NBME 27

Question 1

______ sign is produced by eliciting pain from tapping the Median nerve, which is suggestive of Carpal Tunnel.

Answer 1

Tinel’s sign

Question 2

Multiple Myeloma is a ____-lymphocyte neoplasia typically identified by an ____-spike on serum electrophoresis. Diagnosis is confirmed with a bone marrow biopsy showing at least ____% clonal plasma cells.

Answer 2

B-lymphocyte

M-spike (M protein)

AT LEAST 10%

Question 3

Chronic Myelogenous Leukemia is defined by the presence of the ______ chromosome, which occurs via translocation between chromosomes ___ and ____ –> results in constitutive activation of ______ tyrosine kinase. Look for immature granulocytes and ______philia.

Answer 3

Philadelphia chromosome

9

22

ABL1

Basophilia

Question 4

Pierre Robin Syndrome - Newborn presenting with _______ (jaw size), cleft ______, and ________ (tongue). Look for these patients to have issues breathing while laying down.

This is due to altered development of the ______ Pharyngeal pouch.

Answer 4

Micrognathia

Cleft Mandible

Glossoptosis

1st

Question 5

Proliferating spindles cells forming slit-like spaces filled with blood on histology is diagnostic of ____ ____ (can be induced by which virus?)

Look for immunocompromised patients to present with violaceous raised cutaneous plaques. Which screening tests are recommended?

Answer 5

Kaposi Sarcoma

Can be induced by HHV-8

Screen for fecal occult blood (stool sample) and Pulmonary lesions (x-ray)

Question 6

Doll’s Eye Maneuver refers to what?

Answer 6

Patient’s eyes remain midline when their head is turned by the examiner –> indicates severe brainstem damage.

Question 7

Abnormal ______ exposure is the greatest predisposing factor for Endometrial carcinoma.

Answer 7

Estrogen

Question 8

______ sign is when tapping of the facial nerve (CN VII) causes spasm of facial muscles –> sign of hypocalcemia

Answer 8

Chvostek sign

Question 9

_______ sign is positive when an inflated BP cuff occluding the brachial artery results in carpopedal spasm –> sign of hypocalcemia

Answer 9

Trousseau sign

Question 10

If the sum of the magnitudes of S wave in lead V2 and R wave in lead V5 of an ECG is > ______, it is suggestive of Hypertrophic Cardiomyopathy.

Answer 10

> 35mm

Question 11

______ deficiency is the hallmark of Chronic Granulomatous disease, in which patients acquire recurrent infections from ______-positive organisms.

Answer 11

NADPH oxidase

Catalase-positive

Question 12

IkB is a molecule that binds and inhibits _____. In an inflammatory response, it is phosphorylated, releasing _____, promoting the production of inflammatory mediators.

Answer 12

NF-kB

NF-kB

Question 13

Acute Intermittent Porphyria results from and Autosomal _______ deficiency of the enzyme _______ ______, leading to accumulation of Porphobilinogen in the Heme synthesis pathway. Look for patients to present with dark colored urine, irrational _____, and abdominal pain after an inciting event (i.e. shortly after starting a medication.).

Answer 13

Dominant

Porphobilinogen Deaminase

Irrational Behavior

Question 14

______ is a medication that reacts with Hydrochloric Acid in the stomach to form a thick/viscous coating that binds proteins on the mucosal surface and acts as a physical buffer to acid.

Answer 14

Sucralfate

Question 15

_____ is a chemokine that promotes the chemotaxis of neutrophils and granulocytes.

Answer 15

IL-8

Question 16

Vit E deficiency can present similarly to Vit B12 deficiency, but which will present with Sideroblastic anemia and which will present with Megaloblastic anemia?

Answer 16

Vit E deficiency –> Sideroblastic

Vit B12 deficiency –> Megaloblastic

Question 17

Steroid Sulfatase deficiency can manifest as X-linked __________.

Answer 17

Ichthyosis

Question 18

Prader-Willi and Angleman syndromes are caused by deletions or mutations on Chromosome ____.

Which parental alleles are normally imprinted (silenced) in each?

Answer 18

Chromosome 15

Prader-Willi –> maternal copy normally silenced, so syndrome is from mutation or deletion of paternal copy

Opposite is true for Angleman.

Question 19

Hydroxyurea is used in the treatment of Sickle cell disease because it promotes the production of _____. How does this help?

Answer 19

HbF

Fetal hemoglobin has a greater affinity for O2 –> when Hb is Oxygenated, it is in the relaxed form (R-form) and less likely to sickle.

Question 20

Homocystinuria can be caused by a deficit in either _______ _-_____ or _______ ______.

Which will present with elevated serum Methionine?

Look for a patient with Marfanoid habitus, intellectual disability, dislocation of ocular lenses, pectus excavatum, and intravascular thrombosis.

Answer 20

Cystathione B-synthase

Methionine Synthase

Cystathione B-synthase

Question 21

_______ ___ is a neurotransmitter responsible for activating Nociceptors. Topical cream containing ______ can, over time, deplete this neurotransmitter, thereby relieving pain.

Answer 21

Substance P

Question 22

Topical _______ ointment is applied to newborn’s eyes to prevent gonococcal conjunctivitis.

Answer 22

Erythromycin