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Hbss Sickle Cell Disease And Anesthesia Questions And Answers Well Elaborated Rated A+
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Hbss Sickle Cell Disease AndAnesthesia Questions And Answers Well
Elaborated Rated A+
definition of sickle cell disease - ANSWER✔✔ a genetic disorder resulting from the
presence of a mutated form of hemoglobin (HbS). the most common form of SCD
is homozygous HbS disease (HbSS) causing significant morbidity and mortality in
African and mediterranean ethnicities
structure of hemoglobin - ANSWER✔✔ alpha globulin protein
made up of heme and globin
What is heme and what is globin? - ANSWER✔✔ heme: the iron containing part of
the molecule
globin: the protein part of the molecule
what are the 3 normal types of hemoglobin - ANSWER✔✔ HbA- 2 alpha, 2 beta
HbF- 2 alpha, 2 gamma
HbA2- 2 alpha, 2 delta
pathophys of sickle cell disease - ANSWER✔✔ in HbS, valine is substituted for
glutamic acid in the 6th amino acid on the beta chain
upon deoxygenation, HbS undergoes polymerization which causes sickling
how does HbS affect the RBC - ANSWER✔✔ rbc survival reduced to 10-15 days,
instead of the normal 120 days
, HbS has less affinity for oxygen as well as decreased solubility
sickle shaped RBCs cause vaso-occlusive crises leading to infarct
sickling may initially be reversible but becomes irreversible
types of sickle cell disease - ANSWER✔✔ Sickle cell SS
Sickle cell SC
Sickle cell trait
sickle cell SS (HbSS) - ANSWER✔✔ homozygous form of hemoglobin S disease
sickle cell SC (HbSC) - ANSWER✔✔ HbC inherited from one parent, and HbS
inherited from the other
similar s/s to individuals with HbSS but anemia is less severe
sickle cell trait - ANSWER✔✔ heterozygous form of Hb S
patients are generally not anemic, are asymptomatic, and have a normal life span
SCD and CNS - ANSWER✔✔ stroke, cranial nerve neuropathy, peripheral
neuropathy
SCD and opthal - ANSWER✔✔ sickle retinopathy
SCD and pulmonary - ANSWER✔✔ infarct, pneumonia, acute chest syndrome
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