Question 1

Which of the following is NOT one of the nutritional treatments that would be provided to patients with metabolic disorders?

Accepted Answer

A)  Restrict the amount of a specific substrate (nutrient) in the diet. 
B)  Supplement the diet with a greater amount of a "safe" product of metabolism. 
C)  Replace the defective enzyme through the diet. 
D)  Supplement the defective enzyme cofactor. 
A)  Restrict the amount of a specific substrate (nutrient) in the diet. 
B)  Supplement the diet with a greater amount of a "safe" product of metabolism. 
C)  Replace the defective enzyme through the diet. 
D)  Supplement the defective enzyme cofactor. C

Question 2

Which of the following fruits and vegetables would be allowed in the diet of a person with galactosemia?

Accepted Answer

A)  Bell peppers 
B)  Watermelon 
C)  Raisins 
D)  Tomatoes 
A)  Bell peppers 
B)  Watermelon 
C)  Raisins 
D)  Tomatoes C

Question 3

Which of the following is reduced in the diet in the treatment of maple syrup urine disease (MSUD)?

Accepted Answer

A)  Fructose 
B)  Aromatic amino acids 
C)  Branched-chain amino acids 
D)  Sulfur-containing amino acids 
A)  Fructose 
B)  Aromatic amino acids 
C)  Branched-chain amino acids 
D)  Sulfur-containing amino acids C

Question 4

Which of the following foods would be allowed in the diet of a child with PKU?

Accepted Answer

A)  Peanut butter and jelly sandwich 
B)  Sugar-free gum 
C)  Potato chips 
D)  Spaghetti and meatballs 
A)  Peanut butter and jelly sandwich 
B)  Sugar-free gum 
C)  Potato chips 
D)  Spaghetti and meatballs

Question 5

For infants and children younger than 10 years of age with PKU, which amino acid has to be supplemented in the diet?

Accepted Answer

A)  Cysteine 
B)  Methionine 
C)  Phenylalanine 
D)  Tyrosine 
A)  Cysteine 
B)  Methionine 
C)  Phenylalanine 
D)  Tyrosine

Question 6

Which of the following is the deficient enzyme in glycogen storage disease Ia (GSD Ia)?

Accepted Answer

A)  Glucose-1,6-phosphatase 
B)  Fructose-1,6-diphosphatase 
C)  Fructose-1-phosphate aldolase 
D)  Hexokinase 
A)  Glucose-1,6-phosphatase 
B)  Fructose-1,6-diphosphatase 
C)  Fructose-1-phosphate aldolase 
D)  Hexokinase

Question 7

The current preferred method for screening for inborn errors of metabolism is

Accepted Answer

A)  microarray technology. 
B)  tandem mass spectrometry. 
C)  Guthrie bacterial inhibition assay. 
D)  enzyme-linked immunosorbent assay. 
A)  microarray technology. 
B)  tandem mass spectrometry. 
C)  Guthrie bacterial inhibition assay. 
D)  enzyme-linked immunosorbent assay.

Question 8

An appropriate description of a metabolic disorder is an autosomal recessive disorder that

Accepted Answer

A)  involves amino acids. 
B)  results in the reduced activity or absence of a specific enzyme. 
C)  occurs because the body cannot use dietary amino acids, fatty acids, or monosaccharides. 
D)  results in the buildup of metabolites in the blood, urine, or both. 
A)  involves amino acids. 
B)  results in the reduced activity or absence of a specific enzyme. 
C)  occurs because the body cannot use dietary amino acids, fatty acids, or monosaccharides. 
D)  results in the buildup of metabolites in the blood, urine, or both.

Question 9

Which of the following metabolic disorders is NOT a disorder of carbohydrate metabolism?

Accepted Answer

A)  Hereditary glucose intolerance 
B)  Glycogen storage disease 
C)  Galactosemia 
D)  Fructose 1,6-diphosphatase deficiency 
A)  Hereditary glucose intolerance 
B)  Glycogen storage disease 
C)  Galactosemia 
D)  Fructose 1,6-diphosphatase deficiency

Question 10

Which of the following conversions is defective in galactosemia?

