[sicklecell] Flashcards
Beta
Glutamic acid –> valine
HbS
africans
heterozygotes (HbAS)
anaemia
heterozygote (HbAS)
hypoxia
anaethesia/enclosed spaces etc
vessels occluded by build up of sickle cells leading to painful ischaemia
thalassaemia
HbC
polymerisation of deformed HbS when deoxygenated
sickle cells are fragile
sickle cells
Target cells
Hb electrophoresis
allows distinction between HbSS and HbAS
at birth (cord blood)
pneumococcal prophylaxis
sickle cell reduces spleen function i.e.immunocompromised
vaso-occlusive crisis
acute abdomen
vaso-occlusive crisis
doppler US
bone
e.g. avascular necrosis of femoral head
priapism
parvovirus b19
invades and destroys reticulocytes
especially RBCs
the spleen hasnt undergone atrophy
intrasplenic trapping of RBCs usually affecting children
sequestration crisis
death in 1-2 hours due to circulatory failure
infection
hydroxycarbamide
increases the level of HbF
bone marrow transplant
urgent transfusion
acute chest syndrome
pulmonary infiltrates
tachypnoea
pain
fever
O2
analgesia
emirical antibiotics
transfusion if severe (X-match)
fat embolism
infection
chlamydia
mycoplasma
severe chest crisis
CNS event
multiorgan failure