Metabolic Test 1

39 Questions | Total Attempts: 78

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Metabolic Test 1

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Questions and Answers
  • 1. 
    DNA and RNA are found in which part of the human body cell?
    • A. 

      Lysosome

    • B. 

      Cytochrome

    • C. 

      Nucleus

    • D. 

      Cell membrane

  • 2. 
    Most metabolic diseases are inherited by which genetic transmission?
    • A. 

      Autosomal dominant

    • B. 

      Autosomal recessive

    • C. 

      X-linked

    • D. 

      All the above

  • 3. 
    One reason patients with the same disease do NOT respond to nutrition therapy in a similar way is due, in part, to mutations in a defective gene.
    • A. 

      True

    • B. 

      False

  • 4. 
    Which of the following was the first metabolic disease detected by newborn screening?
    • A. 

      Diabetes

    • B. 

      Congenital hypothyroidism

    • C. 

      Conjunctivitis

    • D. 

      PKU (phenylketonuria)

  • 5. 
     “Genotype” is the observable or physical features of a person?
    • A. 

      True

    • B. 

      False

  • 6. 
    Principal goals of nutrition therapy in inborn errors include all of the following EXCEPT:
    • A. 

      Restrict substrates that may be toxic to the body

    • B. 

      Supplement the body with a product that might be deficient due to an enzyme block

    • C. 

      Limit catabolism

    • D. 

      Provide coenzymes, like vitamins and minerals, when required

    • E. 

      ALL of the above are goals of therapy

  • 7. 
    Which of the following is NOT a major factor in diet management of inborn errors:
    • A. 

      Ability to communicate

    • B. 

      Growth acceleration or retardation

    • C. 

      Tolerance to restricted substrates

    • D. 

      Presence or absence of illness

    • E. 

      Biochemistries

  • 8. 
    Name the enzyme that is deficient in classic PKU.
    • A. 

      Phenylalanine hydroxylase

    • B. 

      Phenyl-lactic acid

    • C. 

      Sulfite oxidas

  • 9. 
    Which amino acid is considered conditionally essential in PKU?
    • A. 

      Tryptophan

    • B. 

      Tyrosine

    • C. 

      Phenylalanine

  • 10. 
    Diet therapy for an affected PKU baby should begin
    • A. 

      Within 2 months of life

    • B. 

      It does not matter how soon diet therapy is initiated

    • C. 

      Within 2 weeks of life.

  • 11. 
    Principles of PKU therapy are:
    • A. 

      Prescribed amounts of PHE and tyrosine

    • B. 

      Prescribed amounts of natural protein

    • C. 

      Both of the above

  • 12. 
    Patients calculate phenylalanine intake
    • A. 

      In exchanges of 15 mg phenylalanine

    • B. 

      Every mg of phenylalanine

    • C. 

      In grams of protein

    • D. 

      All of the above is possible

  • 13. 
    Children’s (age 1-12 years) blood phenylalanine level is typically monitored
    • A. 

      Once a week

    • B. 

      Once a month

    • C. 

      Twice a month

    • D. 

      Once per year

  • 14. 
    Raised blood phenylalanine levels are due to
    • A. 

      Catabolism due to infection, accidents or surgery.

    • B. 

      Inadequate intake of PKU formula.

    • C. 

      Dietary indiscretion and food pilfering.

    • D. 

      All of the above may be the reason.

  • 15. 
    Effects of untreated PKU are among others
    • A. 

      Severe mental retardation

    • B. 

      Heart disease

    • C. 

      Liver failure

  • 16. 
    Large Neutral Amino Acids compete at the gut and blood brain barrier level
    • A. 

      True

    • B. 

      False

  • 17. 
    Which number represents most accurately the average amount of protein coming from medical food in the case of a non-PKU diet.
    • A. 

      80%

    • B. 

      50%

    • C. 

      20%

  • 18. 
    In long-chain fatty acid oxidation disorders, which clinical symptoms would you NOT see during an acute episode associated with illness or prolonged fasting?
    • A. 

      Hypoglycemia

    • B. 

      Excessive ketone production

    • C. 

      Vomiting

    • D. 

      Lethargy

  • 19. 
    XPhe Maxamum drink provides ___ g PE per tetra pak
    • A. 

      10 g PE

    • B. 

      25 g PE

    • C. 

      15 g PE

    • D. 

      40 g PE

  • 20. 
    Monogen….
    • A. 

      Contains 42% of total kcals from fat

    • B. 

      Comes in a liquid form

    • C. 

      Is supplemented with arachidonic acid and DHA

    • D. 

      Is low in vitamin K and ultratrace minerals

    • E. 

      None of the above are true

  • 21. 
    The following are some of the treatment principles for LCHADD and VLCADD EXCEPT aggressive illness management
    • A. 

      Avoid fasting

    • B. 

      Increase long chain fatty acids in the diet

    • C. 

      Supplement with medium chain fatty acids in the diet

    • D. 

      Supplement with medium chain fats to bypass enzymatic block

  • 22. 
    Sensorimotor neuropathy is a possible long-term complication for which disorder:
    • A. 

      MCADD

    • B. 

      SCADD

    • C. 

      VLCADD

    • D. 

      LCHADD

    • E. 

      Both c and d

  • 23. 
    Is Monogen indicated for MCADD diets?
    • A. 

      True

    • B. 

      False

  • 24. 
    Which of the following is NOT characteristic of a LCHADD patient’s diet?
    • A. 

      Reduce total fat

    • B. 

      Reduce LCT

    • C. 

      Reduce MCT

    • D. 

      All of the above

  • 25. 
    Which EFA source provides the best N6-N3 ratio? 
    • A. 

      Flax oil

    • B. 

      Walnut oil

    • C. 

      Soy oil