Question 1

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. B

Question 2

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 A

Question 3

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 B

Question 4

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 5

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 6

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

Question 7

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

Question 8

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

Question 9

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 10

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.

Question 11

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 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 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

Question 14

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 15

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 16

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 17

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 18

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 19

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 20

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

Exam 44: Medical and Nutrition Therapy for Genetic Metabolic Disorders

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

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

Which fatty acid oxidation disorder is the most common?

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

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

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 metabolic disorders is NOT classified as an organic acidemia?

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 nutritional treatments that would be provided to patients with metabolic disorders?

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

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

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

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

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.

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

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

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

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

Digestion, Absorption, Transport, and Excretion of Nutrients

Energy

The Nutrients and Their Metabolism

Intake: Analysis of the Diet

Assessment: Nutritional Genomics

Clinical: Inflammation, Physical and Functional Assessment

Water, Electrolytes, and Acid-Base Balance

Clinical: Biochemical Assessment

Clinical: Food-Drug Interactions

Behavioral-Environmental: The Individual in the Community

Overview of Nutrition Diagnosis and Intervention

Food-Nutrient Delivery: Planning the Diet With Cultural Competency

Food and Nutrient Delivery: Bioactive Substances and Integrative Care

Food-Nutrient Delivery: Nutrition Support Methods

Interventions: Education and Counseling for Behavior Change

Nutrition in Pregnancy and Lactation

Nutrition During Infancy

Nutrition in Childhood

Nutrition in Adolescence

Nutrition in the Adult Years

Nutrition in Aging

Weight Management

Nutrition in Eating Disorders

Nutrition in Exercise and Sports Performance

Nutrition and Bone Health

Nutrition for Oral and Dental Health

Mnt for Food Allergy and Food Intolerance

Medical Nutrition Therapy for Upper Gastrointestinal Tract Disorders

Medical Nutrition Therapy for Lower Gastrointestinal Tract Disorders

Medical Nutrition Therapy for Liver, Biliary System, and Exocrine Pancreas Disorders

Medical Nutrition Therapy for Diabetes Mellitus and Hypoglycemia of Nondiabetic Origin

Medical Nutrition Therapy for Thyroid and Related Disorders

Medical Nutrition Therapy for Anemia

Medical Nutrition Therapy for Cardiovascular Disease

Medical Nutrition Therapy for Pulmonary Disease

Medical and Nutrition Therapy for Renal Disorders

Medical and Nutrition Therapy for Cancer

Medical and Nutrition Therapy for HIV and AIDS

Medical and Nutrition Therapy for Metabolic Stress: Sepsis, Trauma, Burns, and Surgery

Medical and Nutrition Therapy for Rheumatic Disease

Medical and Nutrition Therapy for Neurologic Disorders

Medical and Nutrition Therapy for Psychiatric Conditions

Medical and Nutrition Therapy for Low-Birth-Weight Infants

Medical and Nutrition Therapy for Developmental Disabilities

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