Uworld: 5-15

Front 1

Cardiac effects of abnormal neural crest migration.

Back 1

Tetralogy of Fallot
Transposition of great vessels
Truncus Arteriosus

Front 2

Cardiac features of Tetralogy of Fallot

Back 2

PROVe
1) Pulmonary stenosis
2) RVH
3) Overriding Aorta
4) Ventricular Septal Defect

Front 3

Why does squatting improve symptoms of cyanosis?

Back 3

Inc'd systemic pressure reduces amount of R to L shunting through VSD (decreasing amount of blood bypassing the lungs)

Front 4

5 year-old male
Cyanosis with minimal exertion
Occasional cyanosis since birth
Squatting relieves cyanosis

Diagnosis

Back 4

TOF

Cyanotic episodes since birth excludes L to R shunt

Front 5

Back 5

Caseating granuloma

Front 6

Oral flora:
Examples
Conditions prone to aspiration

Back 6

Bacteroides
Fusobacterium
peptostreptococcus

Risk factors for aspiration:
LOC
EtOH
Seizure disorders
Prolonged anesthesia
Severe neuro dz

Front 7

Listeria:
Gram -/+
Shape
Mode of infection
Effects

Back 7

Gram poz
Rod

Transmitted through food and causes spesis, meningitis in IMMUNOCOMP'D ADULTS.

Can also cause neonatal meningitis (transplacental or vaginal contact during labor)

Listeria grows well in cold temperatures (cold enrichment)-->contaminate refrigerated food

Front 8

Tumbling motility
Gram positive
Rod

Back 8

Listeria

Front 9

Unpasteurized milk

Back 9

Listeria

Front 10

Sickle Cell Anemia:
Mode of inheritance

Back 10

AR

Front 11

34 year-old male
HIV positive
Fever, chills, pleuritic chest pain
Bronchial breath sounds
CD4 count = 800

Diagnosis
Cause

Back 11

Pneumonia:
Strep pneumo

Not Pneumocystis jiroveci bc CD count is too high (need to be <200 to worry about PCP)

Front 12

Where should an injection to the gluteal injection be given?
Why?
Risks?
What would you see if you didn't?

Back 12

Should inject superlateral quadrant to avoide damage to GLUTEAL and SCIATIC NERVES.

If injure superior gluteal-->hip will dip downward when ipsilateral foot lifted off ground.

Front 13

Pterion:
What is it?
Effects of fracture?

Back 13

Pterion = where frontal, parietal, temporal, and sphenoid bones meet (near the temples)

This bone is thin and the Middle Meningeal Artery courses deep to it (risk epidural hematoma!)

Note: The middle meningeal artery is a branch of the MAXILLARY artery, which is a branch of the external carotid.

Front 14

Diagnosis
Cause

Back 14

Broad nonseptate hyphae

Mucormycosis:
Caused by
-Rhizopus
-Mucor
-Absidia

Patient is either DM or immunocomp'd

Front 15

Major determinant of risk for osteoporotic fractures

Back 15

Bone Mass (increases during childhood, adolescent; peaks between 20 and 30)

GENETIC FACTORS responsible for variation among individuals

Front 16

Widened pulse pressure

Back 16

Aortic regurgitation

Widened pulse pressure causes INVOLUNTARY HEAD BOBBING

Front 17

This anti-HLD drug can cause gout.

Back 17

Niacin (nicotinic acid)

Front 18

This anti-HLD drug can cause hepatitis.

Back 18

Statins
Niacin (nicotinic acid)

Front 19

This anti-HLD drug can cause gallstones.

Back 19

Fibrates

Front 20

When is metformin contraindicated?

Back 20

In those that would be prone to developing lactic acidosis:
-Renal failure
-Liver failure/alcoholics
-Heart failure
-ETOH

Front 21

What is the cellular effect of progesterone withdrawal on the endometrium?

Back 21

APOPTOSIS

not atrophy!!!

Front 22

What are the most important mediators of coronary vascular dilation?

Back 22

Nitric Oxide (acts in large arteries and pre-arteriolar vessels)

Adenosine (byproduct of ATP metabolism--acts as vasodilatory element in small coronary arterioles)

Front 23

Anti-Polyribosyl-ribitol-phosphate antibodies

Back 23

Protection against H. flu type B (Hib)

Hib vaccine is composed of polyribosyl-ribitol-phosphate (component of Hib capsule) conjugated w/diphtheria or tetanus toxoid

Front 24

Antistreptolysin O antibodies

Back 24

a/w recent Strep infection

Front 25

Osteomyeolitis in health children/adults

Back 25

Staph aureus

Front 26

Beginning from within the fibroblast, describe the steps of collagen synthesis.

