Nokea
A 3-month-old infant presents with poor feeding and lethargy. Labs show hypoglycemia and lactic acidosis. Most likely diagnosis is:
- A. Sepsis
- B. Glycogen storage disease
- C. Galactosemia
- D. Fatty acid oxidation disorder
Correct Answer: D
Rationale: FAOD causes hypoglycemia and lactic acidosis, per NORD. A-C, E differ.
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A nurse is providing parental instruction for a 24-hour urine collection for a hospitalized child. Which of the following statements by the child's parent needs further instruction?
- A. This test is to evaluate kidney function.'
- B. This test is timed with the first urine collection.'
- C. This container can be kept in the unit refrigerator.'
- D. The container comes from the laboratory.'
Correct Answer: C
Rationale: Urine collection containers should be kept cool but not in a shared refrigerator due to contamination risks; a specific cooler is used, indicating a need for clarification.
A child with leukemia is prone to
- A. Bleeding
- B. Infection
- C. Anemia
- D. All of the above
Correct Answer: D
Rationale: All D result from leukemia, per document p57, 7.
A 3-month-old infant presents with tachypnea and poor feeding. Chest x-ray shows cardiomegaly and pulmonary edema. Most likely diagnosis is:
- A. Pneumonia
- B. Congestive heart failure
- C. Bronchiolitis
- D. Cystic fibrosis
Correct Answer: B
Rationale: CHF shows cardiomegaly and edema, per AHA. A, C-E differ.
What is the risk the fetus will be affected?
- A. 1 in 2
- B. 1 in 4
- C. 1 in 50
- D. 1 in 200
Correct Answer: B
Rationale: Spinal muscular atrophy (SMA) type 2 is autosomal recessive. If the mother’s new partner is a carrier (assuming population risk isn’t specified), the risk is 1 in 4 (Choice B): 25% chance the fetus inherits two mutated SMN1 alleles (50% from each carrier parent). 1 in 2 (Choice A) applies if one parent is affected. 1 in 50 (Choice C) or 1 in 200 (Choice D) suggest unrelated carrier frequencies, not applicable without data. 1 in 4 is standard for recessive inheritance with two carriers.
A physician should be informed if a 7-year-old with a fractured femur in a cast shows
- A. Cast damp and warm after 24 hours
- B. Increased urinary output
- C. Inability to move toes
- D. Pedal pulse of 90 bpm
Correct Answer: C
Rationale: Inability to move toes C suggests circulation issues, per document p11, .