اول محاضرة Flashcards
Sickle cell disease (hemoglobin S disease)
is an ?
autosomal recessive disorder
What is the Variables that increase sickling ?
1- Increased pCO2.
2- Decreased pH
3- Dehydration
What is the Thalassemias ?
Synthesis of β-globin chains is decreased or absent.
How many copies in Thalassemias ?
the β-globin
What is the difference between β-thalassemia minor and β-thalassemia major ?
β-thalassemia minor if they have only one
defective β-globin gene
β-thalassemia major if both genes are
defective.
Qualitative hemoglobinopathy occurs in ?
1- sickle cell disease
2- hemoglobin C
3- SC disease
What is the Variables that increase sickling ?
1- Decreased oxygen
2- Increased pCO2
3- Decreased pH
4- Dehydration
Why Sickle Cell Disease give this name ?
Because have sickle shaped
In the HbC the glutamic acid is changed into ?
lysine
What is the HBCS ?
some ß-globin chains have HbC and some HbS.
In the Sickle Cell Disease the rigid polymers distort the cell and cause it to ?
bend out of shape.
What is the drug that induces fetal
hemoglobin production in some patients
with sickle cell disease and improves the
clinical condition of some of them ?
hydroxyurea
What is the complications of sickle cell disease?
1- promotes many of the heme component
2- free heme lodges in the red cell membrane.
What is the most common of Thalassemias ?
are α, β, and δ β
silent carrier of α – thalassemia.
If one of the four genes