Accepted Answer

A)  Glucose to galactose 
B)  Galactose to glucose 
C)  Lactose to galactose 
D)  Galactose to lactose 
A)  Glucose to galactose 
B)  Galactose to glucose 
C)  Lactose to galactose 
D)  Galactose to lactose

Question 11

The metabolic result that occurs in all of the urea cycle disorders is an accumulation of _____ in the blood.

Accepted Answer

A)  ammonia 
B)  purines 
C)  ketones 
D)  ketoacid 
A)  ammonia 
B)  purines 
C)  ketones 
D)  ketoacid

Question 12

Beyond fatty acid oxidation disorders, which other metabolic disorder is treated with supplemental carnitine?

Accepted Answer

A)  Isovaleric acidemia 
B)  Ketone utilization disorder 
C)  Ornithine transcarbamylase deficiency 
D)  Tyrosinemia 
A)  Isovaleric acidemia 
B)  Ketone utilization disorder 
C)  Ornithine transcarbamylase deficiency 
D)  Tyrosinemia

Question 13

Which of the following metabolic disorders is NOT classified as an organic acidemia?

Accepted Answer

A)  Methylmalonic metabolic disorder 
B)  Propionic metabolic disorder 
C)  Isovaleric metabolic disorder 
D)  Argininosuccinic aciduria 
A)  Methylmalonic metabolic disorder 
B)  Propionic metabolic disorder 
C)  Isovaleric metabolic disorder 
D)  Argininosuccinic aciduria

Question 14

Which of the following metabolic disorders is NOT treated with a low-protein diet?

Accepted Answer

A)  Argininemia 
B)  Methylmalonic acidemia 
C)  Ketone use disorder 
D)  Galactosemia 
A)  Argininemia 
B)  Methylmalonic acidemia 
C)  Ketone use disorder 
D)  Galactosemia

Question 15

Which fatty acid oxidation disorder is the most common?

Accepted Answer

A)  Short-chain acyl-CoA dehydrogenase deficiency 
B)  Medium-chain acyl-CoA dehydrogenase deficiency 
C)  Long-chain acyl-CoA dehydrogenase deficiency 
D)  Long-chain 3-hydroxy acyl-CoA dehydrogenase deficiency 
A)  Short-chain acyl-CoA dehydrogenase deficiency 
B)  Medium-chain acyl-CoA dehydrogenase deficiency 
C)  Long-chain acyl-CoA dehydrogenase deficiency 
D)  Long-chain 3-hydroxy acyl-CoA dehydrogenase deficiency

Question 16

Which of the following will NOT result in the development of phenylketonuria?

Accepted Answer

A)  Deficiency in phenylalanine hydroxylase 
B)  Deficiency in dihydropteridine reductase 
C)  Insufficient synthesis of biopterin 
D)  Insufficiency of dietary tyrosine 
A)  Deficiency in phenylalanine hydroxylase 
B)  Deficiency in dihydropteridine reductase 
C)  Insufficient synthesis of biopterin 
D)  Insufficiency of dietary tyrosine

Question 17

The desirable range for blood phenylalanine in a child with PKU is _____ mg/dl.

Accepted Answer

A)  2 to 6 
B)  6 to 10 
C)  8 to 12 
D)  10 to 20 
A)  2 to 6 
B)  6 to 10 
C)  8 to 12 
D)  10 to 20

Question 18

Which of the following is NOT one of the possible infant outcomes of PKU mothers with elevated phenylalanine levels?

Accepted Answer

A)  Liver dysfunction 
B)  Microcephaly 
C)  Congenital heart disease 
D)  Low birth weight 
A)  Liver dysfunction 
B)  Microcephaly 
C)  Congenital heart disease 
D)  Low birth weight

Question 19

Which of the following is used in the nutritional management of patients with glycogen storage disease?

Accepted Answer

A)  Oral intake of cornstarch 
B)  Small, frequent high-protein meals 
C)  Severe restriction of all carbohydrates 
D)  Three well-balanced meals a day 
A)  Oral intake of cornstarch 
B)  Small, frequent high-protein meals 
C)  Severe restriction of all carbohydrates 
D)  Three well-balanced meals a day

Question 20

Which of the following is NOT one of the enzyme deficiencies associated with urea cycle disorders?

