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Peds > hematology (2) > Flashcards

hematology (2) Flashcards

1
Q

what does blood arise from

A

bone marrow

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2
Q

what system regulates (direct or indirect) all other body functions

A

hematologic

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3
Q

what are the 2 major differences of bones in pediatrics compared to adults?

A
  • all bones have red bone marrow
  • all bones are capable of hematopoiesis
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4
Q

at what age range does bone growth cease?

A

adolescents

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5
Q

which bones contain red marrow (produce blood cells) in adolescents

A

flat bones (sternum, vertebrae, pelvis)

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6
Q

describe RBCs, WBCs, and platelet levels in neonate vs. older

A

RBCs and WBCs are higher in infancy, platelets are lower (all clotting factors are lower)

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7
Q

what is the most common anemia and nutritional deficiency

A

iron deficiency (anemia)

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8
Q

when are children screened for iron deficiency anemia?

A

9-12 mo

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9
Q

Case study:
3 y.o. is pale, lethargic, irritable. Always seems tired, can’t concentrate, ice craving. Assessment revelas 145 HR (tachy)

A

iron deficiency anemia

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10
Q

s/sx of iron deficiency anemia

A

pale, lethargy, irritable, tired, dec. LOC, dec. concentration, ice craving

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11
Q

what tests would be ordered for possible iron deficiency anemia?

A

H&H, CBC

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12
Q

what would a CBC show with iron deficiency anemia?

A
  • inc. RBCs
  • mean corpuscualr volume (MCV)
  • mena corpuscular hemoglobin (MCH)
  • inc. reticulocytes
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13
Q

what do cells with anemia look like under observation

A

pale, “bleached out”

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14
Q

what are the hct and hgb criteria to supplement iron in the diet

A

hct= <34%
hgb= < 11.3 g/dl

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15
Q

what is the dose of ferrous sulfate used to orally supplement?

A

3-6 mg/kg/day

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16
Q

what are the 3 causes of iron deficiency anemia?

A

inadequate diet intake
malabsorption in GI
chronic blood loss

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17
Q

what can lead to iron defiency anemia from chronic blood loss?

A
  • cows milk protein allergy
  • crohns
  • celiac disease
  • inc. physiologic demand (growth)
  • menorrhage (period)
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18
Q

what should iron be given on (stomach, fluid)

A

empty stomach with OJ (any juice w/ vit. c)

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19
Q

what is an inherited autosomal recessive genetic disease that affects RBCs

A

sickle cell

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20
Q

in sickle cell, what is hemoglobin replaced or partially replaced with?

A

hemoglobin S

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21
Q

are cells chronicly or acutely sickle shaoed with the disease?

A

acute (crisis)

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22
Q

why do sickled cells occlude vessels

A

“sticky”

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23
Q

what is the first organ affected by sickle cell?

A

spleen

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24
Q

which races are affected by sickle cell

A

african, hispanic, middle eastern, southern european, asian indian

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25
Q

wha ttriggers cause sickling of cells

A
  • infection/fever
  • dehydrtion
  • cold/high altitude
  • overexertion
  • alcohol
  • stress, fatigue
    (amnything that dec. oxygen)
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26
Q

to get sickle cell, the child must receive the gene from how many parents?

A

both

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27
Q

if both parents have the sickle cell gene, what are the chances of having, carrying, and not being effected?

A

25% having
50% carrying
25% not effected

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28
Q

what is the treatment of vaso-occlusive sickle cell crisis

A
  • hydration (IV)
  • aggressive pain management
  • oxygen
  • bed rest
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29
Q

how does hydration help sickle cell crisis

A

reduces dehydration (trigger)
decreases blood viscosity

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30
Q

is pain management suring a sickle cell crisis scheduled or PRN

A

scheduled

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31
Q

which immunizations can help sickle cell

A
  • pneumococcal
  • Hib
  • hep b
  • influenza
  • 23-valent vaccine @ 2 and 5 yrs. after forst dose
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32
Q

what is the prophylactic antibiotic for sickle cell

A

low doses of penicillin

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33
Q

what ages is prophylactic penicillin used in sickle cell?

A

newborn - 5 y.o. (unless they ahd splenectomy)

34
Q

what is the prophylactic dose of penicillin for sickle cell for ages newborn-3 y.o.

A

125 mg 2x/day

35
Q

what is the prophylactic dose of penicillin for sickle cell for ages greater than 3 y.o.

A

250 mg 2x/day

36
Q

which testing is done annualy for sickle cell

A

transcranial doppler testing

37
Q

what haooens if transcranial doppler testing for sickle cell comes back abnormal?

