Nokea
An 8-year-old boy undergoes a resection of a tumor in the right cerebrum next to the lateral ventricle (supratentorial). The pathologist feels the histology is most consistent with an ependymoma. The tumor is sent for advanced molecular and genetic testing. Which of the following findings would further support a diagnosis of ependymoma?
- A. BRAFKIAA1549 fusion
- B. BRAFV600E mutation
- C. RELA fusion
- D. H3K27M mutation
Correct Answer: C
Rationale: The correct answer is C: RELA fusion. Ependymomas are known to have specific molecular characteristics, with RELA fusion being a common genetic alteration associated with these tumors. The fusion of RELA gene is a key genetic event in the pathogenesis of supratentorial ependymomas. This fusion leads to the activation of the NF-κB signaling pathway, which is important in the development and progression of ependymomas.
Now, let's summarize why the other choices are incorrect:
A: BRAFKIAA1549 fusion - This fusion is typically associated with pilocytic astrocytomas, not ependymomas.
B: BRAFV600E mutation - This mutation is commonly found in other types of brain tumors like pleomorphic xanthoastrocytomas and gangliogliomas, not ependymomas.
D: H3K27M mutation - This mutation is typically seen in diffuse midline gliomas, such as diffuse
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A 2-month-old infant is brought to your clinic with an extensive scaly rash on the scalp, which has been biopsied and shown to be Langerhans cell histiocytosis (LCH). You want to determine whether this patient has skin-only LCH or involvement of any of the 'high-risk' organs. The child has a normal CBC; normal liver enzymes and bilirubin; and a normal skeletal survey, skull films, and chest X ray. What other screening test will be important for finding involvement of a high-risk organ?
- A. Reticulocyte count
- B. Erythrocyte sedimentation rate
- C. Alkaline phosphatase
- D. Serum albumin and total protein
Correct Answer: D
Rationale: The correct answer is D: Serum albumin and total protein. In Langerhans cell histiocytosis (LCH), involvement of high-risk organs like the liver, spleen, and bone marrow can occur. Serum albumin and total protein levels can indicate liver involvement as hypoalbuminemia and hypoproteinemia can be seen in liver dysfunction. The other choices, A (Reticulocyte count), B (Erythrocyte sedimentation rate), and C (Alkaline phosphatase) are not specific for evaluating high-risk organ involvement in LCH. Reticulocyte count is related to red blood cell production, ESR is a nonspecific marker of inflammation, and alkaline phosphatase is commonly elevated in various conditions, not specific for high-risk organ involvement in LCH.
A client is having a bone marrow aspiration and biopsy. What action by the nurse takes priority?
- A. Administer pain medication first.
- B. Ensure that valid consent is in the medical record.
- C. Have the client shower in the morning.
- D. Premedicate the client with sedatives.
Correct Answer: B
Rationale: The correct answer is B: Ensure that valid consent is in the medical record. This is the priority because obtaining informed consent is a legal and ethical requirement before any invasive procedure like a bone marrow aspiration and biopsy. Without valid consent, the procedure cannot proceed. Administering pain medication (A) can be important, but ensuring consent comes first. Having the client shower (C) is not a priority before the procedure. Premedicating with sedatives (D) may not be necessary for every client and should be based on individual assessment.
A nurse is caring for a patient with Hodgkin lymphoma at the oncology clinic. The nurse should be aware of what main goal of care?
- A. Cure of the disease
- B. Enhancing quality of life
- C. Controlling symptoms
- D. Palliation
Correct Answer: A
Rationale: The correct answer is A: Cure of the disease. The main goal of care for a patient with Hodgkin lymphoma is to achieve a cure whenever possible. This is because Hodgkin lymphoma is a potentially curable cancer with appropriate treatment. Achieving a cure means eradicating the cancer cells completely and preventing its recurrence in the future. This is the most desirable outcome for the patient's long-term health and well-being.
Incorrect choices:
B: Enhancing quality of life - While improving the patient's quality of life is important, the primary goal in treating Hodgkin lymphoma is to cure the disease.
C: Controlling symptoms - Symptom management is important in providing comfort to the patient, but the main goal is to cure the disease.
D: Palliation - Palliative care focuses on relieving symptoms and improving quality of life in patients with advanced or incurable diseases. For Hodgkin lymphoma, the main goal is to aim for a cure rather than palliation.
An infant is born with a firm mass over the chest with a central area of purpura and a 'halo' around it. An ultrasound reveals a high-flow lesion. What is the most likely diagnosis?
- A. Fibrosarcoma
- B. Infantile hemangioma
- C. Congenital hemangioma
- D. Capillary malformation
Correct Answer: C
Rationale: The most likely diagnosis in this case is congenital hemangioma (Choice C). This is because the description of a firm mass over the chest with central purpura and a 'halo' suggests a rapidly involuting congenital hemangioma. The high-flow lesion seen on ultrasound is characteristic of this type of hemangioma.
Choice A (Fibrosarcoma) is incorrect as fibrosarcoma typically presents differently and does not typically have a central area of purpura. Choice B (Infantile hemangioma) is incorrect as infantile hemangiomas are not usually present at birth and do not typically have a 'halo' appearance. Choice D (Capillary malformation) is incorrect as capillary malformations do not usually present as a firm mass with central purpura and a 'halo'.
In summary, the key features described in the question point towards a diagnosis of congenital hemangioma, making Choice C the most likely
The nurse is reviewing the activated partial thromboplastin time for a patient receiving heparin. Which value indicates that the medication is within the therapeutic range?
- A. 2.5 to 9.5 minutes
- B. 9.5 to 11.3 seconds
- C. 1.5 to 2.0 times normal
- D. 2.0 to 3.0 times normal
Correct Answer: C
Rationale: The correct answer is C (1.5 to 2.0 times normal) because the therapeutic range for heparin therapy is typically considered to be 1.5 to 2.0 times the normal control value for activated partial thromboplastin time (aPTT). This range ensures adequate anticoagulation to prevent clot formation without increasing the risk of bleeding. Options A, B, and D are incorrect because they provide ranges that are either too short or too long for the therapeutic goal of heparin therapy. Option A (2.5 to 9.5 minutes) and Option D (2.0 to 3.0 times normal) are outside the typical therapeutic range for heparin, indicating subtherapeutic and supratherapeutic levels, respectively. Option B (9.5 to 11.3 seconds) is also incorrect as it reflects a range for prothrombin time (PT) rather than aPTT.