Question 1

Sapropterin dihydrochloride is an oral formulation of the biologically active tetrahydrobiopterin, cofactor to the _______________ enzyme.

Accepted Answer

phenylalanine hydrox

Question 2

Impaired metabolism of nutrients can be caused by a defective gene that results in a change to the binding site of cofactors.

Accepted Answer

A) True 
 B)False

Question 3

Which of the following statements is true of the amino acid disorder homocystinuria?

Accepted Answer

A)  Cysteine is not converted to homocysteine. 
B)  Homocysteine is not converted to cysteine. 
C)  Homocysteine is not converted to lysine. 
D)  Homocysteine is not converted to leucine. 
E)  Cysteine is not converted to valine. 
A)  Cysteine is not converted to homocysteine. 
B)  Homocysteine is not converted to cysteine. 
C)  Homocysteine is not converted to lysine. 
D)  Homocysteine is not converted to leucine. 
E)  Cysteine is not converted to valine.

Question 4

Which amino acid is affected in the metabolic disorder isovaleric acidemia?

Accepted Answer

A)  valine 
B)  tryptophan 
C)  leucine 
D)  isoleucine 
E)  arginine 
A)  valine 
B)  tryptophan 
C)  leucine 
D)  isoleucine 
E)  arginine

Question 5

The genetic defect in phenylalanine hydroxylase leads to a reduction in or an absence of phenylalanine hydroxylase activity.

Accepted Answer

A) True 
 B)False

Question 6

Glycogen storage disease type I (GSD I) is characterized by a deficiency of the enzyme _______________.

Accepted Answer

glucose-6-

Question 7

Describe the use and benefits of sapropterin dihydrochloride.

Accepted Answer

Sapropterin dihydrochloride is an oral f

Question 8

What type of fatty acids are linoleic and linolenic acids?

Accepted Answer

A)  short-chain 
B)  medium-chain 
C)  long-chain 
D)  very long-chain 
E)  polysaccharide 
A)  short-chain 
B)  medium-chain 
C)  long-chain 
D)  very long-chain 
E)  polysaccharide

Question 9

The urea cycle resides primarily in the _______________, but this process can also take place to a lesser extent in the kidneys and small intestine.

Accepted Answer

The answer of The urea cycle resides primarily in the...

Question 10

How many enzymes are needed to convert fructose into intermediates of the glycolytic gluconeogenic pathway?

Accepted Answer

A)  one 
B)  two 
C)  three 
D)  four 
E)  five 
A)  one 
B)  two 
C)  three 
D)  four 
E)  five

Question 11

Mitochondria are essential for the production of _______________ in all types of tissues.

Accepted Answer

The answer of Mitochondria are essential for the production of...

Question 12

Galactose is a monosaccharide found in milk products; galactosemia may be rapidly fatal in infants if milk feedings are not discontinued.

Accepted Answer

A) True 
 B)False

Question 13

Carnitine is usually supplemented if the amount of free carnitine is _______________ and/or the ratio of esterified to free carnitine is _______________.

Accepted Answer

The answer of Carnitine is usually supplemented if the amount...

Question 14

Additional amounts of a cofactor can provide for a chaperoning effect where the cofactor helps to stabilize the enzyme complex, allowing for an increase in enzyme activity.

Accepted Answer

A) True 
 B)False

Question 15

The deficiency or absence of the phenylalanine _______________ enzyme leads to the inability to convert the essential amino acid phenylalanine into the amino acid _______________.

Accepted Answer

hydroxylas

Question 16

Which of these is a predominant dietary source of galactose?

Accepted Answer

A)  fructose from fruit 
B)  lactose from milk 
C)  glucose from honey 
D)  glycogen from meat 
E)  fiber from vegetables 
A)  fructose from fruit 
B)  lactose from milk 
C)  glucose from honey 
D)  glycogen from meat 
E)  fiber from vegetables

Question 17

Describe supplementation of end products as an approach for chronic management of metabolic disorders, and provide a specific example.

Accepted Answer

Substrates that should have been formed

Question 18

PD is an 11-month-old boy with phenylketonuria (PKU). His mother brought him to an outpatient nutrition center in the local pediatric hospital for evaluation.
Length: 28 inches Weight: 19 pounds (8.7 kg)
-What are PD's daily protein needs?

