Hematologic

Question 1

the bone marrow of a healthy fetus produces _________ of cells every day

Answer 1

Billions

Question 2

What is the challenge of the bone marrow in a fetus vs. an adult?

Answer 2

The marrow must produce enough cells to maintain a stable cell number per body mass as the infant grows

Question 3

Preterm birth is a/w additional hematologic stressors which can result in _______ or __________

Answer 3

Anemia

Neutropenia

Question 4

What is Erythropoetin?

Answer 4

Glycoprotein that regulates erythrocyte production (RBC’s).

Question 5

What is the pathway of erythropoetin synthesis in the fetus/NB?

Answer 5

Yolk sac–>Liver–>Kidneys

Question 6

Where is Epo production much of fetal life?

Answer 6

Liver

Question 7

When does Epo production change to the kidneys?

Answer 7

Around time of birth

Question 8

Epo maintains RBC production by inhibiting ______ of erythroid progenitors and stimulating their _________ & ________ into normoblasts

Answer 8

Apoptosis

Proliferation & Differentiation

Question 9

Does EPO cross the placenta?

Answer 9

No, Fetus produces their own

Question 10

To maintain the increase in RBC volume a/w fetal growth, it is estimated ______ erythrocytes/day must be produced

Answer 10

50 x 10 to the 9th power

Question 11

Erythropoetin production is stimulated by ________ induceable Factor __ & __, and regulated by ______ requirements for oxygenation

Answer 11

Hypoxia
Factor 1 & 2
Tissue requirements

Question 12

In healthy, term infants, serum erythropoetin concentrations reach Nadir between ___-___wks.

By ___-___ wks, they reach adult concentrations.

Answer 12

4-6 wks

10-12 wks

Question 13

In preterm infants, the fallen erythropoetin is more _______ and lasts ______—>what?

Answer 13

profound
longer
Anemia of prematurity

Question 14

What does rEPO stand for?

Answer 14

Recombinant EPO (the med)

Question 15

What medication is widely used to treat or prevent Anemia due to a variety of causes including Renal failure and prematurity?

Answer 15

rEPO

Question 16

Name the 4 clinical trials w/EPO currently under way

Answer 16

1. Anemia of prematurity
2. Anemia from Rh-hemolytic Dz
3. Anemia of BPD
4. CHD

Question 17

Name the other medication that is an erytrhopoesis stimulating protein.
Advantages?
Disadvantages?

Answer 17

• Aranesp
• Single 1x/wk injection
• Well-studied (in adults)
• Very few studies in preterm or term babies re: Pharmacokinetics, efficacy, risks/benefits of use

Question 18

What other med must be given w/EPO?

Answer 18

Iron

Question 19

Neonates require ______ dosing of rEPO/kg and more _______ dosing to achieve an equivalent hematopoetic repsonse r/t…?

Answer 19

Higher
Frequent
Increased plasma clearance, high vol. of distribution, short fractional elimination time

Question 20

Garcia et. al showed that for doses at _____ u/kg/wk rEPO, the average # tranfusions per pt decreased by 3/4 of a transfusion.

Answer 20

500 u/kg/wk

Question 21

What lab level may be helpful in assessing Fe stores?

Answer 21

Ferritin

Question 22

What are the adverse effects of rEPO in adults?

Answer 22

Hypertension, thrombus formation, polycythemia, red cell aplasia

Question 23

Early, high-dose rEPO may have a _______ effect on the retina by ameliorating the first stage of ROP .

Answer 23

Protective effect

–but one study suggested an increased risk of ROP in pts started on early high-dose rEPO and Iron.

Question 24

In 2008, the FDA put out a warning for pts getting rEPO at higher than recommended doses w/what Dz?
What were the effects?

Answer 24

Chronic Kidney Failure

Increased risk blood clots, stroke, death

Question 25

In preterm infants, rEPO for treatment of Anemia has been very ____ with ____ adverse side effects of adults.

Answer 25

Safe | none

Question 26

What is a potential side effect of EPO?

Answer 26

an effect on Iron balance

Question 27

Preterm babies are at risk for Iron deficiency r/t ...?

Answer 27

Bulk of Iron transfer is during 3rd trimester

Question 28

Iron is required for normal ______ & _______

Answer 28

growth and development

Question 29

Iron deficiency can lead to?

