13 Sickle Cell
Last updated
Last updated
2 genes, 1 from each parent
A to T leads to aa substition
dehydration/acidosis: acute exacerbation
polymerize and make RBC form crescents
decrease ESR instead of up
hemolytic anemia similar to beta thalassemia major
vaso occlusion: more dangerous and unique symptoms
sometimes simply rupture in vasculature
heart, brain
first thing that happen
jagged femoral head
dehydration lead to sickling
can become dependent on narcotics
have spleen but not working
can die from infection
salmonella encapsulated, staph not
The spleen may demonstrate brownish discoloration (hemosiderosis) due to extensive ingestion of sickled RBCs by splenic macrophages (extravascular hemolysis).
fish bone: Osteomyelitis
sickle: In Sickle Cell disease
sickle: Increased risk of infection in sickle cell or asplenic patients
pooling of RBC in spleen from obstruction
children: spleen not yet fibrosis
treated with antibiotics because looks just like pneumonia. Don't know if microbe present or not
papillary necrosis
high concentration in medulla abolished
most severe form
just as likely to be infected, less likely to die
less beta production depending on how severe infection is
lysine more polar than valine: not as severe of a problem
lysing more positive than glutamate, less travel than HbS and Hb normal on electrophoresis
very rare to be homozygous
heterozygous: usually mild anemia without sickling
except HbS plus HbC