MMSC 433 Exam 2 with accurate detailed solutions ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
hemoglobinopathy - correct answer✔✔genetic mutation in the genes that affect hemoglobin ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
synthesis, leading to a diseased hemoglobin molecule ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
qualitative hemoglobinopathy - correct answer✔✔gene mutation leads to altered amino acid ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
sequence in the hemoglobin molecule, affecting the structure and function of the
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
hemoglobin
-amino acids with different charges and biochemical characteristics leads to dysfunctional and
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
unstable hemolgobin
||//\\|| ||//\\||
-e.g. sickle cell disease, hemoglobin C disease, etc.
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
quantitative hemoglobinopathy - correct answer✔✔reduced rate of globin chain production ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
leads to decreased amounts of hemoglobin
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-e.g. thalassemias, etc. ||//\\|| ||//\\||
hemoglobin S mutation - correct answer✔✔a2B2(6glu-val) ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-in the 6th position of the beta chain glutamic acid is replaced by valine
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-change in charge of +1 leads to changes in solubility, polymerization of hemoglobins (leads
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
to sickle shaped cells), crystal formation and decreased O2 carrying capacity
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
hemoglobin C mutation - correct answer✔✔a2B2(6glu-lys) ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-in the 6th position of the beta chain glutamic acid is replaced with lysine
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
sickle cell disease clinical manifestations - correct answer✔✔-reversible sickling
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-irreversible sickling ||//\\||
-vasoocclusive crisis (VOC)/ painful crisis ||//\\|| ||//\\|| ||//\\|| ||//\\||
,-autosplenectomy
-acute chest syndrome (ACS) ||//\\|| ||//\\|| ||//\\||
-fatal bacterial infections
||//\\|| ||//\\||
-avascular necrosis (AVN) ||//\\|| ||//\\||
-leg ulcers
||//\\||
-hand-foot syndrome (dactylitis) ||//\\|| ||//\\||
reversible sickling (SCD) - correct answer✔✔poor oxygenation causes RBCs to lose their
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
normal biconcave shape and become sickle cells
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-returning to proper oxygenation allows normal shape to return ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-occurs at ≤ 85% O2 in SCD ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-occurs at ≤ 40% oxygenation in sickle trait
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
irreversible sickling (SCD) - correct answer✔✔at a certain point sickle cells are too damaged
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
and cannot return to their normal RBC shape, even after proper oxygenation is restored
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-leads to poor oxygenation and excessive removal of RBCs by macrophages
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
vasoocclusive crisis (VOC/ painful crisis) (SCD) - correct answer✔✔*hallmark of SCD* ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-acidosis, hypoxia, dehydration, infection, inflammation, fever, extreme cold and hypoxia
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
causes dehydration and sickling of RBCs
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-blood becomes viscous, and has decreased flow, blocking up microvasculature
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-painful manifestations present around the body ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
autosplenectomy (SCD) - correct answer✔✔gradual loss of spleen function due to damage ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
and obstruction by sickle cells, eventually leading to nonfunctional spleen
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-increased risk of bacterial infection, HJ bodies and pappenheimer bodies ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
, acute chest syndrome (ACS) (SCD) - correct answer✔✔pulmonary infarction due to
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
microvascular damage by sickle cells ||//\\|| ||//\\|| ||//\\|| ||//\\||
-causes cough, chest pain and dyspnea ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-pulmonary infiltrates (white out) visible on chest radiograph ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-often occurs after VOC ||//\\|| ||//\\|| ||//\\||
fatal bacterial infections (SCD) - correct answer✔✔infections with Staph aureus, Strep
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
pneumoniae and Haemophilus influenzae can be fatal to a person with SCD ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
avascular necrosis (AVN) (SCD) - correct answer✔✔impaired blood flow to the femur and ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
humerus leads to increased intramedullary pressure in the bone ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-hip or shoulder implants often required
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-seen in 50% of SCD patients by age 35 ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
leg ulcers (SCD) - correct answer✔✔formation of recurring leg ulcers with chronic pain and
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
scarring
hand foot syndrome (dactylitis) (SCD) - correct answer✔✔*earliest manifestation of SCD,
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
seen in children* ||//\\|| ||//\\||
-sickling of RBCs blocks blood vessels, leading to tissue infarct in the metacarpals, metatarsals
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
and phalanges
||//\\|| ||//\\||
-hands and feet are puffy, warm, tender, and painful with reduced movement and fever
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
sickle cell disease treatments - correct answer✔✔-RBC transfusion: increases Hct and % Hgb
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
A
-analgesics
-desferroxamine: iron chelator that prevents iron over load from transfusion ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-stem cell transplant: induces production of normal Hgb
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
hemoglobinopathy - correct answer✔✔genetic mutation in the genes that affect hemoglobin ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
synthesis, leading to a diseased hemoglobin molecule ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
qualitative hemoglobinopathy - correct answer✔✔gene mutation leads to altered amino acid ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
sequence in the hemoglobin molecule, affecting the structure and function of the
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
hemoglobin
-amino acids with different charges and biochemical characteristics leads to dysfunctional and
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
unstable hemolgobin
||//\\|| ||//\\||
-e.g. sickle cell disease, hemoglobin C disease, etc.
