Nokea
A patient is suffered from vitamin K deficiency. Which of the coagulation factors will not be affected?
- A. Factor II
- B. Factor VII
- C. Factor VIII
- D. Factor IX
Correct Answer: C
Rationale: The correct answer is C: Factor VIII. Factor VIII is not dependent on vitamin K for its synthesis, so its levels will not be affected by vitamin K deficiency. Factor II, VII, and IX are all vitamin K-dependent factors, so their levels will decrease with vitamin K deficiency, leading to impaired blood clotting. This is due to the role of vitamin K in the post-translational modification of these factors, which is necessary for their activation. Therefore, Factor VIII is the only factor that will not be affected by vitamin K deficiency.
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You are consulted on a 4-year-old girl who is newly diagnosed with standard-risk pre-B acute lymphoblastic leukemia. After reviewing her previous complete blood examinations, you note she has had a platelet count ranging from 80,000 to 100,000 cells/mcL over the past 2 years. Her father mentions that he has also been told he has mild thrombocytopenia. You suspect the child may have a cancer predisposition syndrome. Which sample should you send for analysis, and which gene is most likely implicated?
- A. Skin fibroblasts to evaluate the RUNXI gene
- B. Skin fibroblasts to evaluate the ETV6 gene
- C. Buccal swab to evaluate the RUNXI gene
- D. Buccal swab to evaluate the ETV6 gene
Correct Answer: B
Rationale: The correct answer is B: Skin fibroblasts to evaluate the ETV6 gene. In individuals with familial thrombocytopenia and a history of malignancy, ETV6 gene mutations are often implicated. Sending skin fibroblasts for analysis allows for genetic testing to identify any ETV6 gene mutations that may be present. Skin fibroblasts are commonly used for genetic testing as they provide a stable and reliable source of DNA.
Choice A (Skin fibroblasts to evaluate the RUNXI gene) is incorrect because RUNXI gene mutations are not typically associated with familial thrombocytopenia and cancer predisposition syndromes. Choice C (Buccal swab to evaluate the RUNXI gene) is also incorrect for the same reason. Choice D (Buccal swab to evaluate the ETV6 gene) is incorrect because buccal swabs may not provide enough genetic material for comprehensive testing of the ETV6 gene, which is
A patient's complete blood count (CBC) shows a hemoglobin of 19 g/dL and a hematocrit of 54%. Which question should the nurse ask to determine possible causes of this finding?
- A. Have you had a recent weight loss?
- B. Do you have any history of lung disease?
- C. Have you noticed any dark or bloody stools?
- D. What is your dietary intake of meats and protein?
Correct Answer: B
Rationale: The correct answer is B: Do you have any history of lung disease? A high hemoglobin and hematocrit can be indicative of chronic hypoxia, often seen in lung diseases like COPD. The nurse should ask about lung disease to explore the possibility of chronic hypoxia as the underlying cause. Choices A, C, and D are incorrect as they do not directly relate to the potential cause of elevated hemoglobin and hematocrit levels in this scenario.
A 50-year-old male who had frequent infections of his upper respiratory tract manifested with subcutaneous petechiae over his arms and legs. Investigations revealed low hemoglobin and a dry tap marrow. The most likely condition the patient is suffering from
- A. Iron deficiency anemia
- B. Megaloblastic anemia
- C. Hemolytic anemia
- D. Aplastic anemia
Correct Answer: D
Rationale: The correct answer is D: Aplastic anemia. This condition is characterized by pancytopenia, which includes low hemoglobin (anemia), low platelets (manifested as petechiae), and low white blood cells (frequent infections). Dry tap marrow is a classic finding in aplastic anemia due to bone marrow failure. Iron deficiency anemia (choice A) typically presents with microcytic hypochromic anemia without pancytopenia. Megaloblastic anemia (choice B) is characterized by macrocytic anemia with hypersegmented neutrophils and is usually caused by deficiencies in vitamin B12 or folate. Hemolytic anemia (choice C) presents with anemia due to increased destruction of red blood cells, which does not explain the low platelets and white blood cells seen in this patient.
A 2-month-old girl is found to have a small, hard mass on her scalp. The mass increases in size over the next 4 weeks. A biopsy is performed that confirms a diagnosis of embryonal rhabdomyosarcoma. You initiate chemotherapy with vincristine, dactinomycin, and cyclophosphamide. The child presents to clinic for day 1 of cycle 3 of chemotherapy, and the mass on her scalp is smaller. She is afebrile, absolute neutrophil count is 1,405 cells/mcL, platelet count is 154,000/mcL, and total bilirubin is 0.8 mg/dL. Her mother reports she looks very tired because her eyelids have been 'very droopy,' and she thinks she has a sore throat because her cry is hoarse. Her last bowel movement was 2 days ago. What is the most appropriate chemotherapy plan?
- A. Continue vincristine, dactinomycin, and cyclophosphamide at full dosage.
- B. Do not administer any chemotherapy; rhabdomyosarcoma is progressing and she needs different therapy.
- C. Administer dactinomycin and cyclophosphamide but hold the vincristine and reevaluate weekly. If the ptosis and hoarse cry resolve, vincristine can be resumed with a dose reduction and, if tolerated, re-escalated to the full dose in the future.
- D. Administer dactinomycin and cyclophosphamide but discontinue vincristine permanently.
Correct Answer: C
Rationale: The correct answer is C: Administer dactinomycin and cyclophosphamide but hold the vincristine and reevaluate weekly. If the ptosis and hoarse cry resolve, vincristine can be resumed with a dose reduction and, if tolerated, re-escalated to the full dose in the future.
Rationale:
1. Ptosis and hoarse cry are symptoms of vincristine-induced neurotoxicity.
2. Holding vincristine allows for resolution of these side effects.
3. Reevaluating weekly ensures close monitoring of symptoms.
4. If symptoms resolve, vincristine can be cautiously reintroduced with a dose reduction to prevent further neurotoxicity.
Summary:
A: Continuing all chemotherapy may worsen neurotoxicity.
B: Withholding all chemotherapy is not appropriate if two agents are effective.
D: Discontinuing vincristine permanently may limit treatment options.
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.