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NBME CBSE ACTUAL TEST QUESTIONS AND
ANSWERS TEST BANK - COMPLETE SOLUTION
RATED A
- 36-year-old F with 2 week history of fatigue, bleeding of the
gums, and bone pain. Physical examination shows pallor, hepatosplenomegaly, and ecchymotic lesions over extremities.Labs:Hb 8g/dlHt 25%Leukocytes: 36,000segm neutr 4%eosinophils 4%lymph 6%mono 6% promyelo 80%platelets 25,000Polymerase chain reaction test shows an mRNA corresponding to the retinoic acid receptor- alfa/promyelocytic leukemia fusion gene resulting from a reciprocal translocation of chromosomes 15 and 17. Treatment w/ all-trans retinoic acid is started. In response to the therapy, the fusion protein will most likely attract which of the following proteins to form a pre- transcriptional complex?
- Histone acetylase
- Histone acetylation allows for relaxation of the DNA
- Histone Acetylation makes DNA Active
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- Histone Methylation Mostly Makes DNA Mute.
A 1 -month old - 5 days of vomiting after feeding - vomiting is forceful - PE shows decreased skin turgor. Which set of serum findings - most likely in this newborn?Na 132 K 3.2 Cl- 90
HCO3 37
hypochloremia, hypokalemia, hyponatremia, and metabolic alkalosis chronic vomiting, you lose electrolytes and a lot of acid.It triggers metabolic alkalosis which is why all the serum values are low (or on the lower end of the normal range) except for bicarbonate.
A 1 month old - male newborn is brought to the physician for a routine examination. Parents have dark skin and eyes. PE shows
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hypopigmentation of the skin, light blonde hair, and translucent irises. The inherited disorder causes this phenotypic expression is most likely due to a defect in the metabolism of which?Tyrosine albinism, which is due to decreased tyrosinase activity.
If he has a problem metabolizing Phenylalanine, he would be presenting with the PKU sx like intellectual disability, musty body odor, etc., in addition to his fair complexion
- week old newborn - poor feeding, vomiting, progressive
lethargy - No congenital anomalies. PE shows decreased muscle tone and poor responsiveness - reflexes are normal. pH 7.15, bicarb low, high ammonia (10X) - Cause?Organic Acid Metabolism disorder Organic acidemias most commonly present in infancy with poor feeding, vomiting, hypotonia, a high anion gap metabolic acidosis, hepatomegaly, and seizures.
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A 1-year-old boy impaired respiratory burst of phagocytes - Family history of immunodeficiency - Patient has greatest risk of infection by which organism?Staphylococcus aureus Chronic Granulomatous Disease -NADPH deficiency phagocytes need to produce hydrogen peroxide to undergo oxidative burst-
CGD patients can't kill catalase + organisms (Staph Aureus, Aspergillus) - catalase enzymes neutralize superoxide
1-year-old boy with rash for 2 weeks. 10th percentile for height and weight. PE scaly, seborrheic eruption over scalp, palms, back, diaper region and soles of feet. Generalized lymphadenopathy and hepatosplenomegaly. Xray of skull shows osteolytic lesions. EM biopsy of skin shows tennis racket- shaped bilamellar granule in cytoplasm. Immuno studies show CD1a antigen expression. Abnormal cells in patient are derived from which cell?
Dendritic cells