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: The correct answer is C: Interleukin-1 receptor-associated kinase 4 (IRAK4). 1. IRAK4 is involved in the immune response pathway triggered by interleukin-1 receptor signaling. 2. Lack of Toll-like receptor responses in the patient aligns with the role of IRAK4 in the interleukin-1 receptor pathway. 3. Pathogenic variants in IRAK4 can lead to immunodeficiency, explaining recurrent Staph infections. 4. Choices A, B, and D do not accurately reflect the known function of IRAK4 and its association with interleukin-1 receptor signaling.
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. This statement is not true because malignant rhabdoid tumor of the kidney (MRTK) has a poor prognosis, even with aggressive treatment. Here's a breakdown: 1. MRTK is an aggressive and rare tumor that often presents in infancy, supporting statement A. 2. Most patients with MRTK present with metastatic disease, indicating poor prognosis, aligning with statement B. 3. Germline testing for SMARCB1/INI1 mutation is essential due to the genetic predisposition associated with MRTK, supporting statement D. In summary, statement C is incorrect as MRTK typically has a challenging clinical course despite comprehensive treatment approaches.
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 deposition in the pituitary gland can lead to impaired production and secretion of growth hormone, resulting in short stature. This is a known complication of beta thalassemia major. The patient's elevated serum ferritin levels and T2* value of 9 ms indicate iron overload, which can cause damage to organs including the pituitary gland. Choices A, B, and C are incorrect because the patient's growth failure is primarily due to the impact of iron deposition on the pituitary gland rather than lack of transfusion, cirrhosis, or ineffective erythropoiesis. Regular chelation therapy is essential to prevent iron overload and its complications in patients with beta thalassemia major.
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. This patient's history of significant menstrual bleeding, epistaxis, and low hemoglobin suggests a congenital bleeding disorder. Ristoectin cofactor activity is a test for von Willebrand disease (VWD), a common inherited bleeding disorder characterized by a deficiency or dysfunction of von Willebrand factor. VWD typically presents with mucocutaneous bleeding, such as epistaxis and menorrhagia. Choice A: Factor XI level is not the most likely diagnosis as Factor XI deficiency (Hemophilia C) typically presents with bleeding after surgery or trauma, not mucocutaneous bleeding. Choice B: Factor X level is not the most likely diagnosis as Factor X deficiency presents with bleeding that is more severe and can cause hemarthrosis, not typically seen in this patient. Choice C: Factor XIII level is not the most likely diagnosis as Factor XIII deficiency leads to delayed wound healing and poor clot formation,
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 high INR indicates an increased risk of bleeding due to excess anticoagulation. However, since the patient is asymptomatic and the high INR is likely due to transient factors (gastrointestinal illness), the most appropriate initial step is to temporarily hold warfarin to allow the INR to normalize. Rechecking the INR after holding the doses will guide further management. Choice B (Administer oral vitamin K therapy) is incorrect because the patient is not experiencing bleeding symptoms and oral vitamin K should be reserved for patients with active bleeding or high INR with bleeding symptoms. Choice C (Administer fresh frozen plasma) is inappropriate as the patient is not actively bleeding and FFP is typically reserved for acute bleeding situations. Choice D (Administer recombinant factor VIIa) is also incorrect as it is reserved for severe bleeding in patients on war