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1

What class of drugs can be used to treat estrogen receptor-positive breast cancer? What is another use for these drugs? What is the main concern when using tamoxifen? (FA15 p412) (FA16 p413)

Selective estrogen receptor modulators (SERMs)—receptor antagonists in breast and agonists in bone. Block the binding of estrogen to ER ⊕ cells.
(Tamoxifen, raloxifene)
-Raloxifene also useful to prevent osteoporosis.
-Tamoxifen—partial agonist in endometrium, which  the risk of ENDOMETRIAL CANCER; “hot flashes.” (not seen with Raloxifene, estrogen rec. antagonist in endometrial tissue)

2

How does an anterior shoulder dislocation present differently than a posterior shoulder dislocation? :
Arm position
Neurovascular compromise
Classic scenario
Physical exam

ant: external rotation, slight abduction, axillary n./a., due to blow to arm while abducted, externally rotated, extended, if thin may palpate prom. acromion process, loss of shoulder roundness, appears angular


post: internal rotation, adduction, unable to externally rotate arm, unusual to have nv deficits, caused by seizure/electrocution, bulge in post. shoulder area, ant. aspect of shoulder is relatively flat

3

Which primary bone tumor fits each of the following descriptions? (FA15 p401, 428) (FA16 p401, 429)
Most common malignancy arising within bone
 11;22 translocation
 Soap-bubble appearance on x-ray
 Onion-skin appearance of bone (layers of new bone in periosteum)

-Most common malignancy arising within bone: multiple myeloma
-11;22 translocation: Ewing sarcoma (11 + 22 = 33 (Patrick Ewing’s jersey number).
-Soap-bubble appearance on x-ray: giant cell tumor (osteoclastoma)
-Onion-skin appearance of bone (layers of new bone in periosteum): Ewing sarcoma

4

Which primary bone tumor fits each of the following descriptions? (FA15 p401, 428) (FA16 p401, 429)
Codman’s triangle (periosteal elevation on XR)
May arise from osteochondroma or appear as a primary tumor
 Anaplastic small cells, aggressive mets, good chemo response
 Most common malignant bone tumor in children

-Codman’s triangle (periosteal elevation on XR): Osteosarcoma
-May arise from osteochondroma or appear as a primary tumor: chondrosarcoma
-Anaplastic small cells, aggressive mets, good chemo response: Ewing sarcoma
-Most common malignant bone tumor in children: osteosarcoma

5

A 45-year-old woman receives a kidney transplant and receives cyclosporine as part of her anti-rejection regimen. What is the mechanism of action of cyclosporine? (FA15 p218) (FA16 p212)

immunosuppressant: Calcineurin inhibitor; binds cyclophilin. (inactivates NFATc) Blocks *T-cell activation* by *preventing IL-2 transcription*

uses: Transplant rejection prophylaxis, psoriasis, RA

SEs: *Nephrotoxicity* HTN, hyperlipidemia, neurotoxicity, gingival hyperplasia, hirsutism.

6

You are treating a 63-year-old woman with small cell lung cancer who develops SIADH. She becomes disoriented secondary to hyponatremia. Consequently, you rapidly correct the low serum sodium with demeclocycline and water restriction. The patient’s sodium level and disorientation improve, but several days later the patient develops diplopia. What is the cause of this patient’s diplopia?
(FA15 p456) (FA16 p492)

*Massive axonal demyelination in pontine white matter* 2° to osmotic changes. Commonly iatrogenic, caused by overly rapid correction of hyponatremia.
other effects besides diplopia: Acute paralysis, dysarthria, dysphagia, loss of consciousness. Can cause “locked-in syndrome.”)

(In contrast, correcting hypernatremia too quickly results in cerebral edema/herniation)

*Correcting serum Na+ too fast:
ƒ “From low to high, your pons will die” (osmotic demyelination syndrome)
ƒ “From high to low, your brain will blow” (cerebral edema/herniation)*

7

What are some of the possible causes of pericarditis? (FA15 p299) (FA16 p294)

-idiopathic (most common; presumed viral)
-confirmed infection (e.g., Coxsackievirus A or B, TB)
-neoplasia
-autoimmune (e.g., SLE, RA, scleroderma, acute RF (pancarditis)),
-uremia
-CV (acute STEMI or Dressler syndrome)
-radiation therapy, Cancer mets

8

A unilateral lesion to the lateral corticospinal tract results in motor deficits ipsilateral to the lesion. Where in the lateral corticospinal tract is the motor innervation to the legs located? (FA15 p469) (FA16 p472)

Legs (Lumbosacral) are Lateral in Lateral corticospinal, spinothalamic tracts

IN CONSTRAST: Dorsal column is organized as you are, with hands at sides. Arms outside, legs inside.

