sickle cell anemia

Question 1

sickle cells disease is due to

Answer 1

abnormal b hemoglobin chains

Question 2

sickle cells genetics

Answer 2

sing base substitution of adenine changed to thymine

Question 3

amino acid substation in sickle cell anemia

Answer 3

valine for glutamate

Question 4

sickle cells patients have an increase in what type of Hb

Answer 4

HbF (decrease in HbA)

Question 5

deoxygenated HbS is poorly

Answer 5

soluble

Question 6

two major problems from sickle cell anemia

Answer 6

1. hemolytic anemia
2. vaso-occlusion of small blood vessels

Question 7

overtime sickling leads to

Answer 7

RBC membrane damage

Question 8

RBC membrane damage results in

Answer 8

extravascular hemolysis

Question 9

the increase in EPO in sickle cell disease results in

Answer 9

erythroid hyperplasia

Question 10

erythroid hyperplasia sxs

Answer 10

chipmunk facies
crew cut appearnace
osteoporosis

Question 11

infection with parvovirus B19 in sickle cell patients can result in

Answer 11

sickle cell anemia

Question 12

sickle cells may occlude

Answer 12

microvasculature

Question 13

sxs due to sickle cells occluding microvasculature

Answer 13

swollen hands
acute pain crisis
spleen failure
renal dys

Question 14

pain/swelling in hands or feet is a common sxs in __ with sickle cell disease

Answer 14

children

Question 15

avascular necrosis can be a result of

Answer 15

1. sickle cell anemia
2. long term steroid use

Question 16

__ can start to appear in peripheral blood due to splenic failure

Answer 16

howell jolly bodies

Question 17

splenic failure in sickle cell patients increases the risk of

Answer 17

infection by encapsulated bacteria such as (strep pneumoniae and h flu)

Question 18

in sickle cell anemia patients osteomyelitis can occur due to infection from

Answer 18

salmonella

Question 19

vase-occlusion in the spleen causes

Answer 19

pooling of RBCs = splenic sequestration crisis

Question 20

splenic sequestration crisis results in

Answer 20

fall in Hb level
rapidly enlarging spleen
risk of hypovolemic shock

Question 21

sick cell anemia chest syndrome

Answer 21

vase-occlusion of pulmonary microvasculature (often triggered by infection)

Question 22

sickle cell anemia renal dysfunction is due to

Answer 22

occlusion of vasa recta in renal medulla

Question 23

sickle cell anemia renal dysfunction sxs

Answer 23

may impair concentrating ability

Question 24

sickle cell trait is usually more mild except

Answer 24

renal medulla may loose concentrating ability

Question 25

sickle cell trait is protective against

Answer 25

malaria

Question 26

hemoglobin C

Answer 26

rare mut of beta gene (glutamic acid replaced by lysine)

Question 27

hemoglobin C causes presence of

Answer 27

HbC crystals