Question 1

For best utilization of synthetic amino acids, one should:

Accepted Answer

A)  consume large quantities 1 to 2 times per day.
B)  consume smaller quantities 4 to 5 times per day.
C)  consume 1 large quantity right before sleeping.
D)  consume 1 large quantity right upon awakening. B

Question 2

Which of the following urea cycle enzyme deficiencies is inherited as an X-linked dominant trait that is usually lethal in males?

Accepted Answer

A)  argininosuccinic acid synthase
B)  carbamyl phosphate synthase I
C)  ornithine transcarbamylase
D)  arginase C

Question 3

A block in the urea cycle can result from a deficiency in any of these enzymes except:

Accepted Answer

A)  glucokinase.
B)  carbamyl phosphate synthetase I.
C)  arginase.
D)  argininosuccinic acid lyase. A

Question 4

Case Study
GF is a 10-month-old infant with a urea cycle disorder.GF is admitted into the hospital due to a hyperammonemic crisis.
LT: 25.5 inches     WT: 16.5 #
-After the first 24-48 hours, the registered dietitian is going to prescribe PN He/she should work with the pharmacy to ensure that the PN solution is high in:

Accepted Answer

A)  aromatic amino acids.
B)  branched-chain amino acids.
C)  tyrosine.
D)  omega-3 fatty acids.

Question 5

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital.
LT: 28 inches        WT: 8.7 kg
-Should PD's mom not be compliant with her diet, PD could potentially develop all of the following except:

Accepted Answer

A)  mental retardation.
B)  seizures.
C)  tyrosine deficiency.
D)  eczema.

Question 6

Treatment for GSD I involves administering 1 to 2 grams/kg _____ every 3 to 6 hours to help maintain glucose levels

Accepted Answer

A)  syrup
B)  cornstarch
C)  juice
D)  Gatorade

Question 7

Which of the following is one of the appropriate dietary interventions for GSD I?

Accepted Answer

A)  avoidance of fructose and lactose
B)  limiting amino acid intake
C)  increasing fat
D)  decreasing complex carbohydrates

Question 8

The main nutrient intervention for a fat metabolism disorder is to:

Accepted Answer

A)  prevent fasting.
B)  increase intake of fatty acids.
C)  continue with a liberal diet.
D)  consume complex carbohydrates.

Question 9

Those with fructose intolerance should avoid which of the following sugars?

Accepted Answer

A)  fructose, sucrose, and sorbitol
B)  fructose, glucose, and malitol
C)  frucotse, sucrose, and zylitol
D)  fructose, raffinose, and glucose

Question 10

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital.
LT: 28 inches        WT: 8.7 kg
-Calculate PD's daily fluid needs

Accepted Answer

A)  1392 mL
B)  1262 mL
C)  1175 mL
D)  1255 mL

Question 11

Which of the following is not a result of untreated PKU?

Accepted Answer

A)  mental retardation
B)  seizures
C)  eczema
D)  obesity

Question 12

In order for individuals with PKU to obtain the necessary amount of protein that they need to promote adequate growth, they are required to:

Accepted Answer

A)  supplement their diet with capsules containing all the amino acids.
B)  consume a protein supplement containing at least 50 grams of protein a day.
C)  consume a protein supplement containing all the amino acids except for phenylalanine.
D)  consume meats and beans at least twice a day.

Question 13

Which of the following nutrients are individuals with galactosemia most likely to be deficient in?

Accepted Answer

A)  calcium and vitamin D
B)  phosphorus and vitamin A
C)  vitamin D and phosphorous
D)  magnesium and calcium

Question 14

Urea cycle disorders result from an impaired capacity of the body to excrete _____ in the form of urea

Accepted Answer

A)  carbon
B)  nitrogen
C)  organic acids
D)  uric acid

Question 15

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital.
LT: 28 inches        WT: 8.7 kg
-Which of the following is the highest amount (mg) of phenylalanine that PD should consume during any single day?

Accepted Answer

A)  304.5 mg
B)  87 mg
C)  391.5 mg
D)  609 mg

Question 16

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital.
LT: 28 inches        WT: 8.7 kg
-Calculate PD's daily protein needs

Accepted Answer

A)  27.8 g
B)  26.1 g
C)  30.45 g
D)  25 g

Question 17

In glycogen storage disease type I, the enzyme block results in an inability to produce:

Accepted Answer

A)  glycogen.
B)  galactose.
C)  glucose.
D)  fructose.

Question 18

Match between columns
                        Premises: genotype
encephalopathy
glycolysis
pyruvate complex disorders
cofactors
rhabdomyolysis
oligosaccharide
ataxia
tetrahydrobiopterin
intralipid
metabolic decompensation
gluconeogenesis
euglycemia
phenotype
ectopia lentis
ketosis
steatosis
retinopathies
periorificial acrodermatitis
tandem mass spectroscopy
glycogenolysis

Accepted Answer

The Answer of disease of the skin surrounding the mouth is...

Question 19

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital.
LT: 28 inches        WT: 8.7 kg
-Calculate PD's daily kcalorie needs

Accepted Answer

A)  1205 kcal
B)  914 kcal
C)  957 kcal
D)  1044 kcal

Question 20

Those with glycogen storage disease are at highest risk of dying from:

Accepted Answer

A)  failure to thrive.
B)  renal failure.
C)  liver adenma.
D)  hypoglycemia.

Exam 26: Metabolic Disorders

For best utilization of synthetic amino acids, one should:

Which of the following urea cycle enzyme deficiencies is inherited as an X-linked dominant trait that is usually lethal in males?

A block in the urea cycle can result from a deficiency in any of these enzymes except:

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital. LT: 28 inches WT: 8.7 kg -Should PD's mom not be compliant with her diet, PD could potentially develop all of the following except:

Treatment for GSD I involves administering 1 to 2 grams/kg _____ every 3 to 6 hours to help maintain glucose levels

Which of the following is one of the appropriate dietary interventions for GSD I?

The main nutrient intervention for a fat metabolism disorder is to:

Those with fructose intolerance should avoid which of the following sugars?

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital. LT: 28 inches WT: 8.7 kg -Calculate PD's daily fluid needs

Which of the following is not a result of untreated PKU?

In order for individuals with PKU to obtain the necessary amount of protein that they need to promote adequate growth, they are required to:

Which of the following nutrients are individuals with galactosemia most likely to be deficient in?

Urea cycle disorders result from an impaired capacity of the body to excrete _____ in the form of urea

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital. LT: 28 inches WT: 8.7 kg -Which of the following is the highest amount (mg) of phenylalanine that PD should consume during any single day?

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital. LT: 28 inches WT: 8.7 kg -Calculate PD's daily protein needs

In glycogen storage disease type I, the enzyme block results in an inability to produce:

Matching

PD is an 11-month-old M with phenylketonuria.His mother brought him in to visit you at an outpatient nutrition center in the local pediatric hospital. LT: 28 inches WT: 8.7 kg -Calculate PD's daily kcalorie needs

Those with glycogen storage disease are at highest risk of dying from: