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Neonatal Board Questions > Heme/Onc > Flashcards

Heme/Onc Flashcards

1
Q

Primary site of hematopoiesis in fetus (in order)

A

Secondary yolk sac (until ~10 weeks)
Liver (6-22 weeks)
Bone marrow (after 22 weeks)

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2
Q

Predominant cell type in fetal liver

A

Mature macrophage

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3
Q

HgF made of which global chains

A

Alpha 2 gamma 2

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4
Q

HgA made of which global chains

A

Alpha 2 Beta 2

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5
Q

Which thalassemia detected at birth

A 
Alpha thalassemia 
(HgF has alpha chains but no beta chains so don't notice beta thal until ~6 months when HgA predominant)
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6
Q

Absence of 1 alpha gene

A

Silent carrier

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7
Q

Absence of 2 alpha genes

A

Alpha thal trait = mild microcytosis, hypochromia, may be asymptomatic

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8
Q

Absence of 3 alpha genes

A

Hb H

moderately severe hemolytic anemia, Heinz bodies

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9
Q

Absence of 4 alpha genes

A

Hemoglobin barts

hydrops fetalis

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10
Q

Frontal bossing, maxillary hypertrophy, severe anemia

A

Beta thalassemia disease (homozygous) or Cooley anemia

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11
Q

Most common hemoglobinopathy in the world

A

Hemoglobin E

Decreased production of beta chains, resulting in chronic, mild, microcytic anemia

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12
Q

FA vs FAS vs FSA

A

FA normal
FAS sickle cell trait
FSA sickle B+ thalassemia

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13
Q

FS

A

Sickle cell anemia, sickle beta 0 thalassemia, sickle with hereditary persistence of hemoglobin

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14
Q

Kleihauer-Betke vs Apt test

A

KB on maternal blood, detects fetal blood

Apt test on fetal sample, detects maternal blood

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15
Q

Triphalangeal thumbs + macrocytic anemia

A

Diamond Blackfan and Fanconi anemia

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16
Q

Radial hypoplasia

A

Fanconi anemia and TAR

in TAR, thumbs and digits are developed normally

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17
Q

Hypersegmented neutrophils

A

Vitamin B12 and folate deficiency

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18
Q

Most frequently inherited enzyme defect

A

G6PD deficiency

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19
Q

G6PD catalyzes which process?

A

G6P to pentose phosphate and makes NADPH

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20
Q

Pyruvate Kinase catalyzes which process?

A

PEP to pyruvate and makes ATP

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21
Q

Normal paO2 but decreased oxygen saturation

A

Methemoglobinemia

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22
Q

Maternal platelet count in NAIT vs Neonatal autoimmune thrombocytopenia

A

Normal vs low

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23
Q

Bleeding with prolonged PTT

A

Hemophilia A or B

A in 70% cases

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24
Q

Bleeding with normal coagulation studies

A

Factor XIII (Autosomal recessive)

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25
Q

Bleeding with abnormal BT +/- prolonged PTT

A

vWF disease (AR or AD)

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26
Q

Bleeding with prolonged PT and normal PTT

A

Vitamin K deficiency

if extended time, PTT will also be prolonged

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27
Q

MC solid tumor in the neonatal period

A

Teratoma

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28
Q

What do ceftriaxone, sulfonamides and indomethacin do to bilirubin?

A

Bind albumin and displace bilirubin, increasing indirect hyperbilirubinemia

29
Q

Hemangiomas + thrombocytopenia

A

Kasabach Merritt syndrome
(Coagulation activated locally within the hemangioma and platelets are sequestered)
Only 50% infants have visible hemangioma

30
Q

chromosomal fragility and breakage

A

Fanconi’s anemia

31
Q

First line treatment for NAIT

A

random donor platelets
IF ineffective, HPA-1a negative donor
IVIG and steroids may help reduce immune mediated destruction

32
Q

Early onset hemorrhagic disease of the newborn

A

Within 24 hours
Placental transferred drugs that inhibit vit K ie anticonvulsants, cephalosporins, warfarin, barbiturates, rifampin, isoniazid

33
Q

Classic hemorrhagic disease of the newborn

A

2-7 days

34
Q

Late onset hemorrhagic disease of the newborn

A

2 weeks to 6 months
MC in boys, summer
Poor enteral intake of Vitamin K or liver disease

