology of Glanzzman thrombasthenia

A

Defect in GP2b-3a

58
Q

Differentiate between platelt aggregation in bernard soulier syndrome vs glanzmann thrombosthenia

A

Glanzzman: reacts to all except risocetin
Bernard: doesn’t react to risocetin

59
Q

What is the origin of acquired bleeding disorders

A

Underlying disease

“Immune, liver, renal, DIC, vitamin & absorption”

60
Q

What is the most common cause of acute thrombocytopenia in a healthy child?

A

ITP

61
Q

What is the etiology of ITP?

A

Anti platelet glycoprotein antibodies

62
Q

What are the viruses that could be associated with ITP?

A

EBV, CMV, H-pylori, MMR

63
Q

How are the platelet distroyed in ITP?

A

Antibodies coat the platelet and are recognized by splenic macrophage to be destroyed

64
Q

What are the findings in ITP?

A

All normal except for bleeding mainfestations “epistaxis, petechia, purpora, gum bleeding”

65
Q

What are the positive findings of ITP?

A
  • platelet count: <20x10^9

- platelet size: normal to large

66
Q

What is the triad of ITP?

A

Mild bleeding - normal PE - isolated thrombocytopenia

67
Q

What is the management plan for ITP?

A

Supportive

- w\ IVIG, Prednisolone -

68
Q

What determines the prognosis of ITP?

A

The time from presentation

  • acute: within 3 months
  • presistant: 3-12 months
  • chronic: >12months
69
Q

What is the most common cause of pediatric thromboembolism?

A

Provoked (2ndary to something)

70
Q

In which group of pediatric population is pediatric thromboembolism the greatest risk

A

Neonates

2nd peak during puberty\adolescence

71
Q

How to treat pediatric thromboembolism

A

Acute: heparin LMW

Subacute\chronic: warfarin + Heparin LMW

72
Q

What is the most common cause of acquired thrombophilia

A

APS antiphospholipid syndrome

73
Q

What are the positive lab findings in antiphospholipid syndrome?

A
  • positive lupus anticoagulant AB in 2 testing 2 weeks apart
  • prolonged aPTT not corrected by mixing
74
Q

How to treat antiphospholipid

A

Lifelong anticoagulant

Pediatrics (41 decks)

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