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Urinalysis > Chapter 8: 1.2- Branched Chain Amino Acid > Flashcards

Chapter 8: 1.2- Branched Chain Amino Acid Flashcards

1
Q
  • differ from other amino acids by having a methyl group that branches from the main aliphatic carbon chain
A

Branched-Chain Amino Acid Disorders

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2
Q

Branched-Chain amino acid: (2)

A
  1. Maple Syrup Urine Disease

2. Organic Acidemias

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3
Q

-mainly acused by accumulation of one or more of the early amino acid degradation product.

A

Maple Syrup Urine Disease

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4
Q

maple syrup urine disease

- deficiency of the metabolic enzyme , (BCKDH)

A

branched chain a-keto acid dehydrogenase

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5
Q

results to buildup of branched-chain amino acids (3) in the blood and urine

A

Maple Syrup Urine Disease

  • Leucine
  • Isoleucine
  • Valine
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6
Q

pappear normal at birth but by age 4-7 days, develop lethargy, severe vomiting and signs of failure to thrive

A

Maple Syrup Urine Disease

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7
Q
  • Death usually occurs during the first year in the classic form of the disease
  • if left untreated, the disease causes severe mental retardation, convulsion, acidosis and hypoglycemia
A

Maple Syrup Urine Disease

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8
Q

Maple Syrup Urine Disease Test:

6

A
  1. Modified Guthrie test
  2. DNPH
  3. Amino acid chromatography
  4. Ferric chloride
  5. Nitroso-napthol
  6. Acetest
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9
Q

Maple Syrup Urine Disease Test:
DNPH
color

A

yellow turbidity or precipitate

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10
Q

Maple Syrup Urine Disease Test:

confirmatory

A

amino acid chromatography

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11
Q

Maple Syrup Urine Disease Test:
ferric chloride
color

A

green-gray

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12
Q

Maple Syrup Urine Disease Test:

nitro-napthol

A

red

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13
Q

Maple Syrup Urine Disease Test:

acetest

A

purple

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14
Q
  • results in accumulation of organic acids produced further down in the amino acid metabolic pathway
A

Organic Acidemias

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15
Q

Organic Acidemias:

kinds (3)

A
  1. Isovaleric Acidemia
  2. Propinic Acidemia
  3. Methylmalonic Acidemia
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16
Q

urine has swety feet odor

A

Isovaleric Acidemia

17
Q
  • results from error in the metabolic pathway convertin isoleucine, valine, threonine, and methionine to succinyl coenzyme
A

Propionic Acidemia

18
Q

Methylamolnic Acidemia screening test and color

A

p-nitroaniline test

- emerald green

Urinalysis (24 decks)

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