Nokea
A nurse is caring for a patient who is being treated for leukemia in the hospital. The patient was able to maintain her nutritional status for the first few weeks following her diagnosis but is now exhibiting early signs and symptoms of malnutrition. In collaboration with the dietitian, the nurse should implement what intervention?
- A. Arrange for total parenteral nutrition (TPN).
- B. Facilitate placement of a percutaneous endoscopic gastrostomy (PEG) tube.
- C. Provide the patient with several small, soft-textured meals each day.
- D. Assign responsibility for the patient's nutrition to the patient's friends and family.
Correct Answer: C
Rationale: The correct answer is C: Provide the patient with several small, soft-textured meals each day. This intervention is appropriate because it focuses on improving the patient's nutritional intake through easily digestible meals, which can help address early signs of malnutrition. Small, soft-textured meals are easier for the patient to eat, especially if they are experiencing symptoms like mouth sores or difficulty swallowing. This approach also promotes regular intake of nutrients throughout the day, which can be more beneficial than relying solely on one large meal.
Incorrect answers:
A: Total parenteral nutrition (TPN) is typically reserved for patients who cannot tolerate oral or enteral nutrition. It is not the first-line intervention for early signs of malnutrition.
B: Percutaneous endoscopic gastrostomy (PEG) tube placement is usually considered for patients who are unable to eat orally in the long term. It is not indicated for early signs of malnutrition.
D: Assigning responsibility for the patient's nutrition to friends and
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You are consulting on a 10-year-old male with severe persistent neutropenia, a history of recurrent infections, and warts. The rest of the peripheral blood count is normal. His mother also has neutropenia. Bone marrow examination shows a hypercellular marrow and retained myeloid cells with vacuolated cytoplasm. There are no abnormalities in the red cells or platelet precursors. Cytogenetics are 46XY. You start granulocyte colony stimulating factor therapy and the neutrophil count increases. A mutation in which of the following genes is most likely to have caused this familial inherited bone marrow failure syndrome?
- A. CXCR4
- B. ELANE
- C. GATA 2
- D. Mitochondrial DNA
Correct Answer: A
Rationale: The correct answer is A: CXCR4. In this case, the patient presents with severe neutropenia, recurrent infections, and warts, suggestive of WHIM syndrome, where CXCR4 mutations are often involved. CXCR4 plays a crucial role in immune cell trafficking and retention in the bone marrow. The hypercellular marrow and vacuolated myeloid cells are consistent with WHIM syndrome. The absence of abnormalities in red cells or platelet precursors rules out other syndromes. Mutations in ELANE are commonly associated with congenital neutropenia, not familial inherited bone marrow failure syndromes. GATA2 mutations are linked to familial myelodysplastic syndromes, not typically presenting with neutropenia and warts. Mitochondrial DNA mutations are more related to mitochondrial disorders, which usually manifest with multi-system involvement, not specific to bone marrow failure syndromes.
You are seeing a 12-year-old female who presented to the emergency department with the sudden onset of severe abdominal pain. Imaging that was obtained to rule out appendicitis revealed a mass adjacent to the bladder. The mass was surgically resected, and pathology demonstrated a paraganglioma. Which of the studies below would be most useful to determine disease stage for this patient?
- A. Bone Scan
- B. Lumbar puncture for cerebrospinal fluid cytology
- C. Bone marrow aspirate and biopsy
- D. Ga 68-DOTATATE PET/CT
Correct Answer: D
Rationale: The correct answer is D: Ga 68-DOTATATE PET/CT. Paragangliomas are neuroendocrine tumors that can secrete catecholamines and have a high expression of somatostatin receptors. Ga 68-DOTATATE PET/CT is the most appropriate imaging study to determine disease stage in patients with paragangliomas because it can detect the somatostatin receptor expression in these tumors, aiding in localization, staging, and treatment planning.
A: Bone scan is not the most useful study for determining disease stage in paraganglioma.
