ATI RN
ATI Hematologic System Questions
Question 1 of 5
A 4-year-old male child presents to the emergency department with his fourth invasive Staph infection. CBC consistently identifies moderate neutropenia. Sophisticated lab testing identifies lack of Toll-like receptor responses. The patient undergoes whole exome sequencing and is found to have pathogenic variants in IRAK4. What does 'IRAK4' stand for?
Correct Answer: C
Rationale: Step 1: Identify the function of IRAK4. IRAK4 is involved in the signaling pathway of the immune system, particularly in response to interleukin-1 (IL-1) receptor activation. Step 2: Break down the acronym IRAK4. IRAK4 stands for Interleukin-1 Receptor-Associated Kinase 4. Step 3: Link the information in the question to the correct answer. Given that the patient has a lack of Toll-like receptor responses and pathogenic variants in IRAK4, it indicates a problem with the interleukin-1 signaling pathway, making choice C (Interleukin-1 receptor-associated kinase 4) the correct answer. Summary: A: Incorrect - Interferon gamma receptor-associated kinase does not match the function of IRAK4. B: Incorrect - Inducible RAS activating kinase does not match the function of IRAK4. C: Correct - Matches the function of IRAK4
Question 2 of 5
A 3-month-old female presents to the emergency room with vomiting and abdominal distension. She has a left-side abdominal mass, and an abdominal ultrasound confirms an 8-cm mass arising from the left kidney. Liver lesions are also noted. Nephrectomy is performed and reveals a histologic diagnosis of malignant rhabdoid tumor of the kidney (MRTK). Which of the following is not a true statement about the management of this patient?
Correct Answer: C
Rationale: The correct answer is C: She has an excellent prognosis with surgery, chemotherapy, and radiation. Rationale: 1. Malignant rhabdoid tumor of the kidney (MRTK) is an aggressive cancer with poor prognosis. 2. Even with aggressive treatment, including surgery, chemotherapy, and radiation, the prognosis is generally poor due to high rates of recurrence and metastasis. 3. Therefore, stating that the patient has an excellent prognosis with the mentioned treatments is not true. Summary of other choices: A: Most patients with rhabdoid tumor of the kidney present in infancy - True, MRTK commonly presents in infancy. B: Most patients with rhabdoid tumor of the kidney present with metastatic (stage III or IV) disease - True, MRTK often presents with metastatic disease. D: Germline testing for SMARCB1/INI1 mutation on chromosome 22 is recommended, with brain MRI every 3 months until she is
Question 3 of 5
A 14-year-old Syrian male with beta thalassemia major has relocated to your community as a refugee. He has been receiving chronic transfusion therapy in Turkey for the past 3 years. On his first visit, you notice that his height is below the fifth percentile. He has skin discoloration and hepatosplenomegaly. His mother reports they have not had regular access to chelation therapy. Laboratory testing shows a serum ferritin of 6,200 ng/mL. A cardiac MRI shows grossly normal cardiac function but a T2* value of 9 ms. What is the most likely cause of his short stature?
Correct Answer: D
Rationale: The correct answer is D: Growth hormone deficiency due to iron deposition in the pituitary. Iron overload in patients with beta thalassemia major can lead to iron deposition in various organs, including the pituitary gland, impairing its function. This can result in growth hormone deficiency, leading to short stature. In this case, the patient's history of chronic transfusions and high serum ferritin level indicate iron overload, which can affect the pituitary gland. Choices A, B, and C are incorrect because growth failure in beta thalassemia major is primarily attributed to endocrine complications such as growth hormone deficiency, rather than lack of transfusions, cirrhosis, or ineffective erythropoiesis.
Question 4 of 5
A 12-year-old girl presents to your clinic with significant menstrual bleeding at the onset of menarche and is noted to have a hemoglobin of 9.9, although she is not symptomatic from her anemia. Her mother reports that she has a history of epistaxis when she was a child with some episodes lasting 30 minutes and that she also has heavy menstrual bleeding. Which of the following tests will lead to the most likely diagnosis?
Correct Answer: D
Rationale: The correct answer is D: Ristoectin cofactor activity. The scenario describes a young girl with significant menstrual bleeding and a history of prolonged epistaxis, suggesting a bleeding disorder. Given the clinical presentation, the most likely diagnosis is von Willebrand disease (vWD), the most common inherited bleeding disorder. Ristocetin cofactor activity is a specific test for vWD, as it assesses the ability of von Willebrand factor to bind to platelets in the presence of ristocetin. Factors XI, X, and XIII are not typically associated with vWD, making choices A, B, and C incorrect. Testing for Factor VIII/von Willebrand factor antigen levels and vWF multimer analysis may also be considered to confirm the diagnosis.
Question 5 of 5
You receive a phone call that a 3-year-old patient on long-term warfarin therapy for congenital heart disease has an international normalized ratio (INR) of 5.8. On further history, you learn the patient and several family members have had recent gastrointestinal illnesses, but the patient is recovering. His mother reports he is not experiencing bleeding symptoms. Which of the following interventions would be most reasonable in this clinical scenario?
Correct Answer: A
Rationale: The correct answer is A: Hold 1 to 2 doses of warfarin and recheck INR. In this scenario, the patient's elevated INR of 5.8 indicates an increased risk of bleeding due to excessive anticoagulation. Since the patient is not experiencing bleeding symptoms and is recovering from gastrointestinal illness, temporarily holding 1 to 2 doses of warfarin is the most reasonable intervention to prevent bleeding complications while allowing the INR to normalize. Rechecking the INR after holding the doses will help assess the patient's response to the intervention. Choices B, C, and D are incorrect because administering oral vitamin K therapy, FFP, or recombinant factor VIIa are more aggressive interventions that are not warranted in this case where the patient is asymptomatic and recovering from a transient illness.