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Renal Exam 1 > Nitrogen Metabolism > Flashcards

Nitrogen Metabolism Flashcards

1
Q
  • What is the first step in nitrogen removal?
A
  • Oxidative deamination
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2
Q
  • What is the rate limiting step in N2 metabolism?
A
  • Converting ammonia to carbamoyl phosphate via carbamoyl phosphate synthetase and NASG
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3
Q
  • Who is the star of N2 flow in the liver?
  • Who is the star of N2 flow in the other tissues?
A
  • Glutamate (liver has a mate)
  • Glutamine
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4
Q
  • What ion are amino acids commonly transported with?
A
  • Na+
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5
Q
  • Where in the nephron does AA reabsorption occur?
A
  • Proximal convuluted tubule
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6
Q
  • Hartnup disease is a defect in transport of what type of amino acids?
  • What amino acids will you see elevated in urine?
  • What is Trp (one of the AAs of Hartnup) a common precursor for?
  • What are the symptoms of Hartnup?
  • Tx?
A
  • Non-polar or neutral
  • Ala, Ser, THr, Val, Leu, Ile, Phe, Tyr, Trp, Gln, Asn, His
  • Serotonin, Melatonin, Niacin (precursor for NAD)
  • Failure to thrive, nystagmus, photosensitivity
  • Tx-niacin repletion and high protein diet (can also do nicotinamide supplementation)
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7
Q
  • Why is NA (vitamin b3) a good tx for Hartnup?
A
  • Can make NAD without need for de novo pathway using tryptophan
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8
Q
  • A defect in what type of amino acid transport leads to cytinuria?
  • How is the disease inherited?
  • What is the common presentation?
A
  • Dibasic
  • AR
  • Cystine crystals in kidneys, renal colic

COAL stones

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9
Q
  • What is the biochemical defect in PKU (Classical)?
  • What is Phe converted to instead?
  • What are the common symptoms?
  • How is it treated?
A
  • Phe cannot be converted to Tyr
  • Converted to phenypyruvate and phenynllacetate (musty urine odor) and phenylacetate
  • Severe impairment of brain function
  • Tyr supplementation and dietary limit of Phe
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10
Q
  • What is the biochemical defect in non-classical PKU?
    *
A
  • Deficiency in THB (Defects in synthesis or regeneration of BH4)
  • THB is a cofactor for phenylaline hydroxylase
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11
Q
  • What are the key diagnosis and treatment plans for a patient with PKU?
A
  • Diagnose before 2 weeks of age
  • Levels > 360 umol/L
  • Treat with THB (if non-classical)
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12
Q
  • What enzymatic defect is present in Type I Tyrosinemia?
  • What is excreted that is toxic to the liver and kidneys?
  • What is their presentation?
  • How is it treated?
A
  • Fumarylacetoacetate hydroxylase
  • Converts fumarylacetoacetate to fumarate (like the last step in the pathway she gave us)
  • Excrete succinylacetone (interferes w/ TCA cycle)
  • Renal tubule dysfunction, lack of heme, cabbage like smell
  • Liver transplant or Nitisinone (Prevents formation of funarylacetoacetate so it does not have to be broken down)
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13
Q
  • What enzymatic deficiency is present in type II tyrosinemia?
  • What are presenting sx?
A
  • Tyrosine aminotransferase (can’t convert tyrosine to p-hydroxyphenylpyruvate)
  • Photophobia, skin lesions
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14
Q
  • What enzymatic deficiency is present in Type III tyrosinemia?
  • What are common presenting sx?
A
  • P-hydroxyphenylpyruvate oxidase
  • Intermittent ataxia
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15
Q
  • What enzymatic deficiency is present in individuals with alkaptonuria?
  • Describe key characteristics of the disease
A

Defect in homogentisate oxidase

AR

Black urine disease

Auto-oxidation of homogensitic acid produces dark pigments in urine and bone

Black pigment in intervertebral discs, degenerative arthritis, onchonosis

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16
Q
  • Ammonia Toxicity is excessive ammonia d/t disorders in the _ or _ failure and can have highly toxic effects on the CNS
  • _ can permeate membranes and cause pH imbalance, swelling of astrocytes-causing _ edema and hypertension
  • Can be due to a deficiency in the _ enzyme that converts glutamate to alphaketoglutarate
  • Glutamate is essential for the formation of the _ NTX
  • Issues with _ cycle of metabolism
A
  • urea cycle, liver
  • NH3, cerebral
  • glutamate dehydrogenase
  • GABA
  • TCA
17
Q
  • Gout is characterized by high levels of _ in the blood
  • Primary hyperuricemia v. Secondary? (which is more common?)
  • _ deposits in joints of extremeties and in kidneys
  • What kind of diet/population is at risk?
  • Tx options?
A
  • Urate/uric acid
  • Secondary is more common (secondary is underexcretion of uric acid) and primary is caused by the overproduction of uric acid
  • Sodium urate
  • Purine rich diet (beans, spinach, meats, alcohol), diabetics, men
  • Allopurinol-xanthine oxidase inhibitor OR Colchine-decreases movement of granulocytes to affected areas
18
Q
  • Acidemia stimulates the reabsorption of uric acid in the kidney via _ transporter
A

URAT1

19
Q
  • Xanthine oxidase is responsible for what two reactions?
  • What would there be a buildup of if allopurinol is used?
A
  • Converting hypoxanthine to xanthine
  • Converting xanthine to uric acid
  • Hypoxanthine
20
Q
  • Hyperammonemia occurs with defects in what three transporters? What else can cause it?
  • What is the treatment?
A
  • Mitochondrial ornithine carries, mitochondrial Asp/Glu carrier, dibasic amino acid transporter
  • Defects w/ enzymes of the urea cycle
  • Tx- limiting protein consumption (and components that conjugate and promote excretion of ammonia)
21
Q
  • Most cases of hyperammonemia are inherited in which fashion?
  • What is an exeption and how is this inherited?
  • What is the common presentation with this case?
A
  • AR
  • X linked; Ornithine transcarboxylase enzymatic deficiency hyperammonemia
  • Orotic aciduria
22
Q
  • What is important about CYTOSOLIC Carbamoyl phosphate synthase II?***
  • What stimulates it and what inhibits it?
  • What is an intermediate in this pathway?
A
  • First step of de novo pyrimidine synthesis (Does not require NAG)
  • PPP, UTP
  • Orotic acid
23
Q
  • Orotic aciduria from _ deficiencies IS NOT accompanied by hyperammonemia or reduced BUN?
A
  • UMP synthase deficiencies
24
Q
  • Reacting plasma with _ reagent in presence or absence of _ will measure _ and _ bilirubin
  • Difference between the two will give you _ bilirubin levels
  • What is the difference between direct/indirect bilirubin>?
A
  • Diazo, methanol, total, direct
  • Indirect
  • Direct=conjugated=water soluble
  • Indirect=unconjugated=insoluble
25
Q
  • Blue light is used to treat _ in newborns by aiding in the _ of bilirubin
  • Severe jaundice occurs when?
A
  • Jaundice, conjugation
  • When there is a need to replace the number of red blood cells
    • Abnormal blood cell chapes
    • RH incompatability
    • Cephalohematoma (her son had this so I would know it)
    • Infection
    • Enzymatic deficiencies
    • Premature birth
    • Hemolytic anemias

Renal Exam 1 (7 decks)

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