Back 26

Pro alpha-collagen
Hydroxylation of proline/lysine select residues
Glycosylation of select lysine residues
Assembly of pro-alpha chains into triple helix

This is known as PROCOLLAGEN; secreted in Golgi-->ECM

N/C propeptide terminals cleaved to become collagen FIBRIL

These fibrils are then covalently cross-linked by lysyl oxidase

Front 27

PAH:
How is it treated by the kidney?
Describe saturable processes.

What is it a good estimate of?

Back 27

PAH is both filtered from the blood and secreted by the glomerulus/renal tubules

Clearance of PAH ~ RPF

Filtration of PAH CANNOT be saturated, however, its secretion into tubular fluid (from peritubular capillaries) can be saturated as it is carrier-mediated.

Front 28

ADH:
Receptors

Back 28

V2 receptors (G-protein coupled!)--allow for aquaporins to be expressed in CD

Front 29

Diabetes Insipidus:
Central vs Nephrogenic
Complete vs Partial Central

Back 29

If during water deprivation and following injection of vasopressin:
-Urine osmolality changes less than 10%-->nephrogenic DI (kidneys not responding to hormone)

Central:
See an increase in urine osmolality
Due to lack of vasopressin

Complete central: osmolality increases more then 50% following vasopressin injection

A more moderate response would indicate partial central DI (some vasopressin present, but not enough to allow normal kidney fn)

Front 30

Anterior Cerebral Artery:
Region of brain supplied
Effect of occlusion

Back 30

Supplied MEDIAL region of ipsilateral hemisphere from frontal pole to parietoccipital sulcus

Occlusion-->lose motor function of CONTRALATERAL leg/foot

Would also see sensory deficits in contralateral legs, trunk, genitals

May also see behavior/mood changes due to injury of frontal lobe structures

Front 31

Middle Cerebral Artery:
Effect of occlusion

Back 31

Motor control of hand, head, neck

Front 32

Alendronate:
Drug Class
Structural similarities

Back 32

Bisphosphonate

Structurally similar to pyrophosphate--an important component of hydroxyapatite

Front 33

Why must patients remain upright after taking bisphosphonates?

Back 33

Bisphosphonates are poorly absorbed by GI tract. Need to be administered in fasting state with plenty of water.

Must stay upright to prevent reflux bc bisphosphonates are caustic to the esophagus.

Front 34

Enterococci:
Methods of resistance and associated drugs

Back 34

1) Produce beta-lactamase (PCNs)
2) Low affinity PCN binding protein (AminoPCNs, tazobactam)
3) Contain enzymes in cytoplasm that transfer different chemical groups (acetyl, adenyl, phosphate groups) onto exogenous substances, i.e., aminoglycosides (can no longer bind ribosomes!)

Front 35

Xanthomas:
Underlying abnormality

Back 35

LDL receptor abnormality

Front 36

What are the NON-noradrenergic innervations of the sympathetic nervous system?

Back 36

Sweat glands (ACh)
Renal vasculature (D1)

Front 37

What combination of anti-HLD drugs increases the risk of myopathy?

Back 37

Statin + fibrate

Front 38

What combination fo anti-HLD drugs increases the risk for cholesterol gallstones?

Back 38

Fibrates + bile acid resins (due to inc'd cholesterol concentration in bile)

Front 39

Which antiarrhythmics have highest selectivity for ischemic myocardium compared to normal cardiac tissue?

Why?

Back 39

Class IB antiarrhythmics: TLM: Tocainide, Lidocaine, Mexiletine

This drugs depress conduction in rapidly depolarizing and depolarized myocardial cells as are found in ischemic myocardium.

Front 40

Which antiarrhythmics are useful for suppressing arrhythmias arising from centers of normal automaticity?

Back 40

IA: PDQ: Procainamide Disopyramide Quinidine

Front 41

Anti-depressant causing priapism.

Back 41

Trazodone

Front 42

Alcohol-induced hepatic steatosis:
Pathophys

Back 42

Dec'd FFA oxidation secondary to excess NADH production by Alcohol DH and Aldehyde DH

Front 43

RANK-ligand:
Produced by
Function
Effects/Times of Overexpression

Back 43

RANK-ligand produced by obsteoblasts

Binds RANK to form and differentiate osteoclasts.

