Cytosolic glycogen accumulation does not occur in
- A. Pompe disease
- B. Mc Ardle's disease
- C. Phosphofructokinase deficiency
- D. Phosphoglycerate kinase deficiency
Correct Answer: D
Rationale: Phosphoglycerate kinase deficiency does not lead to cytosolic glycogen accumulation.
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Which of the following is a neurocutaneous syndrome without cutaneous manifestations?
- A. Sturge-Weber syndrome
- B. Tuberous sclerosis
- C. Ataxia telangiectasia
- D. Von Hippel-Lindau disease
Correct Answer: D
Rationale: Von Hippel-Lindau disease is a neurocutaneous syndrome that primarily affects internal organs and often lacks cutaneous manifestations.
A client with a productive cough has obtained a sputum specimen for culture as instructed. What is the best initial nursing action?
- A. Administer the first dose of antibiotic therapy
- B. Observe the color, consistency, and amount of sputum
- C. Encourage the client to consume plenty of warm liquids
- D. Send the specimen to the lab for analysis
Correct Answer: B
Rationale: Observing the color, consistency, and amount of sputum helps in assessing the nature of the infection and guides further treatment. Sending the specimen to the lab is important but not the first action.
Family discharge teaching has been effective when the parent of a toddler diagnosed with Kawasaki disease (KD) states:
- A. The arthritis in her knees is permanent. She will need knee replacements.
- B. I will give her diphenhydramine (Benadryl) for her peeling palms and soles of her feet.
- C. I know she will be irritable for 2 months after her symptoms start.
- D. I will continue with high doses of Tylenol for her inflammation.
Correct Answer: C
Rationale: This statement indicates the parent understands that irritability may persist for about 2 months after symptom onset in KD.
Common causes of seizures in a neonate are:
- A. Hypocalcaemia
- B. Hypoglycaemia
- C. Pyridoxine deficiency
- D. Hydrocephalus
Correct Answer: B
Rationale: The correct answer is B because hypoglycaemia is a common cause of neonatal seizures. The other options (a, c, d, e) are also causes but less common.
A 12-year-old male tries out for a middle school hockey team. He has a history of a heart murmur as an infant, but the doctor thought it would go away. During the tryout, he experiences severe dyspnea and becomes light-headed. At your office, he has a normal rhythm, pulse, and blood pressure and is no longer dizzy. There is a grade 4/6 systole ejection murmur that radiates to the neck. There is also an ejection click. An ECG reveals left ventricular hypertrophy. The next approach to his management includes
- A. chest x-ray
- B. exercise test
- C. digitalization
- D. echocardiography
Correct Answer: D
Rationale: Echocardiography is essential to evaluate the severity of aortic stenosis and its effects on the heart.