9

In cystic fibrosis thick, mucous secretions inhibit digestive enzymes, which can lead to malabsorption of fat and fat-soluble vitamins. What are the fat-soluble vitamins? What are the corresponding symptoms of fat-soluble vitamin deficiency? (FA15 p88-94, 361) (FA16 p76-81, 358)

ADEK

A: (antioxidant, visual pigment, essential for differentiation of epi cells into specialized tissue)
*Night blindness (nyctalopia); dry, scaly skin* (xerosis cutis); corneal degeneration (keratomalacia); Bitot spots on conjunctiva; immunosuppression.

D: *Rickets* in children (bone pain and deformity), *osteomalacia* in adults (bone pain and muscle weakness), hypocalcemic tetany. Breastfed infants should receive oral vitamin D. Deficiency is exacerbated by low sun exposure, pigmented skin, prematurity.

E: (antioxidant) Hemolytic anemia (*fragile RBCs*), acanthocytosis, *muscle weakness*, posterior column and spinocerebellar tract *demyelination.

K: needed for maturation of clotting factors II, VII, IX, X, (2,7,9,10) and proteins C and S. (cofactor for y-carboxylation of glutamic acid residues) Warfarin—vitamin K antagonist. deficiency: coag factor defects (blood thinning, potential hemorrhage), osteoporosis, CAD

10

A 22-year-old man recently lost his leg in a tragic accident involving alcohol and heavy machinery. Just prior to discharge, a vigilant nurse informs you that the patient has been very depressed about his lost limb, and she overheard him talking to a friend about just ending his life with his dad’s gun when he gets out of the hospital. What needs to be done? (FA15 p58) (FA16 p42)

Assess the seriousness of the threat. evaluate risk: male, depression, access to weapon, plan, etOH
If it is serious, suggest that the patient remain in the hospital voluntarily; patient can be hospitalized involuntarily if he/she refuses.

11

What branchial arch derivative abnormality causes facial abnormalities by affecting Meckel’s cartilage? What cranial nerves are affected in this abnormality? From what germ layers are the branchial arches derived? (FA15 p564-566) (FA16 p568-570)

1st arch:
Meckel cartilage: Mandible, Malleus, incus, spheno- Mandibular ligament

CN V2 and V3 "CHEW"

Muscles of Mastication (temporalis, Masseter, lateral and Medial pterygoids), Mylohyoid, anterior belly of digastric, tensor tympani, tensor veli palatini

Treacher Collins syndrome—1st-arch neural crest fails to migrate--> mandibular hypoplasia, facial abnormalities

arches derived from mesoderm (musc, arts) and neural crest (bones, cartilage)

12

truncus arteriosus gives rise to ??

Ascending aorta and pulmonary trunk

13

Bulbus cordis gives rise to ??

Smooth parts (outflow tract) of left and right ventricles

14

Primitive atrium gives rise to ??

Trabeculated part of left and right atria

15

Primitive ventricle gives rise to ??

Trabeculated part of left and right ventricles

16

Primitive pulmonary vein gives rise to ??

Smooth part of left atrium

17

Left horn of sinus venosus gives rise to ??

Coronary sinus

18

Right horn of sinus venosus
gives rise to ??

Smooth part of right atrium (sinus venarum)

19

Right common cardinal vein and right anterior cardinal vein gives rise to ??

