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GI II > CHOUD BSC 1-24 > Flashcards

CHOUD BSC 1-24 Flashcards

1
Q

What is the major product of nitrogen metabolism

A

urea

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2
Q

What is the ornithine cycle

A

urea cycle

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3
Q

where is ammonia from

A

aa catabolism

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4
Q

intracellularly what sequesters ammonia,

A

glutamic acid to form glutamine

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5
Q

where is the urea cycle

A

in the liver, and some in kidney

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6
Q

what transports glutamate from muscles

A

alanine

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7
Q

What organ produces alot of ammonia

A

brain

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8
Q

how is ammonium released from glutamine

A

glutamine–>glutamate–>alphaketoglutarate. each step releases an ammonium

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9
Q

how do we measure blood ammonium

A

BUN, nitrogen levels

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10
Q

In which organelle does urea cycle start

A

mitchondria

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11
Q

deficiency in carbamoyl phosphatase 1 will lead to what

A

high levels ammonium in blood= bad

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12
Q

what forms citrulline and why

A

carbamoyl and ornithine form citrulline so can be shuttle out of mitochondria into cytosol of lvier

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13
Q

What are the 5 steps urea cycle

A

carbamoyl phosphate combine with ornithine to form citrulline
citrulline converted to arginino-succinate
argininosuccinate cleaved f=to fumurate and arginine
urea and ornthinite formed
formation urea

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14
Q

What combines carbamoyl phsophate and ornithine to make citrulline

A

ornithine transcarbamoylase

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15
Q

What can result from elevated NH4 in blood

A

hyperammonemia–>toxic in brain, cerebral edema, convulsions, coma and death

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16
Q

What is the role of glutaminase synthetase

A

combines nitrogen and glutamate to make glutamine

17
Q

What is the role of glutaminase

A

deaminates glutamine to glutamate and NH3

18
Q

What 5 enzymes are important in urea cycle

A 
carbamoylphosphate synthetase I
ornithine transcarbamylase
argininosuccinate synthetase
argininosuccinate lyase
arginase
19
Q

What is the pacemaker enzyme of urea cycle

A

carbamoyl phosphate synthase I to keep cycel running

20
Q

activity of CPS I is dictated by what

A

rate of synthesis from aCoA and glutamate because increases Nacetyl glutamateNAG

21
Q

Why does starvation increase CPS I activity

A

increased production of ammonia from increased protein digestion

22
Q

What causes citrullinemia

A

deficiency of argininosuccinic acid synthase

23
Q

all (but one) urea cycle disorders are autosomal recessive. which one is not

A

ornithine transcarbomylase deficiency is X linked dominant- mitochondria

24
Q

infant is vomiting drowsy increased temp and HR, hepatomegaly, high glutamine and uracil
what does this suggest

A

hyperammonemia- impaired urea formation
Probably ornithinine transcarbamoylase because increased uracil (orotic acid)
transamination enzymes are fine because glutamine is being made

25
Q

Tx for ornithine transcarbomoylase deficiency?

A

give less protein in diet

supplemental dietary arginine

26
Q 
PTT that does not get better with Vit K
bilirubin in urine
high liver enzymes
hypocalcemia
low BUN
what does this suggest?
A

chronic hepatitis B- end stage liver disease
Vit K no response shows liver is unable to synthesize the PT protein cofactors II VII IX and X
hypocalcemia because no albumin
low BUN–>liver

27
Q

ALT is higher than AST in what pahtology

A

acute hepatitis

28
Q

hypoabluminemia and prolonged PTT are markers for what

A

severe liver disease since liver synthesizes the proteins

29
Q

macrocytic anemia suggests what

A

folate deficiency or B12 deficiency

30
Q

Extreme levels of plasma ammonium in an infant usually means what

A

OTC deficient– x linked

CPS I affected

31
Q

infants with urea cycle disorders present how

A

normal initially and rapidly develop cerebral edema, lethargy, anorexia, hyperventilation etc.

32
Q

normal urea level

A

20-40 mg/dL

33
Q

high glutamine and orotic acid in urine as well as low BUN is what disorder

A

mitochondrial OTC

34
Q

HArtnup disease patients don’t become deficient in aa because what

A

PepT1

GI II (12 decks)

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