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Own peds boards > Coagulation > Flashcards

Coagulation Flashcards

1
Q

Pattern; impaired plt to plt

A

Glanzmann’s

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2
Q

Pattern: impaired GP11b/IIIa glycoprotein

A

Glanzmann

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3
Q

Pattern: impaired GP1b, V, IX

A

Bernard Soulier Syndrome

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4
Q

Pattern: impaired plt-vwf

A

Bernard Soulier syndrome

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5
Q

Pattern: mild to severe bleeding of mucosa, GI, epistaxis, normal PT/PTT, delayed bleeding time, normal plts and morphology

A

Glanzmann

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6
Q

Pattern: Prolonged bleeding time, severe mucosal bleeding, giant platelets

A

Bernard Soulier Syndrome

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7
Q

Rx for Glanzman and Bernard-Soulier syndrome

A

platelet transfusion, aminocaproic acid, rFVIIA

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8
Q

What questions should you ask if thrombocytopenia seen in neonate?

A

Ask maternal history, dysmorphic features such as skeletal defects, hemangiomas, IUGR, blueberry muffin

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9
Q

What are some acquired platelet causes?

A

Uremia that strips glycoproteins, cardiopulmonary bypass, ECMA, aspirin (irreversible), NSAIDS (transient), ethanol, clopidogrel, ticlopidine

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10
Q

Name four quantitative congenital platelet abnormalities leading to small platelets.

A
  1. Bone marrow failure, Fanconi anemia, pancytopenia
  2. TAR
  3. Congenital amegakaryocytic thrombocytopenia
  4. Wiskott-Aldrich
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11
Q

What is the difference in presentation of neonatal-allo-immune ITP and Maternal ITP?

A

Absent or present of maternal thrombocytopenia (chronic ITP, SLE history)

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12
Q

Rx for neonatal-allo-immune ITM versus maternal ITP?

A

1) Maternal platelets, PLA- platelets, IVIg

2) steroids and/or IVIg given to mother, prenatal or to baby post-natal

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13
Q

Besides ITP what other causes of thrombocytopenia in newborn

A

congenital marrow failure disorders, DIC, sepsis, viral infection (CMV, EBV, rubella)

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14
Q

Pattern: isolated thrombocytopenia, peak age 2-4 yrs, post-infectious, self-limited, petechiae, purpura, epitaxis, gingival bleeding

A

acute ITP

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15
Q

When does acute ITP become chronic

A

6 months

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16
Q

Pattern: mild to moderate bleeding, mucocutaneous bleeding, ecchymosis, menorrhagia, post-surgical bleeding (s/p tonsillectomy, dental extraction)

A

vWD

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17
Q

Name three types of vWD, inheritance pattern, and what the problem is

A

1) AD, not enough vWF, 80%
2) AD, doesn’t work, 20%
3) AR, absent, rare

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18
Q

Name causes of quantitative acquired platelet disorders

A
  1. Destruction
  2. ITP
  3. auto-immune disease (SLE, immunodeficiency, AIDS)
  4. Drug-induced (HIT, quinidine)
  5. Neonatal
  6. Hypersplenism
  7. IV trapping, DIC, Kasabach Merritt, HUS
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19
Q

Pattern: mucocutaneous bleeding and family history, PT nml, PTT nml to prolonged, bleeding time prolonged, vWF antigen absent to decreased, ristocetin cofactor assay decreased, factor VIII nml to decreased

A

vWD

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20
Q

Rx for vWD: mild to moderate and severe

A

mild to moderate: DDAVP for type 2, aminocapric acid inhibits fibrinolysis
severe: replacement with factor VIII, vWF concentrate or cryo fibrinogen, FVIII and vWF

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21
Q

What is the major side effect of DDAVP

A

hyponatremia

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22
Q

What are three drugs that interfere with Vit K

A

warfarin, INH, phenobarbital

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23
Q

Pattern: catastrophic bleeding at birth

A

maternal ingestion of Vit K antag

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24
Q

Pattern: purpura, oozing from umbilical cord, circumcision bleeding, GI, hematuria within 1 wk

