Pediatric Anemia

Question 1

Which hemoglobin dominates At newborn, 6months, and adult for a normal child?
For a sickle cell child?

Answer 1

Normal:
newborn: HbF
6 months: HbA
Adult: HbA

Sickle:
Newborn: HbS
9 months: HbA

Question 2

Differentiate between the structures of HbF and HbA

Answer 2

HbF (alpha, gamma)
HbA (alpha, beta)

Question 3

The smaller or premature the child how does it affect their hemoglobin and MCV?

Answer 3

Lower

Question 4

When is IDA common for under 6 months?

Answer 4

If mother had low iron stores (ferritin)

Question 5

What medications cause anemia?

Answer 5

Anti-folates
- phenytoin
- alcohol

Oxidant stress
- nitrofurantoin

Immune hemolysis
- penicillin

Question 6

What are the 3 most important tests to order to identify anemia

Answer 6

1. CBC - Hb, MCV, RBC, WBC, platelets
2. Reticulocyte count - bone marrow response
3. Peripheral blood - morphological clues

Question 7

What does an increase/normal or decrease reticulocyte count indicate?

Answer 7

Normal or decrease:
- check MCV

Increased
- blood loss
- hemolysis

Question 8

What is important based on reticulocyte count?

Answer 8

The ADEQUATE RETIC RESPONSE not the absolute retic count.

Question 9

What are causes of IDA

Answer 9

1. Inadequate iron stores at birth
2. Inadequate diet (cow milk)
3. Blood loss (GI tract)
4. Malabsorption of iron (meds)

Question 10

What is the dosing of elemental iron for pediatrics

Answer 10

Give 3-6mg/kg per day

Question 11

How do you rule out malabsorption of iron

Answer 11

Perform an oral iron challebnge
- Give 1mg/kg/dose of oral iron
- Fast before and 2 hours after
- Take serum iron tests
- Test should increase 20+ mmol/L

Question 12

What are the steps for evaluating childhood anemia? When do you refer?

Answer 12

1. Review patient history + physical examination
2. Order lab tests: CBC, reticulocyte count, peripheral blood smear
3. Refer if
   - Poor response to oral iron
   - Anemia with additional cytopenia
   - Evidence of acute/chronic hemolysis
   - Unexplained normocytic anemia

Question 13

Explain the conditions associated with quantitative and qualitative hemoglobin

Answer 13

Quantitative: reduced globin snythesis
- thalassemia

Qualititative: structural hemoglobin difference
- sickle cell
- Methaemoglobinemia
- G6PD

Question 14

What is the mutation in sickle cell

Answer 14

B globin (HBB)
- glutamic acid replaced by valine in amino acid ^

Question 15

What is the risk factor for sickle cel

Answer 15

Ancestry from
- africa
- south america

Question 16

What are causes of sickle cell (3)

Answer 16

1. Vaso-occlusion: RBC gets trapped in blood vessel
2. Intravascular hemolysis: promotes inflammation and leads to vasculopathy
3. Chronic hemolysis/anemia
   - inc LDH + bilirubin
   - dec Hgb

Question 17

What is the presentation and treatment for sickle cell crisis?

Answer 17

Presentation
- chest pain
- dyspnea
- low PO2

Treatment
- analgesics: opioids, ketamine, NSAIDs
- bronchodilators O2
- Hydration
- Transfusions

Question 18

What are risk factors for sickle cell crisis

Answer 18

• dehydration
• infections

Question 19

What are treatment options for SCD. When is it safe and effective (age)?

Answer 19

• Hydroxyurea; over 9+ months
• Blood transfusions

Question 20

What is the mechanism for hydroxyurea?

Answer 20

Induces production of fetal hemoglobin (primary)

Question 21

What is the dosing for hydroxyurea for children/adults

Answer 21

Start at 20 mg/kg can go up to 25mg/kg

Adult
1000mg daily and inc as tolerated

Question 22

Which lab values will increase and decrease in response to hydroxyurea

Answer 22

Increase
- HbF
- MCV
- Hgb

Decrease
- Platelet
- neutrophil
- Retic

Question 23

What are some options for supportive care for sickle cell

Answer 23

Penicillin
Splenectomy
Folic acid

Question 24

What can help differentiate between IDA and thalassemia

Answer 24

Mentzer index is divided by RBC

Thalassemia: less than 13
- High HbA2
- rest is normal

IDA: 13+

Question 25

What is the treatment iron overload? Preferred?

Answer 25

Preferred: Deferasirox or DFX (jadenu)
- 1D dosing

Other options
- DFO deferoxamine
- DFP deferiprone