Superior vena cava (SVC)

20

A 22-year-old man who recently immigrated from a developing country is seen at the county health clinic with a complaint of a chronic but recently worsening productive cough and sinusitis symptoms (e.g., headache). He also mentions to you privately that he and his wife have not been able to have children despite trying for two years. While you note this last complaint, you order a chest x-ray out of concern this man may have TB given his immigration status. The chest x-ray appears backwards on the display, but you realize this man’s heart is actually on the opposite side of his chest. What is the primary defect in this syndrome, and what are the characteristic symptoms/signs? (FA15 p74)
(FA16 p60)

Kartagener syndrome (1° ciliary dyskinesia)— immotile cilia due to a dynein arm defect. Results in male and female infertility due to immotile sperm and dysfunctional fallopian tube cilia, respectively;  risk of ectopic pregnancy. Can cause bronchiectasis, recurrent sinusitis, and situs inversus (e.g., dextrocardia on CXR).

21

A mutation in which proto-oncogene is most commonly associated with Hirschsprung disease?

RET gene.

22

When screening a population for a particular disease, would you rather have a high sensitivity or a high specificity? (FA15 p49) (FA16 p33) What test is used to screen for HIV? What test is used to confirm a positive HIV screen? (FA15 p168) (FA16 p158)

high sensitivity to screen

ELISA to screen (high sensitivity)
western blot to confirm (high specificity)

23

How do primary and secondary hyperaldosteronism differ in their effect on plasma renin levels? What agent is used to treat primary hyperaldosteronism? (FA15 p633) (FA16 p639)

primary: DECREASE plasma renin due to negative feedback from aldo-secreting tumor:
secondary: INCREASE plasma renin due to JG apparatus of kidney perceives low intravascular renin

Spironolactone (aldosterone antagonist)

24

Amyloidosis and sarcoidosis are associated with which cardiomyopathy? What are some other diseases or conditions associated with this cardiomyopathy? (FA15 p296) (FA16 p291)

restrictive cardiomyopathy (RCM)
other associated causes: postradiation fibrosis, endocardial fibroelastosis (thick fibroelastic tissue in endocardium
of young children)
Löffler syndrome (endomyocardial fibrosis with a prominent eosinophilic infiltrate), and hemochromatosis (dilated cardiomyopathy can also occur).

25

What does the eustachian (auditory) tube connect? What is its purpose? What drug is commonly prescribed for eustachian tube dysfunction?

connects middle ear to nasopharynx
-makes pressure of middle ear same as atmospheric
-intranasal steroids

26

What is the most common renal malignancy in adults? What is the associated gene deletion with this malignancy? (FA15 p545) (FA16 p550)

Renal cell carcinoma
-Associated with gene deletion on chromosome
3 (sporadic or inherited as von Hippel-Lindau
syndrome).
RCC = 3 letters = chromosome 3.

27

A pediatric patient presents with a noticeable right flank mass. The patient’s mother also reports blood in the patient’s urine. What malignancy would be most likely in this scenario? What is the WAGR complex? (FA15 p546) (FA16 p551)

Wilms tumor (nephroblastoma)
May be part of WAGR complex:
Wilms tumor
Aniridia
Genitourinary malformation
Retardation (mental-motor; intellectual disability)

or Beckwith-Wiedemann syndrome (Wilms tumor, macroglossia, organomegaly,
hemihypertrophy)

-“Loss of function” mutations of tumor suppressor genes WT1 or WT2 on chromosome 11.






28

A 24-year-old young man is fired from his job, and when explaining the chain of events to his roommate, he says that “it doesn’t really matter. I didn’t even need that job.” His roommate is perplexed, wondering how they will pay their rent. Of which immature ego defense could this be an example? (FA15p505)(FA16p509)

Rationalization: Proclaiming logical reasons for actions actually performed for other reasons, usually to avoid self-blame.

29

What is the definition of tidal volume, residual volume, and vital capacity? (FA15 p602) (FA16 p610)

TV: Air that moves into lung with each quiet inspiration, typically 500 mL
RV: air left after maximal expiration, cannot be measured on spirometry
VC: maximum volume of air that can be expired after maximal inspiration (TV+IRV+ERV)

RV+VC = TLC

30

What is the difference between mean, median, and mode? What are their relationships in a positively- skewed curve and a negatively-skewed curve? (FA15 p53) (FA16 p37)

mean: average (sum of values)/(total number of values)
median: middle value
mode: most common value
Positive skew: Typically, mean > median > mode. Asymmetry with longer tail on right (counterintuitive picture, hump more to left)
Negative skew: mean