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*nbme 3 block 4 -q1----------q50 - drona99
#1
lets start..
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#2
1--cc

Pathogenesis of β-thal

Decrease in β-globin chain synthesis
Mild anemia is most often due to DNA splicing defects.
Severe anemia is due to a nonsense mutation with formation of a stop codon.
Premature termination of β-globin chain synthesis or absent β-globin chain synthesis.
Normal synthesis of α, δ, γ-globin chains.

β-Thal minor (β/β+)
Mild microcytic anemia
Mild protective effect against falciparum malaria
RBC life span is shorter than normal.
Decreased MCV, Hb, and Hct
Increased RBC count
Normal RDW and serum ferritin
Hb electrophoresis
Decreased HbA (2α/2βWink
Increased HbA2 (2α/2δWink and HbF (2α/2γWink
There is no treatment.


β-Thal major (Cooley's anemia; β0/β0)
Severe hemolytic anemia
RBCs with α-chain inclusions are removed by macrophages in the spleen.
Causes an increase in unconjugated bilirubin
RBCs with α-chain inclusions undergo apoptosis in the bone marrow (ineffective erythropoiesis).
Extramedullary hematopoiesis
Increased RDW and reticulocytes
Hb electrophoresis
No synthesis of HbA
Increase in HbA2 and HbF
Long-term transfusion requirement
danger of iron overload (called hemosiderosis)
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#3
2--BB
competitve antagonist--
Competitive inhibitors resemble the substrate and overcome by increase in substate concentration so Km will be increased and there is no change in velocity.
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#4
3--cc
Glogi apparatus for post tarnslational modification of golgi apparatus

http://www.britannica.com/EBchecked/medi...osynthesis

http://www.ncbi.nlm.nih.gov/books/NBK269...objectonly

http://bcs.whfreeman.com/thelifewire/con...02002.html

Glogi apparatus adds mannose 6 phosphate to specific lysosomal protin to target the protein to the lysosome .Failure of this results in I cell disease ;enzymes are secreted outside the cell instead of being targeted to lysosome .
c/f coarse fascial features ;clouding of cornea; coarse fascial features ;restricted joint movement and high plasma levels of lysosomal enzymes.
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#5
4--CC
Influx of Na --depolerisation
efflux of K --repolerisation
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#6
5==EE
allel frequency
BB =45 +45
AB=25
so total 115/200
so ee
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#7
6--cc
nuetrophil chemotactic factors are
C5a
LTB4
IL8
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#8
7--AA
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#9
8--A
malignant hyperthermia associated with use of skeletal muscle relaxant succynylcholine .

Genoype susceptibility related to ryanodine receptars and or protein component of L -type calcium channel in skeletal muscle.

Rx with dantrolene which blocks ca channels and release of ca from sarcoplamic reticulum and decreases muscle contractions.
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#10
9--CC
From the graph t 1/2 life of drug is 3 hrs
so afeter 1 half life cocn in plsam will be 50%
2 half life = 75%
3half life=87.5%
4half life =93.75%
5half life =97%

so 5 X 3==15 hrs
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