Cm Iii-Sickle Cell Disease

Front 1

What is sickle cell disease?

Back 1

homozygous inherited hemoglobinopathy

Front 2

Sickle cells results in what substitution?

Back 2

valine for glutamine

Front 3

What is present in all pts with SCD?

Back 3

-anemia

Front 4

What are some other clinical manifestations of SCD?

Back 4

-dactylitis
-strokes, leg ulcers, splenomegaly, painless hematuria

Front 5

What is the hallmark presentation of SCD?

Back 5

-vaso-occlusiveness

Front 6

Vaso-occlusive type of SCD compromises blood supply, which causes what?

Back 6

-hypoxia, tissue death and localized pain

Front 7

Vaso-occlusive SCD is most commonly effects what tissues?

Back 7

bone, chest and abdomen

Front 8

Vaso-occlusive crisis episodes are precipitated by what?

Back 8

-infection
-dehydration
-stress
-menses
-alcohol
-hypoxemia

Front 9

Acute chest syndrome of SCD is what?

Back 9

-very painful and life threatening

Front 10

Acute chest syndrome of SCD can be caused by what?

Back 10

occlusion, lung infarction, pulmonary fat embolism, bone marrow infarction, or bacterial PNA

Front 11

How does acute chest syndrome of SCD present?

Back 11

-fever
-chest pain
-new pulmonary infiltrate

Front 12

What kind of infarcts are common in SCD pts?

Back 12

-splenic

Front 13

Vaso-occulsion where causes strokes in SCD pts?

Back 13

-in the brain

Front 14

What are some other problems associated with SCD?

Back 14

- leg ulcers and priaprism

Front 15

What organ is the most susceptible to the effects of sickling?

Back 15

kidneys

Front 16

Is renal failure an early or late complication to SCD?

Back 16

-late

Front 17

What causes isothenuria in SCD pts with kidney trouble?

Back 17

recurrent infarcts of the renal medulla

Front 18

Aplastic crisis in SCD is characterized by what and mostly caused by what?

Back 18

-sudden decrease in Hgb and reticulocyte count
-caused by parvovirus infection of bone marrow

Front 19

Infectious crisis in SCD is due to what?

Back 19

underlying functional asplenia lead to infection from encapsulated organisms

Front 20

Infectious crisis in SCD is associated with low serum ______ and impaired what?

Back 20

-low serum IgM
-impaired opsonization and complement activation

Front 21

Dx of Sickle Cell Disease.
1-what type of anemia?
2-what values are elevated?
3-what level are low?
4-what does peripheral smear show?

Back 21

1-mild anemia that is nromochromic and macrocytic with reticulocytosis
2-MCV, indirect bilirubin, and LDH are elevated
3-low haptoglobin
4-sickle cells, Howell-Jolly bodies are seen on peripheral smear

Front 22

In what 3 ways does transfusion therapy lower percent of HbS in sickle cell?

Back 22

1-dilution
2-suppression of EPO production
3-increases lifespan of normal RBC

Front 23

Transfusion therapy for SCD:
1-recommended to do what?
2-restores what?
3-what is recommended to stop iron overload and improve viscosity?

Back 23

1-increase oxygen carrying capacity of blood
2-restores volume in splenic sequestration, acute chest syndrome, and sepsis
3-partial exchange transfusions

Front 24

What is the tx for Acute Chest Syndrome?

Back 24

1-supportive: oxygen, abx, pain control, volume repletion
2-exchange transfusion of PaO2 can’t be maintained

Front 25

What is the tx for acute pain episodes in SCD?

Back 25

-admit to hospital
-aggressive rehydration
-pain control with narcotics
-blood transfusions

Front 26

What are some complications associated with SCD?

Back 26

-narcotic addictions
-iron overload
-INFECTION is MCC of death
-stroke, transfusion reactions, splenic sequestration, thromboembolism, renal failure, pulmonary HTN

Front 27

What screening test is done for SCD?

Back 27

-sickle cell solubility test and it shows Hgb S is relatively insoluble in concentrated phosphate buffers and causes a cloudy solution

Front 28

What is used to dx SCD?

Back 28

-hemoglobin electrophoresis and DNA analysis

Front 29

Hemolytic anemia in SCD is caused by what?

Back 29

removal of sickle cells by macrophages

Front 30

How is hemolytic anemia managed in sickle cell disease?

Back 30

transfusion, folate, and iron chelation if needed

Front 31

How is aplastic crisis treated in sickle cell disease?

Back 31

-transfusions

Front 32

Tell me the clinical features and management of each of the following sites of sickling?
1-bone
2-lung
3-brain
4-heart
5-spleen
6-retina

Back 32

1-painful crisis and tx with pain relief and hydration and hydroxyurea
2-acute chest syndrome and tx with transfusion, pain relief and hydration
3-stoke and tx with transfusion
4-MI and tx with transfusion, pain relief and hydration
5-acute splenic sequestration and hyposplenis tx with transfusion, pain relief, hydration, Pneumovax
6-proliferative retinopathy and tx with retinal surveillance and laser

Front 33

In acute chest syndrome there is a high rate of what?

Back 33

-narcotic addiction