35
Q

Leukocytosis with neutrophilic, thrombocytopenia and blasts

A

Transient myeloproliferative disease

5-10% infants with T21

36
Q

Typical symptoms of infants with Transient myeloproliferative disease

A

Typically asymptomatic

can have HSM, effusions, bleeding or petechiae

37
Q

Prognosis of Transient myeloproliferative disease

A

60-70% cases resolve spontaneously by 2 months

20% affected infants will develop AML

38
Q

NAIT vs Rh disease, which can occur in 1st pregnancy?

A

NAIT

Recurrence rate >75% in subsequent pregnancies

39
Q

Where is EPO made in the fetus?

A

Fetal liver (primarily) and kidney

40
Q

When does most Fe transfer to the fetus occur?

A

After 30 weeks (accrued at 1.6-2 mg/kg/d)

41
Q

Hypopigmented and hyperpigmented skin lesions, photosensitive malar rash, mild craniofacial dysmorphisms, high pitched voice

A

Bloom’s syndrome

42
Q

Size and amount of megakaryocytes in adult vs fetus

A

Fetus - smaller but greater number circulating (still only 0.03-0.1% of nucleated cells in the BM)

43
Q

<12 months + neuroblastoma = favorable or unfavorable prognosis?

A

Favorable

44
Q

MC renal malignancy of children

A

Wilm’s tumor

45
Q

Wilm’s tumor associated with which syndromes

A

BW syndrome
Denys drash (progressive renal disease, male pseudohermaphroditism)
Pearlman (fetal gigantism, visceromegaly, abnormal facies, b/l renal hamartomas)
WAGR (Wilm’s, aniridia, genitorurinary, mental retardation)

46
Q

Heme oxygenase catalyzes which step

A

Heme to biliverdin

47
Q

Biliverdin reductase catalyzes which step

A

Biliverdin to bilirubin

48
Q

Can placenta remove indirect bilirubin? Biliverdin?

A

Bilirubin but not biliverdin

49
Q

MC site of bleeding in hemorrhagic disease of the newborn

A

GI tract

50
Q

Why is irradiated blood used?

A

Prevents GVHD

51
Q

How to decrease risk of CMV in blood?

A

Leucodepleted

52
Q

What shifts oxyhemoglobin curve to right and does that increase or decrease affinity?

A

Inc temperature, acidosis (dec pH), altitude, 2,3-DPG, Bohr effect
Inc offloading ie decreases affinity

53
Q

Rh blood system consists of

A

C, c, D, E, e, G antigens

54
Q

Albumin binding to bilirubin decreased by

A

Sepsis, acidosis, hypoxia, free fatty acids, albumin-binding drugs ie. Ceftriaxone
albumin = 2.5 is a risk factor

55
Q

Irreversible sign of kernicterus

A

Opisthonus

56
Q

Most severe of minor antigens

A

Kell

57
Q

Nitrites, aniline dyes, prilocaine, nitrous oxide

A

Acquired methoemloglobinemia

58
Q

Congenital methemoglobinemia tx

A

do NOT respond to methylene blue

59
Q

Family history of parent with intermittent cyanosis

A

Congenital methemoglobinemia (AD)

60
Q

Pure red cell aplasia

A

Diamond Blackfan Anemia (macrocytic)

61
Q

Total blood volume to be exchanged in polycythemia

A

([hematocrit observed - desired) x blood volume] / hematocrit observed

62
Q

Bleeding disorder ass with Noonan

A

Hemophilia C

63
Q

Purpura fulminans

A

Protein C or S deficiency

64
Q

Term infant with IVH, look for?

A

Cerebral sinovenous thromboses –> get MRI

65
Q

When use TPA

A

limited to life or limb threatening thromboses

66
Q

Which criggler najjar responds to phenobarbital?

A

Type II

67
Q

What to do when infant showing advanced stages of acute bilirubin encephalopathy

A

EXCHANGE- DVET (80-100 mL/kg/day x 2)

68
Q

Firm subcutaneous nodules bluish gray

A

leukemia cutis –> neonatal leukemia

Neonatal Board Questions (12 decks)

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