Select the one best response to each question!
All states require newborn screening for PKU for early:
All of the following disorders can be detected by newborn screening except:
The best specimen for early newborn screening is a:
Abnormal urine screening tests categorized as an overflow disorder include all of the following except:
Which of the following disorders is not associated with the phenylalanine-tyrosine pathway?
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Urine screening tests for PKU utilize:
The least serious form of tyrosylemia is:
An overflow disorder of the phenylalanine-tyrosine pathway that could produce a false-positive reaction with the reagent strip test for ketones is:
An overflow disorder that could produce a falsepositive reaction with clinitest is:
A urine that turns black after sitting by the sink for several hours could be indicative of:
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Ketonuria in a newborn is an indication of:
Urine from a newborn with MSUD will have a significant:
A substance that reacts with p-nitroaniline is:
Which of the following has a significant odor?
Hartnup disease is a disorder associated with the metabolism of:
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5-HIAA is a degradation product of:
Elevated urinary levels of 5-HIAA are associated with:
False-positive levels of 5-HIAA can be caused by a diet high in:
Urine from patients with cystine disorders will react with:
Blue diaper syndrome is associated with:
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Homocystinuria is caused by failure to metabolize:
Early detection is most valuable for correction of:
Please select 2 correct answers
The Ehrlich reaction will only detect the presence of:
Acetylacetone is added to the urine prior to performing the Ehrlich test when checking for:
The classic urine color associated with porphyria is:
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Which of the following specimens can be used for porphyrin testing?
The two stages of heme formation affected by lead poisoning are:
Please select 2 correct answers
Hurler, Hunter, and Sanfilippo syndromes are hereditary disorders affecting metabolism of:
Many uric acid crystals in a pediatric urine specimen may indicate:
Deficiency of the GALT enzyme will produce a:
Please select 2 correct answers
Share your Results:
| 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 |
| 2.All of the following disorders can be detected by newborn screening except:
A. Tyrosyluria B. MSUD C. Melanuria D. Galactosemia |
| 3.The best specimen for early newborn screening is a:
A. Timed urine specimen B. Blood specimen C. First morining urine specimen D. Fecal specimen |
| 4.Abnormal urine screening tests categorized as an overflow disorder include all of the following except:
A. Alkaptonuria B. Galactosemia C. Melanuria D. Cystinuria |
| 5.Which of the following disorders is not associated with the phenylalanine-tyrosine pathway?
A. MSUD B. Alkaptonuria C. Albinism D. Tyrosinemia |
| 6.Urine screening tests for PKU utilize:
A. Microbial inhibition B. Nitroso-napthol C. Dinitrophenylhydrazine D. Ferric chloride |
| 7.The least serious form of tyrosylemia is:
A. Immature liver function B. Type 1 C. Type 2 D. Type 3 |
| 8.An overflow disorder of the phenylalanine-tyrosine pathway that could produce a false-positive reaction with the reagent strip test for ketones is:
A. Alkaptonuria B. Melanuria C. MSUD D. Tyrosyluria |
| 9.An overflow disorder that could produce a falsepositive reaction with clinitest is:
A. Cystinuria B. Alkaptonuria C. Indicanuria D. Porphyrinuria |
| 10.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 |
| 11.Ketonuria in a newborn is an indication of:
A. MSUD B. Isovaleric acidemia C. Methylmalonic acidemia D. All of the above |
| 12.Urine from a newborn with MSUD will have a significant:
A. Pale color B. Yellow precipitate C. Milky appearance D. Sweet odor |
| 13.A substance that reacts with p-nitroaniline is:
A. Isovaleric acid B. Propionic acid C. Methylmalonic acid D. Indican |
| 14.Which of the following has a significant odor?
A. Isovaleric acidemia B. Sweet odor Propionic acidemia C. Methylmalonic acidemia D. Indicanuria |
| 15.Hartnup disease is a disorder associated with the metabolism of:
A. Organic acids B. Tryptophan C. Cystine D. Phenylalanine |
| 16.5-HIAA is a degradation product of:
A. Heme B. Indole C. Serotonin D. Melanin |
| 17.Elevated urinary levels of 5-HIAA are associated with:
A. Carcinoid tumors B. Hartnup disease C. Cystinuria D. Platelet disorders |
| 18.False-positive levels of 5-HIAA can be caused by a diet high in:
A. Meat B. Carbohydrates C. Starch D. Bananas |
| 19.Place the appropriate letter in front of the following statements.
A. Cystinuria B. Cystinosis ____IEM ____Inherited disorder of renal tubules ____Fanconi syndrome ____Cystine deposits in the cornea ____Early renal calculi formation |
| 20.Urine from patients with cystine disorders will react with:
A. Dinitrophenylhydrazine B. Sodium cyanide C. Ehrlich reagent D. 1-Nitroso-napthol |
| 21.Blue diaper syndrome is associated with:
A. Lesch-Nyhan syndrome B. Phenylketonuria C. Cystinuria D. Hartnup disease |
| 22.Homocystinuria is caused by failure to metabolize:
A. Lysine B. Methionine C. Arginine D. Cystine |
| 23.Early detection is most valuable for correction of:
A. Homocystinuria B. Cystinuria C. Indicanuria D. Porphyrinuria |
| 24.The Ehrlich reaction will only detect the presence of:
A. Aminolevulinic acid B. Porphobilinogen C. Coproporphyrin D. Protoporphyrin |
| 25.Acetylacetone is added to the urine prior to performing the Ehrlich test when checking for:
A. Aminolevulinic acid B. Porphobilinogen C. Uroporphyrin D. Coproporphyrin |
| 26.The classic urine color associated with porphyria is:
A. Dark yellow B. Indigo blue C. Pink D. Port wine |
| 27.Which of the following specimens can be used for porphyrin testing?
A. Urine B. Blood C. Feces D. All of the above |
| 28.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 |
| 29.Hurler, Hunter, and Sanfilippo syndromes are hereditary disorders affecting metabolism of:
A. Porphyrins B. Purines C. Mucopolysaccharides D. Tryptophan |
| 30.Many uric acid crystals in a pediatric urine specimen may indicate:
A. Hurler syndrome B. Lesch-Nyhan disease C. Melituria D. Sanfilippo syndrome |
| 31.Deficiency of the GALT enzyme will produce a:
A. Positive Clinitest B. Glycosuria C. Galactosemia D. Both A and C |
| 32.Match the metabolic urine disorders with their classic urine abonormalities.
____PKU A. Sulfur odor ____Indicanuria B. Sweaty feet odor ____Cystinuria C. Orange sand in diaper ____Homogentisic acid D. Mousy odor ____Lesch-Nyhan disease E. Black color F. Blue color |
