Nokea
Which of the following characteristics are similar with respect to Factor VIII and von Willebrand factor (vWF)?
- A. Both are made in endothelial cells and megakaryocytes.
- B. Both are activated by thrombin.
- C. They are present in normal to high relative amounts in newborns.
- D. They are stored in Weibel-Palade bodies in endothelial cells.
Correct Answer: C
Rationale: Rationale:
- Factor VIII and vWF are present in normal to high amounts in newborns due to the physiological immaturity of the hemostatic system.
- Choice A is incorrect as vWF is mainly produced in endothelial cells while Factor VIII is produced in both endothelial cells and liver.
- Choice B is incorrect as Factor VIII is activated by thrombin, but vWF is not.
- Choice D is incorrect as vWF is stored in Weibel-Palade bodies, but Factor VIII is not.
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A 6-year-old boy is noted to have worsening visual acuity on serial eye exams. Recent MRI of his brain and orbits reveals an optic chiasm mass, most consistent with an optic pathway glioma. Upon further examination, the physician notes axillary freckling and numerous “spots†on the patient's skin. This patient most likely has a which of the following genetic disorders?
- A. Neurofibromatosis type-1 (NF1)
- B. Li Fraumeni syndrome
- C. Tuberous sclerosis
- D. Cystic Fibrosis
Correct Answer: A
Rationale: The correct answer is A: Neurofibromatosis type-1 (NF1). This is because the patient presents with optic pathway glioma, axillary freckling, and numerous skin spots, which are classic features of NF1. NF1 is associated with multiple neurocutaneous manifestations, including café-au-lait spots, neurofibromas, and optic pathway gliomas. Li Fraumeni syndrome (B) is a cancer predisposition syndrome characterized by a high risk of developing various cancers, not typically associated with optic pathway gliomas or skin manifestations. Tuberous sclerosis (C) presents with features like facial angiofibromas, cortical tubers, and cardiac rhabdomyomas, not axillary freckling or optic pathway gliomas. Cystic Fibrosis (D) is a genetic disorder affecting the lungs and digestive system, not associated with the symptoms described in the patient.
In a study to investigate the rates of central line–acquired bacterial infections, it is discovered that patient length of stay (LOS) is not normally distributed but is highly right-skewed. What is the correct relationship between the mean, median, and mode of LOS?
- A. The mean is less than the median but greater than the mode.
- B. The mean is equal to the median and the mode.
- C. The mean is greater than the median and mode.
- D. The mean and median will both be less than the mode.
Correct Answer: C
Rationale: The correct answer is C: The mean is greater than the median and mode. In a highly right-skewed distribution, the mean is pulled towards the higher end by the extreme values, making it greater than the median, which is the middle value. The mode is the most frequent value, which is typically lower than the mean in a right-skewed distribution. In summary, the mean is influenced by extreme values, causing it to be greater than both the median and the mode in a highly right-skewed distribution.
You receive a phone call from a community pediatrician who is caring for a 2-year-old toddler with a cancer predisposition syndrome. The pediatrician describes a child at the 95th percentile for height and weight with a history of corrective oral surgery to reduce a large tongue and a history of an omphalocele in infancy. The pediatrician is currently performing ultrasound of the abdomen and laboratory evaluation for this patient every 3 months. Which tumor is this patient most at risk of developing?
- A. Pleuropulmonary blastoma
- B. Hepatocellular carcinoma
- C. Cystic nephroma
- D. Nephroblastoma
Correct Answer: D
Rationale: The correct answer is D: Nephroblastoma. This patient likely has Beckwith-Wiedemann syndrome based on the large tongue (macroglossia) and omphalocele. Beckwith-Wiedemann syndrome is associated with an increased risk of developing nephroblastoma (Wilms tumor). The 95th percentile for height and weight is also a common feature of this syndrome. Pleuropulmonary blastoma (choice A) is more commonly seen in patients with DICER1 mutations. Hepatocellular carcinoma (choice B) is not typically associated with Beckwith-Wiedemann syndrome. Cystic nephroma (choice C) is more commonly seen in patients with DICER1 mutations, not Beckwith-Wiedemann syndrome.
A 16-year-old patient with a left-side pelvic osteosarcoma is taking extended release oxycodone twice daily as well as immediate release oxycodone for breakthrough pain approximately 2 or 3 times per day. She describes her pain as burning, tingling, and shooting in her left leg. Her pain worsens with hot showers. Her most recent EKG has a QTc of 495. What would be the best strategy to manage her pain?
- A. Switch from long-acting oxycodone to methadone.
- B. Recommend more frequent use of her immediate release oxycodone.
- C. Add amitriptyline daily. Start low and titrate upward on dosage.
- D. Add gabapentin three times daily. Start low and titrate upward on dose.
Correct Answer: D
Rationale: Step 1: The patient's pain is described as burning, tingling, and shooting in her left leg, which suggests neuropathic pain.
Step 2: The pain worsens with hot showers, indicating sensitivity to temperature changes, a common feature of neuropathic pain.
Step 3: Gabapentin is a first-line medication for neuropathic pain, as it stabilizes nerve cells and reduces abnormal pain signaling.
Step 4: Starting low and titrating upward on gabapentin dose helps minimize side effects and optimize pain relief.
Step 5: Methadone (Choice A) is not the best option for neuropathic pain. Increasing immediate release oxycodone (Choice B) may lead to opioid tolerance. Amitriptyline (Choice C) may help neuropathic pain but is not as specific as gabapentin.
A patient who is undergoing consolidation therapy for the treatment of leukemia has been experiencing debilitating fatigue. How can the nurse best meet this patient's needs for physical activity?
- A. Teach the patient about the risks of immobility and the benefits of exercise.
- B. Assist the patient to a chair during awake times, as tolerated.
- C. Collaborate with the physical therapist to arrange for stair exercises.
- D. Teach the patient to perform deep breathing and coughing exercises.
Correct Answer: B
Rationale: The correct answer is B because assisting the patient to a chair during awake times, as tolerated, is the most appropriate intervention for a patient experiencing debilitating fatigue during consolidation therapy for leukemia. This option promotes mobility and prevents further deconditioning without overwhelming the patient.
A: Teaching about risks and benefits may not directly address the immediate need for physical activity.
C: Stair exercises may be too strenuous for a fatigued patient and could potentially exacerbate their condition.
D: Deep breathing and coughing exercises are important but do not directly address the need for physical activity to combat fatigue.