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WorksheetsHiggins sickle cell disease pt 1
Total questions: 51
Worksheet time: 29mins
Occurs when valine is substituted for glutamic acid on the 6th codon of the beta globin gene
sickel cell disease
anemia
In sickle cell disease, the polymerization of the hemoglobin (Hgb) S molecule causes chronic hemolysis and vaso-occlusion. Can also cause:
hypoxia
pyrexia
dehydration
acidosis
hypocalcemia
How is the hemoglobin g/dL (Hgb) in Sickle cell disease SCD-SS?
6-9
9-14
7-9
9-12
How is the hemoglobin g/dL (Hgb) in Sickle cell disease SCD-SC?
6-9
9-14
7-9
9-12
How is the hemoglobin g/dL (Hgb) in Sickle cell disease SCD-B-zero thalassemia (SΒ0)?
6-9
9-14
7-9
9-12
How is the hemoglobin g/dL (Hgb) in Sickle cell disease SCD-B-plus thalassemia (SΒ+)?
6-9
9-14
7-9
9-12
What is the reticulocytes % in SCD-SS?
10-20
3-10
15-20
2-5
What is the reticulocytes % in SCD-SC?
10-20
3-10
15-20
2-5
What is the reticulocytes % in SCD-B-zero (SB0) thalassemia?
10-15
3-10
10-20
2-5
What is the reticulocytes % in SCD-B-plus (SB+) thalassemia?
10-20
3-10
10-15
2-5
How is the MCV during SCD-SS?
normal
low normal to slightly low
low
high
How is the MCV during SCD-SC?
normal
low normal to slightly low
low
high
How is the MCV during SCD-B-zero (SB0) thalassemia?
normal
low normal to slightly low
low
high
How is the MCV during SCD-B-plus (SB+) thalassemia?
normal
low normal to slightly low
high
low
The sickle cell trait (SCT) is an heterozygous inheritance of one normal β-globin gene producing hemoglobin A (HbA) and one sickle gene producing hemoglobin S (HbS)
true
false
What is the genotype of a sickle cell trait (SCT)?
HbAS
HbPO
HbOM
In sickle cell disease, patients will have a lot of symptoms
true
false
Distribution of sickle cell trait (SCT) reflects survival advantage in regions where malaria is endemic
true
false
RBCs carrying abnormal sickle Hgb prevent normal growth and development of Plasmodium falciparum
true
false
Individuals without sickle cell trait (SCT) are more likely to survive acute malarial illness
true
false
Normal RBCs have a _________ shape.
biconcave
spherical
elongated
oval
Like normal RBCs, Sickle cell disease RBCs are able to deform to squeeze through capillaries
true
false
Normal adult RBCs contain predominantly HbA, what would be the percentage for it?
96-98%
90-95%
92-96%
Which of the following are considered as other forms of hemoglobin?
HbA2
Fetal hemoglobin
HbA4
HbA2 is another form of hemoglobin, what would be the percentage for it?
96-98%
2-3%
<1%
Fetal hemoglobin is another form of hemoglobin, what would be the percentage for it?
96-98%
2-3%
<1%
Fetal hemoglobin (HbF):
Present predominantly in fetal RBCs
Present predominantly in fetal WBCs
Tetramer of two α-globin chains and two γ-globin chains (α2γ2)
Tetramer of two α-globin chains and two B-globin chains (α2B2)
What happens to fetal hemoglobin around the 32-weeks of gestation?
Switch from production of γ chains to β chains
Increase in HbA production
decrease in HbA production
Switch from production of α chains to β chains
In fetal hemoglobin, an increase in HbF production occurs under severe erythroid stress (e.g. anemia, chemotherapy, etc.)
true
false
The presence of fetal hemoglobin (HbF) do not prevent sickling
true
false
Incidence invasive pneumococcal disease (IPD) was 6 episodes/100 patient years before prophylaxis
true
false
Which of the following statements belongs to the prophylactic penicillin study (PROPS) in 1986?
84% decrease in risk of IPD
Penicillin VK 125 mg BID less than 3 years
Penicillin VK 250 mg BID greater than/equal to 3 years
Prophylactic penicillin could be discontinued at 5 years of age safely unless
splenectomy or invasive pneumococcal infection then continue for life
Pneumococcal conjugate vaccine (PCV) has led to a further 70% decrease in the incidence of IPD (0.3-0.5 episodes/100 patient years)
Which of the following statements belongs to the prophylactic penicillin study (PROPS) II?
