Urinalysis chap 8

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All states require newborn screening for PKU for early: A. Modifications of diet B. Administration of antibiotics C. Detection of diabetes D. Initiation of gene therapy

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27 Terms

1

All states require newborn screening for PKU for early: A. Modifications of diet B. Administration of antibiotics C. Detection of diabetes D. Initiation of gene therapy

A. Modifications of diet

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2

All of the following disorders can be detected by newborn screening except: A. Tyrosyluria B. MSUD C. Melanuria D. Galactosemia

C. Melanuria

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3

The best specimen for early newborn screening is a: A. Timed urine specimen B. Blood specimen C. First morning urine specimen D. Fecal specimen

B. Blood specimen

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4

Abnormal urine screening tests categorized as an overflow disorder include all of the following except: A. Alkaptonuria B. Galactosemia C. Melanuria D. Cystinuria

C. Melanuria

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5

Which of the following disorders is not associated with the phenylalanine-tyrosine pathway? A. MSUD B. Alkaptonuria C. Albinism D. Tyrosinemia

A. MSUD

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6

The least serious form of tyrosylemia is: A. Immature liver function В. Туре 1 С. Туре 2 D. Турe 3

A. Immature liver function

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7

An overflow disorder of the phenylalanine-tyrosine pathway that would produce a positive reaction with the reagent strip test for ketones is: A. Alkaptonuria B. Melanuria C. MSUD D. Tyrosyluria

C. MSUD

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8

An overflow disorder that could produce a false-positive reaction with Clinitest procedure is: A. Cystinuria B. Alkaptonuria C. Indicanuria D. Porphyrinuria

B. Alkaptonuria

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9

A urine that turns black after sitting by the sink for several hours could be indicative of: A. Alkaptonuria B. MSUD C. Melanuria D. Both A and C

D. Both A and C

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10

Ketonuria in a newborn is an indication of: A. MSUD B. Isovaleric acidemia C. Methylmalonic acidemia D. All of the above

D. All of the above

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11

Urine from a newborn with MSUD will have a significant: A. Pale color B. Yellow precipitate C. Milky appearance D. Sweet odor

D. Sweet odor

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12

Hartnup disease is a disorder associated with the metabolism of: A. Organic acids В. Tryptophan C. Cystine D. Phenylalanine

В. Tryptophan

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13

5-HIAA is a degradation product of: A. Heme B. Indole C. Serotonin D. Melanin

C. Serotonin

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14

Elevated urinary levels of 5-HIAA are associated with: A. Carcinoid tumors B. Hartnup disease C. Cystinuria D. Platelet disorders

A. Carcinoid tumors

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15

False-positive levels of 5-HIAA can be caused by a diet high in: A. Meat B. Carbohydrates C. Starch D. Bananas

D. Bananas

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16

Place the appropriate letter in front of the following statements. A. Cystinuria B. Cystinosis ____IEM ____Inherited disorder of tubular reabsorption ___Fanconi syndrome ____Cystine deposits in the cornea ____Early renal calculi formation

B,A,B,B,A

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17

Blue diaper syndrome is associated with: A. Lesch-Nyhan syndrome B. Phenylketonuria C. Cystinuria D. Hartnup disease

D. Hartnup disease

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18

Homocystinuria is caused by failure to metabolize: A. Lysine B. Methionine C. Arginine D. Cystine

B. Methionine

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19

The Ehrlich reaction will only detect the presence of: A. Uroporphyrin B. Porphobilinogen C. Coproporphyrin D. Protoporphyrin

B. Porphobilinogen

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20

Acetyl acetone is added to the urine before performing the Ehrlich test when checking for: A. Aminolevulinic acid B. Porphobilinogen C. Uroporphyrin D. Coproporphyrin

B. Porphobilinogen

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21

The classic urine color associated with porphyria is: A. Dark yellow B. Indigo blue C. Pink D. Port wine

D. Port wine

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22

Which of the following specimens can be used for porphyrin testing? A. Urine B. Blood С. Feces D. All of the above

D. All of the above heme

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23

The two stages of heme formation affected by lead poisoning are: A. Porphobilinogen and uroporphyrin B. Aminolevulinic acid and porphobilinogen C. Coproporphyrin and protoporphyrin D. Aminolevulinic acid and protoporphyrin

D. Aminolevulinic acid and protoporphyrin

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24

Hurler, Hunter, and Sanfilippo syndromes are hereditary disorders affecting metabolism of: A. Porphyrins B. Purines C. Mucopolysaccharides D. Tryptophan

C. Mucopolysaccharides

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25

Many uric acid crystals in a pediatric urine specimen may indicate: A. Hurler syndrome B. Lesch-Nyhan disease C. Melituria D. Sanfilippo syndrome

B. Lesch-Nyhan disease

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26
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27.D,F,A,E,C,B

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27

Deficiency of the GALT enzyme will produce a: A. Positive Clinitest B. Glycosuria C. Galactosemia D. Both A and C

D. Both A and C

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