Nokea
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?
- A. Interferon gamma receptor-associated kinase 4
- B. Inducible RAS activating kinase 4
- C. Interleukin-1 receptor-associated kinase 4
- D. Immune response activating kinase 4
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.
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The nurse is assessing a patient with chronic lung disease. Which finding indicates long-term hypoxia?
- A. Pallor
- B. Dyspnea
- C. Clubbed fingertips
- D. Pulmonary crackles
Correct Answer: C
Rationale: The correct answer is C: Clubbed fingertips. Clubbing of the fingertips is a sign of long-term hypoxia due to chronic lung disease, causing changes in the nail bed angle and soft tissue around the nails. Pallor (A) is a paleness of the skin and not specific to hypoxia. Dyspnea (B) is difficulty breathing, which can occur in acute or chronic conditions. Pulmonary crackles (D) are abnormal lung sounds indicating fluid in the lungs, commonly seen in conditions like pneumonia. Clubbed fingertips specifically point towards long-term hypoxia in chronic lung disease.
A study is designed to investigate the rates of central line–associated blood stream infections among pediatric hematology/oncology patients. Three common central line types (totally implanted catheter [port], peripherally inserted central catheter [PICC], and tunneled externalized catheter [TEC]) were included in the study. What data structure is central line type?
- A. Continuous
- B. Dichotomous
- C. Nominal
- D. Ordinal
Correct Answer: C
Rationale: The correct answer is C: Nominal. Central line type is a nominal data structure because it represents categories without any inherent order or ranking. In this study, the central line types (port, PICC, TEC) are distinct categories with no numerical significance or order. They are simply different types of central lines used for medical purposes. Continuous (A) data involves numerical values with infinite possibilities, which is not the case here. Dichotomous (B) data consists of only two categories, while in this study there are three central line types. Ordinal (D) data involves categories with a specific order or ranking, which is not applicable to central line types as they do not have a natural order.
The nurse is assessing a patient with chronic lung disease. Which finding indicates long-term hypoxia?
- A. Pallor
- B. Dyspnea
- C. Clubbed fingertips
- D. Pulmonary crackles
Correct Answer: C
Rationale: The correct answer is C: Clubbed fingertips. Clubbing is a sign of prolonged hypoxia due to chronic lung disease. It is characterized by enlargement and rounding of the fingertips. This occurs as a result of chronic hypoxia causing tissue changes in the fingers. Pallor (A) is a pale skin color often indicating decreased blood flow. Dyspnea (B) is difficulty breathing and can be an acute symptom of hypoxia. Pulmonary crackles (D) are abnormal lung sounds indicating fluid accumulation and are not specific to long-term hypoxia.
A patient is presented with weakness of legs, arms, trunk, tingling and numbness that progressively worsens. Peripheral blood smear shows macrocytic anemia. Which of the following is not the factor causing this condition?
- A. Chronic atrophic gastritis
- B. Ileal resection
- C. Tapeworm infestation
- D. Alcoholism
Correct Answer: C
Rationale: The correct answer is C: Tapeworm infestation. The symptoms described are consistent with vitamin B12 deficiency, which can result in macrocytic anemia and neurological symptoms. Chronic atrophic gastritis and ileal resection can impair absorption of vitamin B12, leading to deficiency. Alcoholism can also lead to malnutrition and vitamin deficiencies. Tapeworm infestation, on the other hand, does not directly affect vitamin B12 absorption. Therefore, tapeworm infestation is not a factor causing the condition described.
The following pathological features is true regarding non-Hodgkin lymphoma (NHL)
- A. Classical Reed-Sternberg (RS) cells are neoplastic cells
- B. B cell phenotype is more common
- C. Contiguous group of lymph nodes are affected
- D. It is not associated with leukemic phase of disease
Correct Answer: B
Rationale: The correct answer is B because B cell phenotype is more common in non-Hodgkin lymphoma (NHL). This is supported by the fact that the majority of NHL cases arise from B cells in the lymphoid tissue. Choice A is incorrect as RS cells are characteristic of Hodgkin lymphoma, not NHL. Choice C is incorrect because NHL can involve non-contiguous lymph nodes. Choice D is incorrect because NHL can have a leukemic phase known as leukemic lymphoma.