B: Lumbar puncture for cerebrospinal fluid cytology is not relevant for staging paraganglioma.
C: Bone marrow aspirate and biopsy are not the most appropriate studies for staging paraganglioma.
A 9-year-old boy presents to the emergency department with emesis and headache of 3 weeks' duration. MRI reveals a large heterogeneous mass in the cerebellum. He undergoes a resection, and the pathology is most consistent with a classic medulloblastoma. Which of the following findings would classify the patient as a high-risk medulloblastoma?
- A. Elevated serum and CSF AFP and beta-HCG tumor markers
- B. WNT subgrouping on molecular classification
- C. A presurgical spine MRI that reveals bulky tumor in the spine
- D. A postoperative brain MRI with no signs of residual tumor
Correct Answer: C
Rationale: The correct answer is C. A presurgical spine MRI revealing bulky tumor in the spine classifies the patient as high-risk medulloblastoma due to metastasis. This finding indicates dissemination of the tumor beyond the primary site in the cerebellum, which is associated with poorer prognosis and necessitates more aggressive treatment.
Choice A is incorrect because elevated serum and CSF tumor markers are not specific indicators of high-risk medulloblastoma. Choice B (WNT subgrouping) is associated with favorable prognosis, so it does not classify the patient as high-risk. Choice D (postoperative brain MRI showing no residual tumor) is a positive outcome but does not address the presence of metastasis, which is crucial in determining high-risk status.
You are caring for a patient with a large localized Ewing sarcoma of the soft tissues of the arm. The surgeon believes that the tumor can be resected without amputation but asks whether you can give some chemotherapy to shrink the tumor before surgery. Which of the following would you tell the surgeon?
- A. If the tumor can be resected without amputation, then the best time to do the resection is before any chemotherapy to improve the prognosis.
- B. You agree with waiting to do the resection until week 12 of therapy and will begin chemotherapy; you recognize that radiotherapy will not be necessary if the tumor is completely resected at week 12 of therapy.
- C. You agree with waiting to do the resection until week 12 of therapy and will begin chemotherapy; you recognize that radiotherapy will be necessary even if the tumor is completely resected at week 12 of therapy.
- D. If the tumor can be resected without amputation, then the best time to do the resection is before any chemotherapy; you recognize that this is the only way to avoid radiotherapy.
Correct Answer: B
Rationale: The correct answer is B because starting chemotherapy before resection at week 12 can help shrink the tumor, making it more manageable for surgery. A is incorrect because preoperative chemotherapy can improve outcomes. C is incorrect because radiotherapy may not be necessary if the tumor is completely resected. D is incorrect because radiotherapy may still be necessary even if the tumor is resected before chemotherapy.
An 8-year-old girl presents with National Cancer Institute (NCI) Standard Risk acute pre-B-cell acute lymphoblastic leukemia. Her family history is significant for her mother having been diagnosed with breast cancer at age 34 years and a maternal uncle who developed osteosarcoma as a teenager. What cytogenetic abnormality is most likely to be detected in this patient?
- A. t(1;19)
- B. CRLF2 rearrangement with a JAK2 mutation
- C. KMT24 rearrangement
- D. Hypodiploidy with a modal chromosome number of 34
Correct Answer: D
Rationale: The correct answer is D, Hypodiploidy with a modal chromosome number of 34. In pediatric pre-B-cell acute lymphoblastic leukemia, hypodiploidy with fewer than 44 chromosomes is associated with a poor prognosis. This cytogenetic abnormality is commonly seen in cases of NCI Standard Risk acute pre-B-cell ALL.
A: t(1;19) is typically associated with T-cell ALL, not pre-B-cell ALL.
B: CRLF2 rearrangement with a JAK2 mutation is more commonly seen in high-risk B-cell ALL.
C: KMT24 rearrangement is not a recognized cytogenetic abnormality in ALL.
In summary, the presence of hypodiploidy with a modal chromosome number of 34 is the most likely cytogenetic abnormality in this patient based on her clinical presentation and family history.