Answer 29

Adverse Neurodevelopmental consequences: Deficits in executive function & memory

Question 30

T/F: Administration of Iron in the 1st 2 weeks of life is NOT advised d/t potential oxidative effects in the face of deficient anti-oxidant mechanisms.

Answer 30

True

Question 31

The actual side-effects of rEPO are what we are __________ to see-including:

Answer 31

Hoping Increasing Erythropoesis Possible Megakaryocytopoesis

Question 32

What is a nutritional source of highly bio-available Iron?

Answer 32

Breastmilk

Question 33

Iron is a required mineral in transfer of _____from the lungs to the tissues for storage for use during muscle contraction.

Answer 33

Oxygen

Question 34

Iron contained in cow's milk is less well ___________.

Answer 34

Absorbed-a reason it is not recommended until 1 yr/life

Question 35

Iron is found as _____ iron in animal-based foods (meats). This is the most ____-______ form vs. plant-based (grains, fruits, vegetables)

Answer 35

Heme iron Bio-available

Question 36

Preterm formulas only provide ___ mg/kg/day iron. Supplemental iron is required

Answer 36

1.8 mg/kg/kday

Question 37

____ overdose is the most common cause of poisoning death in children

Answer 37

Iron | 6 y/o

Question 38

Endogenous Iron stores are used up by ___ months of age.

Answer 38

6 months

Question 39

Preterm, LBW, & breastfeeding: Iron supplement via gtts at ____ mg/kg/day. Start __-__ wks/age until 12 months.

Answer 39

2 mg/kg/day 2-4 wks/age

Question 40

Healthy, term breastfeeding babys: supplement w/___ mg/kg/day iron. Starting at __-__ months add iron from complimentary foods.

Answer 40

1 mg/kg/day 4-6 months

Question 41

Infants < 12 months should be given only ___ fortified formulas.

Answer 41

Iron-fortified

Question 42

What other possible effects might EPO have?

Answer 42

Neuroprotection | Protection of other organs: Heart, Kidneys

Question 43

Fer-in-sol supplement provides more/less iron per dose than multi-vitamin product?

Answer 43

More~25 mg/mL vs. 10 mg/mL

Question 44

Studies show children Anemic in early childhood continue to have _____ ________ & motor development and depressed school achievement into middle childhood.

Answer 44

Poor Cognitive

Question 45

What does G-CSF stand for?

Answer 45

Granulocyte Colony Stimulating Factor

Question 46

G-CSF is a physiologic _______ of ______production and function.

Answer 46

Regulator of Neutrophil

Question 47

G-CSF has multiple effects on what type of cell's maturation and function?

Answer 47

WBC

Question 48

How does G-CSF enhance Neutrophil functions?

Answer 48

Chemotaxis Phagocytosis Superoxide Production Bacteriocidal activity

Question 49

Administration of G-CSF has long been used to prevent _______ in pts w/non-myeloid malignancies receiving Anti-CA drugs and suffering Febrile Neutropenia. It is also used to supplement _________after bone marrow transplant.

Answer 49

Infection Recovery

Question 50

Name the 5 G-CSF clinical trials in process

Answer 50

1. Infants w/bacterial sepsis 2. Infants whose mothers had PIH 3. Alloimmune Neutropenia 4. Autoimmune Neutropenia 5. Chronic Idiopathic Neutropenia

Question 51

What are the 3 adverse effects (rare) of G-CSF?

Answer 51

1. Thrombocytopenia 2. Osteoporosis 3. Contrandicated w/known hpersensitivity to e-coli derived proteins or any components of the product.

Question 52

What is rGM-CSF?

Answer 52

Recombinant Granulocyte Macrophage Colony Stimulating Factor

Question 53

What does rGM-CSF do?

Answer 53

Increases Neutrophil counts and may decrease mortality due to sepsis when used prophylactically. --Significant increase in ANC w/in 48 hrs of administration.

Question 54

Name the 2 clinical trials w/rGM-CSF?

Answer 54

1. Neonatal sepsis | 2. Prophylaxis against Nosocomial Infections

Question 55

What adverse effects are there w/rGM-CSF? | What is needed?

Answer 55

No serious adverse effects reported Long-term studies needed.