Accepted Answer

A)  Ornithine transcarbamylase 
B)  HMG-CoA reductase 
C)  Carbamyl-phosphate synthetase 
D)  Arginase 
A)  Ornithine transcarbamylase 
B)  HMG-CoA reductase 
C)  Carbamyl-phosphate synthetase 
D)  Arginase

Exam 43: Medical Nutrition Therapy for Genetic Metabolic Disorders

Which of the following is NOT one of the nutritional treatments that would be provided to patients with metabolic disorders?

Which of the following fruits and vegetables would be allowed in the diet of a person with galactosemia?

Which of the following is reduced in the diet in the treatment of maple syrup urine disease (MSUD)?

Which of the following foods would be allowed in the diet of a child with PKU?

For infants and children younger than 10 years of age with PKU, which amino acid has to be supplemented in the diet?

Which of the following is the deficient enzyme in glycogen storage disease Ia (GSD Ia)?

The current preferred method for screening for inborn errors of metabolism is

An appropriate description of a metabolic disorder is an autosomal recessive disorder that

Which of the following metabolic disorders is NOT a disorder of carbohydrate metabolism?

Which of the following conversions is defective in galactosemia?

The metabolic result that occurs in all of the urea cycle disorders is an accumulation of _____ in the blood.

Beyond fatty acid oxidation disorders, which other metabolic disorder is treated with supplemental carnitine?

Which of the following metabolic disorders is NOT classified as an organic acidemia?

Which of the following metabolic disorders is NOT treated with a low-protein diet?

Which fatty acid oxidation disorder is the most common?

Which of the following will NOT result in the development of phenylketonuria?

The desirable range for blood phenylalanine in a child with PKU is _____ mg/dl.

Which of the following is NOT one of the possible infant outcomes of PKU mothers with elevated phenylalanine levels?

Which of the following is used in the nutritional management of patients with glycogen storage disease?

Which of the following is NOT one of the enzyme deficiencies associated with urea cycle disorders?

Intake: Digestion, Absorption, Transport, and Excretion of Nutrients

Intake: Energy

Inflammation and the Pathophysiology of Chronic Disease

Intake: Analysis of the Diet

Clinical: Nutritional Genomics

Clinical: Water, Electrolytes, and Acid-Base Balance

Clinical: Biochemical, Physical, and Functional Assessment

Clinical: Food-Drug Interactions

Behavioral-Environmental: the Individual in the Community

Overview of Nutrition Diagnosis and Intervention

Food and Nutrient Delivery: Diet Guidelines, Nutrient Standards, and Cultural Competence

Food and Nutrient Delivery: Complementary and Integrative Medicine and Dietary Supplementation

Food and Nutrient Delivery: Nutrition Support

Education and Counseling: Behavioral Change

Nutrition for Reproductive Health and Lactation

Nutrition in Infancy

Nutrition in Childhood

Nutrition in Adolescence

Nutrition in the Adult Years

Nutrition in Aging

Nutrition in Weight Management

Nutrition in Eating Disorders

Nutrition in Exercise and Sports Performance

Nutrition and Bone Health

Nutrition for Oral and Dental Health

Medical Nutrition Therapy for Adverse Reactions to Food: Allergies and Intolerances

Medical Nutrition Therapy for Upper Gastrointestinal Tract Disorders

Medical Nutrition Therapy for Lower Gastrointestinal Tract Disorders

Medical Nutrition Therapy for Hepatobiliary and Pancreatic Disorders

Medical Nutrition Therapy for Diabetes Mellitus and Hypoglycemia of Nondiabetic Origin

Medical Nutrition Therapy for Thyroid, Adrenal, and Other Endocrine Disorders

Medical Nutrition Therapy for Anemia

Medical Nutrition Therapy for Cardiovascular Disease

Medical Nutrition Therapy for Pulmonary Disease

Medical Nutrition Therapy for Renal Disorders

Medical Nutrition Therapy for Cancer Prevention, Treatment, and Survivorship

Medical Nutrition Therapy for Hiv and Aids

Medical Nutrition Therapy in Critical Care

Medical Nutrition Therapy for Rheumatic Disease

Medical Nutrition Therapy for Neurologic Disorders

Mnt in Psychiatric and Cognitive Disorders

Medical Nutrition Therapy for Low-Birth-Weight Infants

Medical Nutrition Therapy for Intellectual and Developmental Disabilities

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