Question 1

What is the insoluble component of starch?​

Accepted Answer

A)  ​carnitine 
B)  ​glucagon 
C)  ​raffinose 
D)  ​ribose 
E)  amylopectin 
A)  ​carnitine 
B)  ​glucagon 
C)  ​raffinose 
D)  ​ribose 
E)  amylopectin

Question 2

What is a typical sign of an inborn error of metabolism that mothers quickly pick up on?​

Accepted Answer

A)  ​baby smells funny 
B)  ​baby won't wake up 
C)  ​baby bleeds from the ears 
D)  ​baby yawns uncontrollably 
E)  ​baby makes high-pitched screeches 
A)  ​baby smells funny 
B)  ​baby won't wake up 
C)  ​baby bleeds from the ears 
D)  ​baby yawns uncontrollably 
E)  ​baby makes high-pitched screeches

Question 3

Phe restriction should achieve optimal blood Phe levels of:

Accepted Answer

A)  ​20-30 μmol/L 
B)  ​65-105 μmol/L 
C)  ​120-360 μmol/L 
D)  ​400-600 μmol/L 
E)  ​650-820 μmol/L 
A)  ​20-30 μmol/L 
B)  ​65-105 μmol/L 
C)  ​120-360 μmol/L 
D)  ​400-600 μmol/L 
E)  ​650-820 μmol/L

Question 4

What are PD's daily protein needs?

Accepted Answer

A)  ​about 32 g 
B)  ​30.6 g 
C)  ​about 29 g 
D)  ​27.8 g 
E)  ​about 24 g 
A)  ​about 32 g 
B)  ​30.6 g 
C)  ​about 29 g 
D)  ​27.8 g 
E)  ​about 24 g

Question 5

Organic acidemias are a subset of _______________ disorders that involve a dysfunction in the metabolism of intermediary products.

Accepted Answer

The answer of Organic acidemias are a subset of _______________...

Question 6

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

​hydroxyla

Question 7

What is the affected amino acid in the metabolic condition homocystinuria?​

Accepted Answer

A)  ​methionine 
B)  ​leucine 
C)  ​isoleucine 
D)  ​ phenylalanine 
E)  ​cysteine 
A)  ​methionine 
B)  ​leucine 
C)  ​isoleucine 
D)  ​ phenylalanine 
E)  ​cysteine

Question 8

What type of vegetable oil contains the most linoleic acid?

Accepted Answer

A)  ​flaxseed 
B)  ​safflower 
C)  ​corn 
D)  ​peanut 
E)  ​olive 
A)  ​flaxseed 
B)  ​safflower 
C)  ​corn 
D)  ​peanut 
E)  ​olive

Question 9

Isovaleric acidemia (IVA) is an amino acid disorder affecting the metabolism of multiple amino acids.

Accepted Answer

A) True 
 B)False

Question 10

What is the 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 11

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 12

What is the targeted blood Phe range, as recommended in the new ACMG guidelines, for all individuals with PKU?​

Accepted Answer

A)  ​20-30 μmol/L 
B)  ​65-105 μmol/L 
C)  ​120-360 μmol/L 
D)  ​400-600 μmol/L 
E)  ​650-820 μmol/L 
A)  ​20-30 μmol/L 
B)  ​65-105 μmol/L 
C)  ​120-360 μmol/L 
D)  ​400-600 μmol/L 
E)  ​650-820 μmol/L

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

A pharmacologic dose of vitamin B12 is given to a newly diagnosed patient with _______________ acidemia to determine whether the enzymatic defect is vitamin responsive.

Accepted Answer

The answer of A pharmacologic dose of vitamin B12 is...

Question 15

In patients with galactosemia, the enzyme defect occurs in uridyltransferase, which normally converts galactose, in the form of galactose-1-phosphate, to _______________ -1-phosphate.

Accepted Answer

The answer of In patients with galactosemia, the enzyme defect...

Question 16

Tandem _______________ allows clinicians to screen for over 30 metabolic disorders by analyzing metabolites in a blood spot collected on a filter paper from a simple heel prick on the newborn.

Accepted Answer

The answer of Tandem _______________ allows clinicians to screen for...

Question 17

Additional amounts of 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 18

What is a substrate needed for the normal metabolism of fat for energy?​

Accepted Answer

A)  ​carnitine 
B)  ​glucagon 
C)  ​raffinose 
D)  ​ ribose 
E)  ​ amylopectin 
A)  ​carnitine 
B)  ​glucagon 
C)  ​raffinose 
D)  ​ ribose 
E)  ​ amylopectin

Question 19

What is a common neurologic sign of inborn errors of metabolism in neonates?​

Accepted Answer

A)  ​seizures 
B)  ​hyperpnea 
C)  ​tachypnea 
D)  ​vomiting 
E)  ​ectopialentis 
A)  ​seizures 
B)  ​hyperpnea 
C)  ​tachypnea 
D)  ​vomiting 
E)  ​ectopialentis

Question 20

How are most inborn errors of metabolism acquired?

Accepted Answer

A)  ​autosomal dominant inheritance 
B)  ​autosomal recessive inheritance 
C)  ​ infection 
D)  ​dietary deficiency 
E)  ​blood toxicity 
A)  ​autosomal dominant inheritance 
B)  ​autosomal recessive inheritance 
C)  ​ infection 
D)  ​dietary deficiency 
E)  ​blood toxicity

Exam 26: Metabolic Disorders

What is the insoluble component of starch?​

What is a typical sign of an inborn error of metabolism that mothers quickly pick up on?​

Phe restriction should achieve optimal blood Phe levels of:

What are PD's daily protein needs?

Organic acidemias are a subset of _______________ disorders that involve a dysfunction in the metabolism of intermediary products.

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

What is the affected amino acid in the metabolic condition homocystinuria?​

What type of vegetable oil contains the most linoleic acid?

Isovaleric acidemia (IVA) is an amino acid disorder affecting the metabolism of multiple amino acids.

What is the predominant dietary source of galactose?​

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

What is the targeted blood Phe range, as recommended in the new ACMG guidelines, for all individuals with PKU?​

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

A pharmacologic dose of vitamin B12 is given to a newly diagnosed patient with _______________ acidemia to determine whether the enzymatic defect is vitamin responsive.

In patients with galactosemia, the enzyme defect occurs in uridyltransferase, which normally converts galactose, in the form of galactose-1-phosphate, to _______________ -1-phosphate.

Tandem _______________ allows clinicians to screen for over 30 metabolic disorders by analyzing metabolites in a blood spot collected on a filter paper from a simple heel prick on the newborn.

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

What is a substrate needed for the normal metabolism of fat for energy?​

What is a common neurologic sign of inborn errors of metabolism in neonates?​

How are most inborn errors of metabolism acquired?

Role of the Dietitian in the Health Care System

The Nutrition Care Process

Nutrition Assessment - Foundation of the Nutrition Care Process

Nutrition Intervention and Nutrition Monitoring and Evaluation

Enteral and Parental Nutrition Support

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 Gastrointenstinal 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

HIV and Aids

Diseases of the Musculoskeletal System

Filters

What is the insoluble component of starch?

What is a typical sign of an inborn error of metabolism that mothers quickly pick up on?

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

What is the affected amino acid in the metabolic condition homocystinuria?

What is the predominant dietary source of galactose?

What is the targeted blood Phe range, as recommended in the new ACMG guidelines, for all individuals with PKU?

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

What is a substrate needed for the normal metabolism of fat for energy?

What is a common neurologic sign of inborn errors of metabolism in neonates?