A study is designed to investigate the rates of central line–associated blood stream infections among pediatric hematology/oncology patients. Three common central line types (totally implanted catheter [port], peripherally inserted central catheter [PICC], and tunneled externalized catheter [TEC]) were included in the study. What data structure is central line type?

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Question 1 of 5

A study is designed to investigate the rates of central line–associated blood stream infections among pediatric hematology/oncology patients. Three common central line types (totally implanted catheter [port], peripherally inserted central catheter [PICC], and tunneled externalized catheter [TEC]) were included in the study. What data structure is central line type?

Correct Answer: C

Rationale: The correct answer is C: Nominal. The central line type is a categorical variable with no inherent order or ranking. Each type is distinct and cannot be quantitatively ordered or measured. It is not continuous (A), as it is not on a scale. It is not dichotomous (B) as there are more than two categories. It is not ordinal (D) because the types do not have a clear order or ranking. Therefore, central line type is best represented by a nominal data structure.

Question 2 of 5

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?

Correct Answer: D

Rationale: The correct answer is D: Serum albumin and total protein. In LCH, high-risk organ involvement includes the liver and spleen. Serum albumin and total protein levels can help assess liver function, as low levels may indicate liver involvement. A normal CBC, liver enzymes, and bilirubin do not rule out organ involvement, as LCH can affect organs without causing significant abnormalities in these tests. Reticulocyte count (A) is not relevant for assessing high-risk organ involvement in LCH. Erythrocyte sedimentation rate (B) is a nonspecific marker of inflammation and not specific for organ involvement. Alkaline phosphatase (C) is more indicative of bone or liver disease rather than specifically assessing high-risk organ involvement in LCH.

Question 3 of 5

A 9-month-old boy has been referred to you for the evaluation of an enlarged abdomen. Imaging studies show a large liver mass (PRETEXT III). Alfa-fetoprotein is 98 ng/mL, and a CT scan of the lungs show bilateral lung metastases. A needle biopsy is performed, and you are planning to review the specimen with the pathologist. Which of the following diagnoses are you suspecting?

Correct Answer: D

Rationale: The correct answer is D: Small cell undifferentiated hepatoblastoma. In this case, the key features to consider are the patient's age (9 months old), large liver mass with lung metastases, and elevated alpha-fetoprotein level. Small cell undifferentiated hepatoblastoma is commonly seen in infants, presents as a large liver mass, and frequently metastasizes to the lungs. The alpha-fetoprotein level in this case is also elevated, which is typical for hepatoblastoma. Pure fetal histology hepatoblastoma (A) is less common in older infants, and the presence of lung metastases is not typical. Embryonal sarcoma (B) typically presents as a solitary mass without metastases. Fibrolamellar hepatocellular carcinoma (C) is rare in infants and does not typically present with elevated alpha-fetoprotein levels. Thus, based on the clinical presentation and imaging findings, small cell undifferentiated hepatob

Question 4 of 5

An otherwise healthy 18-year-old female is diagnosed with high-risk neuroblastoma after presenting with fatigue and bony pain. Imaging findings demonstrate a left adrenal mass with multiple osseous metastases. She successfully completes standard therapy for high-risk neuroblastoma, but experiences several episodes of disease recurrence and ultimately dies of her disease 10 years after her initial diagnosis. During her treatment, her tumor was sent for molecular analysis. Of the following, what molecular aberration was most likely to have been detected?

Correct Answer: C

Rationale: The correct answer is C: ATRX mutation. In neuroblastoma, ATRX mutations are associated with poor prognosis and increased risk of disease recurrence. This mutation disrupts chromatin remodeling, leading to genomic instability and aggressive tumor behavior. ETV6-NTRK3 gene fusion (Choice A) is more commonly associated with pediatric mesenchymal tumors. PTPN11 mutation (Choice B) is typically found in Noonan syndrome and other malignancies, not neuroblastoma. WT1 mutation (Choice D) is more commonly seen in Wilms tumor, not neuroblastoma. In summary, the ATRX mutation is the most likely molecular aberration detected in this case based on the clinical presentation and outcomes of the patient.

Question 5 of 5

A 9-year-old boy is being treated for standard-risk acute lymphoblastic leukemia. His treatment protocol calls for administration of intravenous methotrexate and intramuscular L-asparaginase during interim maintenance chemotherapy. What is the most appropriate sequence of drug administration?

Correct Answer: B

Rationale: The correct answer is B: Administer L-asparaginase immediately after the methotrexate infusion. This sequence is appropriate because methotrexate can interfere with the activity of L-asparaginase if given concurrently, affecting the efficacy of both drugs. Administering L-asparaginase immediately after the methotrexate infusion allows for optimal therapeutic effects of both medications without compromising their individual actions. Choice A (Administer L-asparaginase during the methotrexate infusion) is incorrect because it may lead to drug interactions and reduced effectiveness of both drugs. Choice C (Administer both drugs at the same time) is incorrect for the same reason as choice A. Choice D (Administer methotrexate 24 hours after the asparaginase) is incorrect as it does not follow the optimal timing for these medications during treatment.

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