84% decrease in risk of IPD
Penicillin VK 125 mg BID less than 3 years
Penicillin VK 250 mg BID greater than/equal to 3 years
Prophylactic penicillin could be discontinued at 5 years of age safely unless
splenectomy or invasive pneumococcal infection then continue for life
Pneumococcal conjugate vaccine (PCV) has led to a further 70% decrease in the incidence of IPD (0.3-0.5 episodes/100 patient years)
Which of the following statements belongs to the Pneumococcal vaccine?
84% decrease in risk of IPD
Penicillin VK 125 mg BID less than 3 years
Penicillin VK 250 mg BID greater than/equal to 3 years
Prophylactic penicillin could be discontinued at 5 years of age safely unless
splenectomy or invasive pneumococcal infection then continue for life
Pneumococcal conjugate vaccine (PCV) has led to a further 70% decrease in the incidence of IPD (0.3-0.5 episodes/100 patient years)
Pneumococcal conjugate vaccine (PCV) has led to a further ____ decrease in the incidence of IPD (0.3-0.5 episodes/100 patient years)
50%
70%
80%
90%
Which of the following statements has to do with the pneumococcal vaccine?
All infants should receive complete
series of PCV13
All children should receive PPSV23 at age 2 and 5 years
Adults vaccination status depends on if they have received PCV13 or PPSV23
Usual childhood vaccine schedule
Annually for 6 months and older
Which of the following immunizations are recommended to prevent sickle cell disease?
Pneumococcal vaccine
Haemophilus influenza (HiB) vaccine
Meningococcal vaccine
Influenza vaccine
Which of the following statements has to do with the Haemophilus influenza (HiB) vaccine?
All infants should receive complete
series of PCV13
All children should receive PPSV23 at age 2 and 5 years
Adults vaccination status depends on if they have received PCV13 or PPSV23
Usual childhood vaccine schedule
Annually for 6 months and older
Which of the following statements has to do with the Meningococcal vaccine?
Four dose primary series with MenACWY-
CRM (Menveo®) (2, 4, 6, 12 months)
Booster dose to be administered every 5 years with MenACWY-CRM or MenACWY-D (Menactra®)
Adults previously vaccinated should receive MenACWY-CRM or MenACWY-D every 5 years
Serogroup B meningococcal (MenB): age 10 or older (Use either Trumemba or Bexcero)
Annually for 6 months and older
Which of the following statements has to do with the Influenza vaccine?
Four dose primary series with MenACWY-
CRM (Menveo®) (2, 4, 6, 12 months)
Booster dose to be administered every 5 years with MenACWY-CRM or MenACWY-D (Menactra®)
Adults previously vaccinated should receive MenACWY-CRM or MenACWY-D every 5 years
Serogroup B meningococcal (MenB): age 10 or older (Use either Trumemba or Bexcero)
Annually for 6 months and older
Chemotherapeutic agent stimulates HbF production and increases the number of HbF- containing reticulocytes and intracellular HbF
hydroxyurea
vaso-occlusive crisis (VOC)
antiemetic
Baby HUG study found children 9 months and older treated with hydroxyurea to have fewer episodes of pain with no significant toxicities
true
false
Recommended for all adults and to offer to children 9 months and older with HbSS or HbSβ0-thalassemia
hydroxyurea
vaso-occlusive crisis (VOC)
antiemetic
The baby HUG study with hydroxyurea showed a reduce in:
vaso-occlusive crisis
acute chest syndrome
hospitalizations
transfusions
operations
What is the initial dose of hydroxyurea in adults?
15 mg/kg PO daily
20 mg/kg PO daily
What is the initial dose of hydroxyurea in children?
15 mg/kg PO daily
20 mg/kg PO daily
What can be done or what should be monitored when taking Hydroxyurea?
CBC
pregnancy test (as needed)
LFTs
HbF
glucose
When taking Hydroxyurea, a CBC should be done:
every 4 weeks until has tolerated dose then every 8 weeks
every 3 months x 2 then every 6 months
every 3-6 months
as needed
When taking Hydroxyurea, HbF should be monitored:
every 4 weeks until has tolerated dose then every 8 weeks
every 3 months x 2 then every 6 months
every 3-6 months
as needed
When taking Hydroxyurea, LFTs should be monitored:
every 4 weeks until has tolerated dose then every 8 weeks
every 3 months x 2 then every 6 months
every 3-6 months
as needed
When taking Hydroxyurea, a pregnancy test should be done or monitored:
every 4 weeks until has tolerated dose then every 8 weeks
every 3 months x 2 then every 6 months
every 3-6 months
as needed