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
quantitative hemoglobinopathy - correct answer✔✔reduced rate of globin chain production ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
leads to decreased amounts of hemoglobin
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-e.g. thalassemias, etc. ||//\\|| ||//\\||
hemoglobin S mutation - correct answer✔✔a2B2(6glu-val) ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-in the 6th position of the beta chain glutamic acid is replaced by valine
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-change in charge of +1 leads to changes in solubility, polymerization of hemoglobins (leads
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
to sickle shaped cells), crystal formation and decreased O2 carrying capacity
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
hemoglobin C mutation - correct answer✔✔a2B2(6glu-lys) ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-in the 6th position of the beta chain glutamic acid is replaced with lysine
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
sickle cell disease clinical manifestations - correct answer✔✔-reversible sickling
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-irreversible sickling ||//\\||
-vasoocclusive crisis (VOC)/ painful crisis ||//\\|| ||//\\|| ||//\\|| ||//\\||
,-autosplenectomy
-acute chest syndrome (ACS) ||//\\|| ||//\\|| ||//\\||
-fatal bacterial infections
||//\\|| ||//\\||
-avascular necrosis (AVN) ||//\\|| ||//\\||
-leg ulcers
||//\\||
-hand-foot syndrome (dactylitis) ||//\\|| ||//\\||
reversible sickling (SCD) - correct answer✔✔poor oxygenation causes RBCs to lose their
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
normal biconcave shape and become sickle cells
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-returning to proper oxygenation allows normal shape to return ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-occurs at ≤ 85% O2 in SCD ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-occurs at ≤ 40% oxygenation in sickle trait
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
irreversible sickling (SCD) - correct answer✔✔at a certain point sickle cells are too damaged
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
and cannot return to their normal RBC shape, even after proper oxygenation is restored
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-leads to poor oxygenation and excessive removal of RBCs by macrophages
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
vasoocclusive crisis (VOC/ painful crisis) (SCD) - correct answer✔✔*hallmark of SCD* ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-acidosis, hypoxia, dehydration, infection, inflammation, fever, extreme cold and hypoxia
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
causes dehydration and sickling of RBCs
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-blood becomes viscous, and has decreased flow, blocking up microvasculature
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-painful manifestations present around the body ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
autosplenectomy (SCD) - correct answer✔✔gradual loss of spleen function due to damage ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
and obstruction by sickle cells, eventually leading to nonfunctional spleen
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-increased risk of bacterial infection, HJ bodies and pappenheimer bodies ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
, acute chest syndrome (ACS) (SCD) - correct answer✔✔pulmonary infarction due to
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
microvascular damage by sickle cells ||//\\|| ||//\\|| ||//\\|| ||//\\||
-causes cough, chest pain and dyspnea ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-pulmonary infiltrates (white out) visible on chest radiograph ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-often occurs after VOC ||//\\|| ||//\\|| ||//\\||
fatal bacterial infections (SCD) - correct answer✔✔infections with Staph aureus, Strep
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
pneumoniae and Haemophilus influenzae can be fatal to a person with SCD ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
avascular necrosis (AVN) (SCD) - correct answer✔✔impaired blood flow to the femur and ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
humerus leads to increased intramedullary pressure in the bone ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-hip or shoulder implants often required
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-seen in 50% of SCD patients by age 35 ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
leg ulcers (SCD) - correct answer✔✔formation of recurring leg ulcers with chronic pain and
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
scarring
hand foot syndrome (dactylitis) (SCD) - correct answer✔✔*earliest manifestation of SCD,
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
seen in children* ||//\\|| ||//\\||
-sickling of RBCs blocks blood vessels, leading to tissue infarct in the metacarpals, metatarsals
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
and phalanges
||//\\|| ||//\\||
-hands and feet are puffy, warm, tender, and painful with reduced movement and fever
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
sickle cell disease treatments - correct answer✔✔-RBC transfusion: increases Hct and % Hgb
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
A
-analgesics
-desferroxamine: iron chelator that prevents iron over load from transfusion ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
-stem cell transplant: induces production of normal Hgb
||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\|| ||//\\||
