Hemoglobin Flashcards

(61 cards)

1
Q

in what form must iron be to bind oxygen?

A

reduced - ferrous

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2
Q

what is hemoglobin with iron in ferric form called?

A

methemoglobin

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3
Q

which enzyme reduces iron

A

cytochrome b5 reductase

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4
Q

confirmation of deoxy hemoglobin?

A

taut (T)

due to salt bonds, hydrogen bonding, and hydrophobic interactions

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5
Q

confirmation of oxy hemoglobin

A

relaxed - binding oxygen causes taut bonds to progressively break
(R)

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6
Q

p50 of HbA at standard conditions

A

27mmHG

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7
Q

2,3BPG is by-product of?

A

anaerobic glycolysis

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8
Q

2,3BPG binds?

A

pocket between beta chains, stabilizing hgb in deoxygenated T state –> decreased O2 affinity –> right shift

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9
Q

cardiac left to right shunt –> 2,3bpg?

A

increased 2,3 bpg –> right shift

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10
Q

severe anemia –> 2,3bpg?

A

increase

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11
Q

septic shock –> 2,3bpg?

A

decrease

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12
Q

hpophophatemia –> 2,3bpg

A

decrease

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13
Q

congestive heart failure –> 2,3bpg

A

increase

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14
Q

bpg mutase deficiency –>

A

decrease in 2,3bpg

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15
Q

hepatic cirrhosis –> 2,3bpg

A

increase

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16
Q

transfusion of stored blood –> 2,3bpg

A

decrease

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17
Q

severe acidosis –> 2,3bpg

A

decrease (because beyond bohr effect)

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18
Q

methemoglobinema - curve shift?

A

left

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19
Q

carbon monoxide poisoning - curve shift?

A

left

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20
Q

fetal hemoglobin?

A

alpha2gamma2

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21
Q

when does fetal hemoglobin dominate

A

after 8 weeks

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22
Q

does Hgb F bind 2,3BPG better or worse than adult?

A

worse

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23
Q

fetal curve relative to adult

A

left

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24
Q

Hemoglobin A2

A

alpha2/delta2
2% adult
functions like HgbA except more heat stabile and slightly higher affinity for O2

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25
HgbA2 elvated in?
beta thalassemia sickle cell hyperthyroidism megaloblastic anemia
26
unstable hemoglobins have tendency to spontaenously denature often due to mutations that disrupt the stablility of?
the heme-globin linkage
27
untable hemoglobins may lead to
hemolytic anemia with jaundice and splenomegaly
28
unstable hemoglobins can be referred to as?
Heinz body anemia
29
hemoglobin koln
unstable one of the most common mutation in beta increased affinity (left)
30
hemoglobin poole
unstable hemoglobin gamma chain mutation infants with hemolytic anemia - which resulsove within a few months becasue you no longer make gamma
31
do unstable hemoglobins usually need blood transfusions?
no
32
unstable hemoglobin treatment
folic acid * splenectomy is not curative * may not see Heinz bodies until after splenectomy
33
Hemoglobin chesapake
high affinity | alpha globin chain
34
altered oxygen affinity hemoglobins and their stabilty
usually stable
35
altered oxygen affinity hemoglobins and electrophoresis
usually abnormal
36
altered oxygen affinity hemoglobins and hemolysis
usually none
37
high oxygen affinity variants and tissue oxygenation
leads to hypoxia --> leads to EPO production by kidney --> increased RBC count *generally no treatment need
38
low O2 affinity Hb anemia? presenation?
mild anemia | cyanosis
39
cyanosis most common cause?
deoxygenated hemoglobin - not necessarily hypoxia
40
differential dx | cyanosis and globins (3)
too much deoxy too much methemoglobin sulfhemoglobinemia
41
differential dx of cyanosis: | inadequate O2 of Hb (5)
``` pulmonary disorders cardiac right to left shunt congestive heart failur cardiovascular collapse (shock) low o2 affinity Hb variant ```
42
differenital methemoglobinemia (5)
``` congenital cytochrome b5 reductase deficiency cytochrome b5 deficiency M hemoglobins Acquired (drugs / toxins) ```
43
differential sulfemoglobinemia
acquired (drugs/toxins)
44
methemoglobinemia
Fe3+ cannot carry oxygen curve shifts left p50 goes down 1% is normal
45
color of methemoglobin
chocolate :)
46
congential methhemoglobinemia
cytochrome b5 reductase deficiency (autosomal recessive)
47
congential methemoglobinemia - clinical
increased o2 affinity blue and well at birth (will be blue but does not affect qol) up to 40% methemoglobin asymtpomatic heterozygotes unless oxidant expozed
48
hemoglobin M
autosomal dominant mutation in a or b chain making fe3+ resistant to reduction asymptomatic cyanosis normal amount of hemoglobin with mild hemolysis abnormal electrophoresis - M band
49
acquired methemoglobinemia | potential ways to aquire (2)
drugs | well water
50
acquired methemoglobinemia when and what to treat with?
if level is greater than 40% and there are symtpoms - give methylene blue (an artificial electron acceptor)
51
methemoglobin (acute vs chornic?)
acutely ill will yield problems - give methylene blue | chronic not so much
52
hemoglobin F | is it oxidized more or less readily to ferric state
more readily
53
cytochrome b5 reductase activity in first few months of life?
low
54
what might cause a bambino to be cyanotic?
well water raw spinach disinfectants benzocaine
55
CO poisoning affinity? curve shift?
more affinity (240x) shift left * so you are not only outcompeting hemoglobin for oxygen binding but you are making it more difficult for the hemaglobin that has successfully bound oxygen to release it)
56
CO poisoning... more or less of leftward shift than mehemoglobin?
more
57
what color are CO people?
cherry red?
58
CO poisoning treatmetn
100% O2/hyerbaric chamber
59
How do we assess oxygen saturation?
arterial blood gas (traditionally) | now we use pulse ox
60
how does pulse ox work?
photo detector and tow light emitting diodes 640 deoxy absorption 940 oxy absorption
61
ideal ox sat?
90s or 100 | 16000 ft? low 80s