Overexpression in times of LOW ESTROGEN cause inc'd oclast activity

Front 44

Osteoprotegrin:
Secreted by
Role

Back 44

Secreted by osteoblasts

Acts as decoy receptor for RANK-ligand and prevents it from activating formation of oclasts.

Front 45

Carcinoid tumors:
Benign vs Metastatic Presentation

Back 45

Benign carcinoid tumor confined to intestine and its secretory products are metabolized by liver, so NO CLINICAL MANIFESTATIONS

If tumor mets to liver or extra-intestinal site, liver can't metabolize vasoactive secretory products and carcinoid syndrome ensues

Carcinoid syndrome presentation:
Cutaneous flushing
Dizziness
Secretory diarrhea
Crampy abdominal pain
Dyspnea w/wheezing
Pulmonary/tricuspic valvular heart dz

Front 46

Elevated H-IAA

Back 46

5-HIAA is a serotonin metabolite and may indicate carcinoid syndrome

Front 47

People with diabetes are most likely to die from _________.

Back 47

Coronary heart disease (HEART ATTACK)

Front 48

How does estrogen affect cholesterol synthesis?

How can this lead to gallstones?

Back 48

Estrogen increases cholesterol synthesis by upregulating hepatic HMG-CoA reductase

Causes bile to become supersaturated with choelsterol

Front 49

How does progesterone affect gallstone formation?

Back 49

PG reduces bile acid secretion and slows gallbladder emptying-->cholesterol (elevated bc of estrogen) prcipitates into insoluble crystals that eventually form gallstones

Front 50

Diminished femoral pulses compared to brachial pulses

Back 50

aortic COARCTATION

Front 51

Marfan's Syndrome:
Heart defect

Back 51

Aortic ANEURYSM
MV prolapse

Front 52

Turner's Syndrome:
Heart defect

Back 52

aortic COARCTATION

Front 53

Tuberous Sclerosis:
Symptoms
Heart Defect

Back 53

Ash leaft spots
Brain hamartomas
b/l Smooth muscle lzns on kidneys/liver/pancreas

VALVULAR OBSTRUCTION due to cardiac RHABDOMYOMAS

Front 54

Down Syndrome:
Heart Defect

Back 54

Endocardial cushion defect (ostium primum ASD)

Front 55

Friedrich's Ataxia:
Symptoms
Heart Defect

Back 55

Spiniocerebellar degeneration with ataxia-->difficulty walking

A/w HYPERTROPHIC CM

Front 56

Identify stage

Back 56

Left: Schizont

Right: Merozoites (ready to pop!)

Front 57

What is the effect of vasopressin on the collecting duct?

Back 57

V2 receptor-mediated increase on permeability to water and UREA.

Will see decrease in renal clearance of urea from plasma.

Front 58

Normochromic, normocytic anemia
Back pain
Elevated gamma-globulin

Diagnosis
Pathophys
Additional Lab Findings

Back 58

Multiple myeloma:
Neoplastic B cells mature into plasma cells that synthesize abnormal amounts of immunoglobulin. Leads to anemia (normochromic, normocytic), lytic bone lesions classically affecting vertebral column (back pain), hypercalcemia

Will also see ELEVATED GAMMA-GLOBULINS (M peak)

Front 59

42 year-old female
Spinning sensation a/w nausea and ringing in left ear
Feels fine between episodes
Left ear hearing intact

Diagnosis
Pathophys

Back 59

Menière's Dz:
Inc'd volume of endolymph due to defective resorption. Leads to damage of versitbular and cochlear components of inner ear.

Char'd by:
-Tinnitus
-Vertigo
-Hearing loss

Front 60

Why do myocardial cells increase in size in response to ischemia?

Back 60

Ion pump failure due to ATP deficiency causes intracellular accumulation of Na+ and Ca2+

Inc'd intracellular solute draws free water into cell-->cellular/mitochondrial swelling

Front 61

Back 61

Glioblastoma:
Arises in cerebral hemispheres
Area of central necrosis
Hemorrhage
Poorly defined

Front 62

Back 62

Meningioma: derived from arachnoid cells
Well circumscribed
Located on brain surface
Second most common brain tumor (primary)

Front 63

Back 63

Acoustic neuroma--type of scwannoma that arise from Schwann cells of CN VIII.
Found at cerebellopontine angle.

Front 64

What is the most common brain tumor?

Back 64

Mets from other cancers (lung, breast, kidney, skin--melanoma)

Front 65

What is the most common PRIMARY brain tumor?

Back 65

Glioblastoma multiforme (terrible prognosis)

Front 66

Brain tumor that arises from arachnoid cells.

Back 66

Mengioma