Nokea
Henoch Schonlein purpura is not associated with:
- A. thrombocytopenia
- B. palpable purpura
- C. intussusception
- D. acute diffuse glomerulonephritis
Correct Answer: A
Rationale: Henoch-Schonlein purpura (HSP) is a vasculitis that primarily affects small blood vessels. Thrombocytopenia, which is a low platelet count, is not typically associated with HSP. Palpable purpura, intestinal intussusception, and acute diffuse glomerulonephritis are commonly seen in HSP due to immune-mediated inflammation of blood vessels, gastrointestinal involvement, and renal complications. Thrombocytopenia, on the other hand, is not a characteristic feature of HSP, making choice A the correct answer.
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A nurse is administering epoetin intravenously to a client who has renal failure. Which of the following actions should the nurse take?
- A. Shake the vial before using.
- B. Administer via IV bolus over 1 to 3 min.
- C. Dilute the drug first with D5W.
- D. Save the used vial for the next dose.
Correct Answer: B
Rationale: The correct answer is B: Administer via IV bolus over 1 to 3 min. This route and method of administration is appropriate for epoetin to ensure proper absorption and effect. Shaking the vial (A) can denature the drug. Diluting with D5W (C) is not recommended as it may alter the drug's effectiveness. Saving the used vial for the next dose (D) is unsafe and violates infection control practices.
Which of the following is not the criteria in the international prognostic index for non Hodgkin's lymphoma?
- A. Number of extranodal sites
- B. LDH level
- C. Lymphocyte count
- D. Performance status
Correct Answer: C
Rationale: The correct answer is C: Lymphocyte count. The International Prognostic Index (IPI) for non-Hodgkin's lymphoma includes factors such as age, number of extranodal sites, LDH level, performance status, and stage. Lymphocyte count is not a specific criteria in the IPI. This index is used to predict the outcome of patients with aggressive lymphomas. A higher score indicates a worse prognosis. A low lymphocyte count is not a consistent prognostic factor for non-Hodgkin's lymphoma, unlike the other factors listed. Therefore, choice C is not a criteria in the IPI, making it the correct answer.
A patient who is suspected of having acute viral hemorrhagic fever reveals history of tick bite. Which of the following is the most possible causative agent?
- A. Hantavirus
- B. Rift Valley virus
- C. Crimean Congo virus
- D. Marburg virus
Correct Answer: C
Rationale: The correct answer is C: Crimean Congo virus. This is the most likely causative agent of acute viral hemorrhagic fever following a tick bite due to its association with tick transmission. Crimean Congo virus is a tick-borne virus that causes severe hemorrhagic fever in humans. The other options, Hantavirus, Rift Valley virus, and Marburg virus, are not typically transmitted by ticks. Hantavirus is spread through rodent feces, Rift Valley virus is transmitted by mosquitoes, and Marburg virus is transmitted through direct contact with infected animals or their bodily fluids. Therefore, based on the history of tick bite, Crimean Congo virus is the most probable causative agent.
You have been asked to see a 15-year-old girl who is being referred for evaluation of an ovarian mass. Her history is also significant for secondary amenorrhea, and physical examination shows signs of virilization. As you review her family history, what syndrome will you consider?
- A. Li-Fraumeni syndrome
- B. DICER-1 syndrome
- C. Turner syndrome
- D. Beckwith-Wiedemann syndrome
Correct Answer: B
Rationale: The correct answer is B: DICER-1 syndrome. This syndrome is associated with ovarian tumors, secondary amenorrhea, and signs of virilization due to androgen-secreting tumors. Li-Fraumeni syndrome (A) is characterized by multiple primary cancers but not specifically ovarian masses. Turner syndrome (C) presents with ovarian dysgenesis and primary amenorrhea. Beckwith-Wiedemann syndrome (D) is associated with overgrowth and abdominal wall defects, not ovarian masses. In this case, the presence of ovarian mass, secondary amenorrhea, and virilization point towards DICER-1 syndrome.
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.