Accepted Answer

A)  32 g 
B)  31.4 g 
C)  30 g 
D)  28.3 g 
E)  24 g 
A)  32 g 
B)  31.4 g 
C)  30 g 
D)  28.3 g 
E)  24 g

Question 19

Which type of glycogen storage disease causes a muscle phosphorylase deficiency?

Accepted Answer

A)  type II 
B)  type III 
C)  type IV 
D)  type V 
E)  type VI 
A)  type II 
B)  type III 
C)  type IV 
D)  type V 
E)  type VI

Question 20

Which amino acid is affected in the metabolic disorder 3-hydroxyisobutyric aciduria?

Accepted Answer

A)  valine 
B)  tryptophan 
C)  leucine 
D)  isoleucine 
E)  arginine 
A)  valine 
B)  tryptophan 
C)  leucine 
D)  isoleucine 
E)  arginine

Exam 25: Metabolic Disorders

Sapropterin dihydrochloride is an oral formulation of the biologically active tetrahydrobiopterin, cofactor to the _______________ enzyme.

Impaired metabolism of nutrients can be caused by a defective gene that results in a change to the binding site of cofactors.

Which of the following statements is true of the amino acid disorder homocystinuria?

Which amino acid is affected in the metabolic disorder isovaleric acidemia?

The genetic defect in phenylalanine hydroxylase leads to a reduction in or an absence of phenylalanine hydroxylase activity.

Glycogen storage disease type I (GSD I) is characterized by a deficiency of the enzyme _______________.

Describe the use and benefits of sapropterin dihydrochloride.

What type of fatty acids are linoleic and linolenic acids?

The urea cycle resides primarily in the _______________, but this process can also take place to a lesser extent in the kidneys and small intestine.

How many enzymes are needed to convert fructose into intermediates of the glycolytic gluconeogenic pathway?

Mitochondria are essential for the production of _______________ in all types of tissues.

Galactose is a monosaccharide found in milk products; galactosemia may be rapidly fatal in infants if milk feedings are not discontinued.

Carnitine is usually supplemented if the amount of free carnitine is _______________ and/or the ratio of esterified to free carnitine is _______________.

Additional amounts of a cofactor can provide for a chaperoning effect where the cofactor helps to stabilize the enzyme complex, allowing for an increase in enzyme activity.

The deficiency or absence of the phenylalanine _______________ enzyme leads to the inability to convert the essential amino acid phenylalanine into the amino acid _______________.

Which of these is a predominant dietary source of galactose?

Describe supplementation of end products as an approach for chronic management of metabolic disorders, and provide a specific example.

PD is an 11-month-old boy with phenylketonuria (PKU). His mother brought him to an outpatient nutrition center in the local pediatric hospital for evaluation. Length: 28 inches Weight: 19 pounds (8.7 kg) -What are PD's daily protein needs?

Which type of glycogen storage disease causes a muscle phosphorylase deficiency?

Which amino acid is affected in the metabolic disorder 3-hydroxyisobutyric aciduria?

Role of the Registered Dietitian Nutritionist in the Health Care System

Overview the Nutrition Care Process

Nutrition Assessment Foundation of the Nutrition Care Process

Nutrition Intervention Nutrition Monitoring and Evaluation

Enteral and Parenteral Nutrition Support

Nutrition Informatics and Documentation of the Nutrition Care Process

Fluid and Electrolyte Balance

Acid Base Balance

Cellular and Physiological Response to Injury the Role of the Immune System

Nutritional Genomics

Pharmacology

Diseases and Disorders of Energy Imbalance

Diseases of the Cardiovascular System

Diseases of the Upper Gastrointestinal Tract

Diseases of the Lower Gastrointestinal Tract

Diseases of the Liver Gallbladder and Exocrine Pancreas

Diseases of the Endocrine System

Diseases of the Renal System

Diseases of the Hematological System

Diseases and Disorders of the Neurological System

Diseases of the Respiratory System

Metabolic Stress and the Critically Ill

Neoplastic Disease

Diseases of the Musculoskeletal System

Filters

Carnitine is usually supplemented if the amount of free carnitine is _ and/or the ratio of esterified to free carnitine is _.

The deficiency or absence of the phenylalanine _ enzyme leads to the inability to convert the essential amino acid phenylalanine into the amino acid _.