A

failure to administer K at birth

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25
Q

Pattern: purpura, oozing from umbilical cord, circumcision bleeding, GI, hematuria within 1 to 3 months

A

Vit K deficiency from breastfeeding

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26
Q

Hemophilia A from deficiency of which factor

A

VIII

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27
Q

Hemophila B from deficiency of which factor

A

IX

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28
Q

Pattern: PT normal, PTT prolonged, deep soft tissue/joint bleeds

A

hemophilia A or B

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29
Q

Pattern: myocardial infarction, stroke, DVT, miscarriages

A

hypercoagulable state

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30
Q

What labs to get for hypercoaguable workup

A

PT, PTT, INR, protein C/S, factor V leiden/activated protein C, MTHFR mutation, prothrombin mutation, DRVVT, ANTIII

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31
Q

Protein C is similar to which molecules

A

All serine protease, similar to Vit K dependent factors

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32
Q

Protein S function

A

cofactor for protein C

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33
Q

Pattern: neonatal pupura fulmins

A

homozygous protein c/s deficiency

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34
Q

Pattern: risk of VTE

A

heterozygous protein c/s deficiency

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35
Q

Pattern: warfarin skin necrosis

A

acquired protein c/s deficiency

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36
Q

Pattern: sudden, massive microthrombosis, skin, necrosis, DIC

A

neonatal purpura fulminans, think of homozygous protein c/s deficiency, consider sepsis

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37
Q

Rx for neonatal purpura fulminans

A

FFP, heparin, then long term warfarin

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38
Q

Long term complication of VitK deficiency

A

chornic hemarthrosis leading to joint destruction, infections comlications (HIV, hepatitis A, V, C, parvovirus), muscle hematomas causing contractures and atrophy

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39
Q

Pattern: heparin fails to anticoagulate

A

low ATIII

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40
Q

Pattern: recurrent severe VTE, fmhx thrombosis (PTT does change), DIC, liver dysfunction, nephrotic syndrome, IBD, L-asparaginase

A

ATIII deficiency

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41
Q

Pattern: late onset thrombosis

A

factor V Leiden

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42
Q

function of mutation in Factor V Leiden

A

prevents protein C from cleaving it

43
Q

Pattern: VTE, hx of DVT, increased prothrombin concentration

A

prothrombin gene mutation

44
Q

Pattern: increased risk of arterial and VTE, increased homocysteine which causes vascular damage

A

hyperhomocysteinemia

45
Q

Pattern: increased PTT, doesn’t cause bleeding instead recurrent VTE, stroke, recurrent pregnancy loss

A

antiphospholipid syndrome

46
Q

What are some acquired thrombotic states?

A

nephrotic, IBD, malignancy, HIT

47
Q

Pattern: relative or absolute thrombocytopenia, heparin exposure >5 days, serotonin release assay positive, heparin associated antibody positive

48
Q

Pattern: similar to ITP. increasing pallor, normochromic normocytic anemia with low retic count, normal WBC and platelets, in todders with antecedent infection

A

transient erythroblastopenia of childhood

49
Q

Pattern: infant, macrocytic anemia, short stature, upper limb abnormalities, non-megaloblastic anemia

A

Diamond Blackfan Anemia

50
Q

Rx for Diamond Blackfan Anemia

51
Q

Pattern: Child, has non-meg anemia, short stature, upper limb abnormalities, leukemia in 15%, progression to severe aplastic anemia

A

Fanconi’s

52
Q

Pattern: recurrent skin infection, photosensitivity, muscle weakness, ataxia, sensory loss, nystagmus

A

Chediak Higashi Syndrome

53
Q

Giant neutrophil granules

A

Chediak Higashi Syndrome

54
Q

Pattern: oculocutaneous albinism, defects in monocytes, lymphocytes, NK cells, CN and peripheral neuropathies

A

Chediak Higashi Sydrome

55
Q

Pattern: early diagnosis, severe infection with Staph, E. coli, pseudomonas, diagnostic bone marrow shows developmental arrest, no mature neutrophils

A

Kostmann’s

56
Q

Pattern: 8 to 11 month old, increased minor but not serious infections, just neutropenia

A

nothing abnormal except neutropenia

57
Q

Pattern: c. perfringens, recurrent episodes of fever, mouth ulcers and furunculosis, oscillation of ANC every 9 to 21 days

A

Cyclic neutropenia

58
Q

How must you work up cyclic neutropenias?

A

CBCs 2x/week over at least 3 weeks

59
Q

Folate or B12?

Hypersegmented polys

60
Q

Folate or B12?

Poor diet, preb, chronic hemolysis

61
Q

Folate or B12?

Ileal resection

62
Q

Folate or B12?

Diphyllobothrium latum infection

63
Q

Folate or B12?

Breast fed/vegetarian

64
Q

Alpha or beta thal?

Blacks, SE Asian

65
Q

Alpha or beta thal?

Mediterranean

66
Q

Alpha or beta thal?

onset newborn

67
Q

Alpha or beta thal?

after 6 months

68
Q

Alpha or beta thal?

jaundice

69
Q

Alpha or beta thal?

hepatosplenomegaly

70
Q

Alpha or beta thal?

HbH, HbBarts

71
Q

Alpha or beta thal?

HbA2 or HbF

72
Q

Alpha or beta thal?

Hydrops fetalis

73
Q

Alpha or beta thal?

Hair on ends on Xray

74
Q

Alpha or beta thal?

Milder form characteried more by ineffective erthyropoiesis than hemolytic anemia

75
Q

Alpha or beta thal?

Severity is proportional to dose of defective gene

76
Q

Alpha or beta thal?

HbH is present

77
Q

Alpha or beta thal?

Elevations in HbA2 and HbF

78
Q

Alpha or beta thal?

Errors in globin

79
Q

Absorption site Fe

80
Q

Absorption site B12

A

terminal ileum

81
Q

Absorption site folate

A

proximal jejunum

82
Q

Iron deficiency or lead toxicity

Hypochromic/microcytic anemia

83
Q

Iron deficiency or lead toxicity

Acute encephalopathy

84
Q

Iron deficiency or lead toxicity

elevated Free Erythrocyte Protoporphyrin (FEP)

85
Q

Iron deficiency or lead toxicity

common in children of low income families

86
Q

Extravascular or intravascular hemolysis?

IgG mediated

87
Q

Extravascular or intravascular hemolysis?

IgM mediated

88
Q

Extravascular or intravascular hemolysis?

macrophages digest

89
Q

Extravascular or intravascular hemolysis?

complement lysis

90
Q

Extravascular or intravascular hemolysis?

increased indirect bili

91
Q

Extravascular or intravascular hemolysis?

spherocytosis

92
Q

Extravascular or intravascular hemolysis?

hemoglobinuria and emia

93
Q

Extravascular or intravascular hemolysis?

schistocytes

94
Q

Extravascular or intravascular hemolysis?

Hereditary spherocytosis

95
Q

Extravascular or intravascular hemolysis?

G6PD, HUS, Kassabach-Merritt

96
Q

Diamond blackfan anemia or Fanconi anemia?

infants

97
Q

Diamond blackfan anemia or Fanconi anemia?

Children

98
Q

Diamond blackfan anemia or Fanconi anemia?

short stature, tri-phalangeal thumbs, renal cardiac

99
Q

Diamond blackfan anemia or Fanconi anemia?

short stature, upper limb abnormality, pigmentation changes

100
Q

Diamond blackfan anemia or Fanconi anemia?

Increased HbF

101
Q

Diamond blackfan anemia or Fanconi anemia?

rarely see leukemia

102
Q

Diamond blackfan anemia or Fanconi anemia?

SAA, leukemia

103
Q

Diamond blackfan anemia or Fanconi anemia?

Steroid and BMT

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