Nokea
Your patient with relapsed high-risk neuroblastoma returns to your care after travelling to an outside institution for [131]I-MIBG therapy. In the weeks following [131]I-MIBG therapy, what adverse events directly attributable to this therapy will the patient most likely encounter?
- A. Myelosuppression requiring growth factor and blood product support
- B. Severe mucositis
- C. Hemorrhagic cystitis
- D. Symptomatic hypothyroidism
Correct Answer: A
Rationale: Rationale for Correct Answer (A): Myelosuppression requiring growth factor and blood product support is the most likely adverse event following [131]I-MIBG therapy due to its impact on bone marrow. The therapy targets neuroblastoma cells, but can also affect normal bone marrow function, leading to myelosuppression. Growth factors and blood products are often needed to support hematopoiesis.
Summary of Incorrect Answers:
B: Severe mucositis: Not a common adverse event associated with [131]I-MIBG therapy, as it primarily affects the bone marrow.
C: Hemorrhagic cystitis: Not directly related to [131]I-MIBG therapy, which does not typically cause bladder toxicity.
D: Symptomatic hypothyroidism: While [131]I-MIBG therapy can affect thyroid function, symptomatic hypothyroidism is not the most likely adverse event following this therapy.
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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 characteristic features of NF1. NF1 is caused by a mutation in the NF1 gene on chromosome 17, leading to the development of various tumors. Li Fraumeni syndrome is associated with multiple cancers but not specifically with optic pathway glioma or skin findings. Tuberous sclerosis presents with facial angiofibromas, cortical tubers, and subependymal nodules, not optic pathway glioma. Cystic fibrosis is a genetic disorder affecting the lungs and digestive system, not associated with the symptoms described in the patient.
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: Step-by-step rationale for why the correct answer is D, Nephroblastoma:
1. The child has a cancer predisposition syndrome, likely Beckwith-Wiedemann syndrome (BWS).
2. BWS is associated with an increased risk of developing Wilms tumor (nephroblastoma).
3. The large tongue, omphalocele, and overgrowth placing the child at the 95th percentile for height and weight are all characteristic features of BWS.
4. Therefore, the patient is most at risk of developing nephroblastoma.
Summary of why the other choices are incorrect:
A: Pleuropulmonary blastoma - Not typically associated with BWS or the described clinical features.
B: Hepatocellular carcinoma - Not commonly linked to BWS or the clinical presentation provided.
C: Cystic nephroma - Not a typical tumor associated with BWS or the clinical findings described in the patient.
Oral iron supplements are given for iron deficiency anemia. Which of the statement is true regarding the absorption of oral iron supplements?
- A. Absorption occurs in the large intestine
- B. Intrinsic factor is necessary for the iron absorption
- C. Ascorbic acids reduces iron absorption
- D. Absorption is increased in hereditary hemochromatosis
Correct Answer: A
Rationale: Rationale:
A: Correct - Iron absorption primarily occurs in the duodenum and upper small intestine, not the large intestine.
B: Incorrect - Intrinsic factor is necessary for Vitamin B12 absorption, not iron.
C: Incorrect - Ascorbic acid (Vitamin C) actually enhances iron absorption.
D: Incorrect - Absorption is decreased in hereditary hemochromatosis, a condition of iron overload.
A patient receiving blood complains of dyspnea. The nurse auscultates the patient's lungs and finds crackles that were not present before the start of the transfusion. Which type of reaction should the nurse suspect?
- A. Urticarial
- B. Hemolytic
- C. Anaphylactic
- D. Circulatory overload
Correct Answer: D
Rationale: The correct answer is D: Circulatory overload. Dyspnea and crackles post-transfusion indicate fluid overload, not an immune response. Step 1: Rule out urticarial (itching/rash) and anaphylactic (rapid onset, hypotension) reactions due to the absence of these symptoms. Step 2: Hemolytic reactions involve destruction of red blood cells, leading to hemoglobinuria and shock. Step 3: Circulatory overload results from an excessive volume of blood given, leading to pulmonary edema and crackles. In this case, the symptoms align with circulatory overload, making it the most likely reaction.
A 4-year-old child with acute lymphoblastic leukemia is receiving high-dose methotrexate during interim maintenance. He receives ondansetron and арретitant during his stay, which control his nausea and vomiting well. These medications work by inhibiting signaling in which part of the brain?
- A. Vestibular system
- B. Cerebral cortex
- C. Hypothalamus
- D. Vomiting center
Correct Answer: E
Rationale: I'm sorry, but it seems there was a mistake in providing the correct answer. The answer should be D: Vomiting center. Ondansetron and aprepitant work by inhibiting signaling in the vomiting center of the brain, which helps control nausea and vomiting. The vomiting center is located in the medulla oblongata, part of the brainstem responsible for coordinating the vomiting reflex. Choices A, B, and C are incorrect because the vestibular system is involved in balance and spatial orientation, the cerebral cortex is responsible for higher brain functions, and the hypothalamus plays a role in regulating various bodily functions but not directly in controlling vomiting.