A

prophylactic blood transfusion

38
Q

which treatment of sickle cell improves blood and tissue oxygenation, reduces sickling, temporary suppression of RBCs with hgb S

A

transfusion

39
Q

complication of blood transfusion for sickle cella and how to help

A

iron overload, use iron-chelating drugs

40
Q

examples of iron chelating drugs

A

deferoxamine, deferasirox, with vitamin c

41
Q

which sickle cell treatment is a cytotoxic drug that decreases production of abnormal blood cells (less pain)

A

hydroxyures

42
Q

what is needed for a hematopoietic stem cell transplant for sickle cell?

A

human leukocyte antigen (HLA) compatible sibling

43
Q

group of hereditary blleding disorders resulting from deficiency in specific clotting factors

A

hemophilia

44
Q

what are the types of hemophilia

A

A,B, and C

45
Q

which hemophilia is classic hemophilia

A

hemophilia A

46
Q

which hemophilia is christmas disease

A

B

47
Q

what is the most common hereditary bleeding disorder?

A

von willebrand disease

48
Q

which hemophilia is caused by a deficiency of factor VIII (8)

A

hemophilia A

49
Q

what gender does hemophilia A effect?

A

Males, X linked recessive

50
Q

why does hemophilia A only happen in men?

A

x-linked recessive trait

51
Q

which hemophilia is a deficiency of factor IX (9)

A

hemophilia B

52
Q

what does the severity of hemophilia B range from?

A

mild to severe bleeding tendencies

53
Q

what kind of genetic trait is hemophilia b

A

x-linked recessive trait (mostly male)

54
Q

which population is most effected by hemophilia b

A

amish

55
Q

which hemophilia is a deficiency of factor XI (11)?

A

hemophilia c

56
Q

what kind of genetic trait is hemophilia c

A

autosomal recessive

57
Q

what gender does hemophilia c primarily effect?

A

equally in males and females

58
Q

how is bleeding in hemophilia c compoared to a and b

A

less bleeding in C than A and B

59
Q

what is vWF (von willebrand factor)

A

plasma protein (usually increases in injury)

60
Q

which blood disorder is poor platelet aggregation

A

von willebrand disease

61
Q

what kind of genetic trait is von willebrand disease

A

autosomal dominant

62
Q

what gender does von willebrand disease primarily effect?

A

M and F equally

63
Q

what is von willebrand disease treated with?

A

DDAVP

64
Q

what kind of cases is von willebrand disease usually seen in?

A

mouth injury, bloody nose, excessive menstrual bleeding

65
Q

which hemophilias are sex-linked recessive clotting disorder

A

A and B

66
Q

hemophilia A and B is carried by the _____ and passed to the ____

A

mother, sons

67
Q

what are complications from hemophilia?

4 of them

A
  • internal hemorrhage
  • transfusion reaction
  • hypovolemic shock
  • death
68
Q

what can not be given for pain in hemophilia?

A

aspirin/ibuprofen (thins blood)

69
Q

what diagnostic tests are used to look for hemophilia?

3 of them

A
  • chorionic villus sampling
  • amniocentesis
  • genetic testing (carriers)
70
Q

what are the 3 treatments for hemophilia

A
  • replacement of missing factor
  • home infusions
  • gene therapy (exploring it)
71
Q

when is replacement of factors especially indicated in hemophilia?

A

when a child experiences mild to severe hemorrhage

72
Q

why would factor replacement be scheduled for a hemophilia patient?

A

episodes can be controlled or avoided (earlier started, better outcome)

73
Q

non-pharm method to help acute bleeding

A

RICE
rest, ice, compression, elevate

74
Q

describe injections in hemophilia patients

A
  • give sub cue if possible
  • if not possible to avoid IM, administer replacement factor first
75
Q

what kinds of replacement factors are used for hemophilia a or b

A
  • pooled factor from blood products
  • recombinant factor
  • desmopressin acetate (DDAVP)
76
Q

which replacement factor is made ina lab and does not contain blood?

A

recombinant factor

77
Q

which repalcement factor increases factor VIII but does not effect factor IX

A

desmopressin acetate (DDAVP)

78
Q

what is DDAVP used for

A

mild hemophilia A

79
Q

what are the 3 signs of blood transfusion reaction

A

fever, chills, irritability

80
Q

what fluid is blood given with fro infusions

A

NS, not D5W

81
Q

what to do if child has transfusion reaction

A

turn off, disconnect, flush with NS and heparin (heparin only if central line)

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