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HLS > Heme Biochemistry > Flashcards

Heme Biochemistry Flashcards

1
Q

What is the inheritance pattern of sideriblastic anemia

A

X-linked

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2
Q

How does protoporphyrin production match to iron availability ?

A

ALAS2 has IRE

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3
Q

How do we manage acute attacks of porphyria?

A

Hemein (IV injection) & glucose (needed for succynil coA)

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4
Q

How is porphyria photosensitivity managed?

A

B-carotene (anti oxidant) & phlebotomy (removing porphyrins)

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5
Q

What does MPS stand for?

A

Mononuclear Phagocyte system (macrophages of liver & spleen)

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6
Q

What is the biochemistry of kernicterus ?

A

Drugs displace bilirubin in liver releasing it into the CNS

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7
Q

At what level of blood bilirubin is it considered jaundice

A

2-3mg/dl

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8
Q

How many a-helices does a-globin have ?

A

7

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9
Q

How many a-helices does b-globin have ?

A

8

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10
Q

How many a-helices does myoglobin have ?

A

8

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11
Q

What is Le Chatalier principle?

A

When H+ decrease the sigmoidal curve is shifted to the left

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12
Q

What is bohr effect

A

Affinity of o2 decreasing with increased co2

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13
Q

What is the mirror effect of bohr in the lungs?

A

Haldane effect

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14
Q

How much higher is the affinity of Hb for CO than for O2?

A

220x

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15
Q

What is the main mode of transport for CO2

A

In plasma as HCO3- (80%)

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16
Q

What types of Hb does the yolk sac produce?

A

Gower & Portland

17
Q

Which chromosome is the gene for a-globin on?

18
Q

Which chromosome is the gene for b-globin on?

19
Q

Position of amino acid affected in sickle cell anemia?

20
Q

How does hydroxyurea help with sickle cell anemia?

A

It increase transcription of HbF

21
Q

What does HbC look like under microscope?

A

Target cells

22
Q

What is the name of the enzyme that corrects oxidation of ferrous in hemoglobin?

A

NADH-Cyt b5 reductase

23
Q

What diseases cause chocolate cyanosis in blood?

A

Methemoglobinemia

24
Q

How does methemoglobinemia happen?

A

Resistance to ferric reducing enzyme NADH-Cyt b5 reductase

25
Q

How is methemoglobinemia treated?

A

Methylene blue or vitamin C

26
Q

Why is sickle cell anemia considered extravascular?

A

Most hemolysis occurs in liver in spleen because of slow blood flow increasing sickling

27
Q

Inheritance pattern of porphyrias?

A

Autosomal dominant

28
Q

What enzyme deficiency causes conginetal erythropoietic porphyria ?

A

Uroporphyrinogen III synthase

HLS (13 decks)

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