NTK_FCR Progress Quizzes

Question 1

What is the most common primary intraocular tumor in peds?

Answer 1

retinoblastoma; mut. in RB1 gene.

no red reflex; exophytic translucent mass adherent to the retina of the right eye.

(d/t to increase elongation factor 3 (EF2) activity)

Question 2

What is the MOA of the Rb protein?

Answer 2

key reg. of the G1–> S cell cycle.

Rb protein is a Tumor suppressor which halts cell cycle progression by inhibiting elongation factor 2 (E2F).

Question 3

How are Rb protein and cyclin-CDK complexes related?

Answer 3

Cyclin-CDK complexes INHIBITS Rb protein.

Cyclin-CDK are pro-progression for the G1–>S transition.

Question 4

Mutation in ___ protein allows defective G1–> S regulation, allowing cells with mutation to progress thru cell cycle unrestricted.

Answer 4

Rb Protein;

–> Retinoblastoma in Peds

Question 5

primary sclerosing cholangitis is associated with what other disease?

Answer 5

ulcerative colitis.

80% of patients with primary sclerosing cholangitis have ulcerative colitis, and as many as 5% of patients with ulcerative colitis develop primary sclerosing cholangitis.

Question 6

PBS is more common in what gender?

Answer 6

Women.

Fat, fertile, female, forty = PBS.

Question 7

Which phase of drug metabolism can involve acetylation, glucuronidation, and sulfation.

Answer 7

Phase II metabolism can involve acetylation, glucuronidation, and sulfation.

Question 8

Morphine undergoes what type of metabolism?

Answer 8

Phase 2, glucuronidation via UDP-glucuronosyltransferase.

Question 9

Alcohol dehydrogenase mediates oxidation, in what phase?

Answer 9

phase I reaction

Question 10

Oxidation and reduction are reactions of what phase of metabolism?

Answer 10

Phase 1

Question 11

What ABX prevents peptide bond formation by preventing translocation and the hydrolysis of the second GTP?

Answer 11

Macrolides

Question 12

echocardiographic finding of an asymmetric hypertrophied interventricular septum ==> ?

Answer 12

hypertrophic cardiomyopathy (HCM)

Question 13

hypertrophic cardiomyopathy (HCM) is ~w/ what genetic disease?

Answer 13

Friedreich’s ataxia, an autosomal recessive genetic disorder

Question 14

Child with extremely pale skin, white hair, and light blue eyes; complaining o burning pain in his hands and feet. ==> ???

Answer 14

Chediak-Higashi syndrome. (AR)

Chediak-Higashi syndrome is caused by a defect in the lysosomal trafficking regulator (LYST) gene that is required for phagosome-lysosome fusion and melanosome transport, amongst other functions.

Question 15

What syndrome is caused by a defect in the lysosomal trafficking regulator (LYST) gene that is required for phagosome-lysosome fusion and melanosome transport, amongst other functions?

Answer 15

Chediak-Higashi syndrome

Question 16

__________________ syndrome is an X-linked disorder that causes thrombocytopenia, severe eczema, and increased susceptibility to bacterial, viral, and fungal infections. It is caused by mutations in the WAS gene that encodes a protein required for reorganization of the actin cytoskeleton in multiple cells, including T cells.

Answer 16

Wiskott-Aldrich syndrome

Question 17

What is an autoimmune disorder characterized by development of IgG antibodies against the GPIIb/IIIa receptor on platelets?

Answer 17

idiopathic thrombocytopenic purpura (ITP)

Question 18

large platelets on peripherial smear, normal PT and aPTT; Intracranial hemorrhage.

Answer 18

idiopathic thrombocytopenic purpura (ITP)

Pt. will have normal hematocrit, normal renal function and no schistocytes on peripheral smear.

Question 19

Tx of idiopathic thrombocytopenic purpura (ITP)?

Answer 19

IVIG

Question 20

What makes

Thrombotic thrombocytopenic purpura (TTP) different from idiopathic thrombocytopenic purpura (ITP).

Answer 20

TTP causes microangiopathic hemolytic anemia, renal failure, encephalopathy, and fever.

Question 21

What is the rate of elim of 100mg vs. 500mg of Aspirin?

Answer 21

Rate of elim will be the SAME for both.

Aspirin is one of the few drugs that have ZERO-ORDER kinetics.

Question 22

When a pt. aspirates something, which lung will it most likely end up in?

Answer 22

superior portion of the right lower lobe;

the right mainstem bronchus is more vertical and wider than the left.

Question 23

What type of diuresis is caused by Glucosuria?

Answer 23

Osmotic diuresis ==> ecretion of additional water along with glucose.

the water loss from plasma increase the conc. of Na+

Question 24

positive HBsAg

positive anti-HBc

negative anti-HBs

negative IgM anti-HBc

Answer 24

Chronic HBV infection

Question 25

What is the sole marker of HBV infection during the “window period” between disappearance of HBsAg and production of anti-HBs?

Answer 25

HBV core immunoglobulin M (IgM anti-HBc)

Question 26

Elevated aldosterone also causes potassium (K+) and hydrogen (H+) wasting in the cortical collecting ducts, leading to what?

Answer 26

hypokalemia and a metabolic alkalosis.

Question 27

Hypokalemia can lead to muscle weakness as well as electrocardiogram changes seen as what?

Answer 27

U-waves

Question 28

Primary hyperaldosteronism (Conn Syndrome) is associated with what? (5)

Answer 28

• hypokalemia
• metabolix alkalosis
• hypokalemia
• elevated aldosterone levels
• hypocalcemia

Question 29

he typical presentation of _________ is a child with a swollen belly due to ascites (accumulation of fluid in the peritoneal cavity), dilated loops of bowel, and an enlarged, fatty liver.

Answer 29

kwashiorkor.

fatty liver is d/t to decrease apoplipoprotein synthesis.

Question 30

Aqueous humor of the eye is produced by what?

Answer 30

ciliary body

Question 31

What is involved in accommodation, in which parasympathetic innervation causes a decreases in tension on the lens?

Answer 31

What is involved in accommodation, in which parasympathetic innervation causes constriction of the ciliary body and decreases tension on the lens.

Question 32

In response to changes in lighting, the iris constricts or dilates. What structure causes this?

Answer 32

The sphincter pupillae muscles are circular muscles around the iris that constrict in response to increased light, decreasing the pupil diameter.

The iris dilator muscle (pupillary dilator) is a smooth muscle that runs radially in the iris. In response to decreased light, the dilatory muscle contracts, causing the pupil to dilate.

Question 33

MOA of Clopidogrel

Answer 33

Clopidogrel inhibits platelet aggregation by irreversibly blocking ADP receptors on the platelets. This prevents expression of glycoproteins IIb/IIIa on the platelet surface, which is necessary for platelets to aggregate.

Question 34

What disease is a defect in platelet-to-platelet aggregation secondary to a decrease or defect in GpIIb/IIIa.

Answer 34

Glanzmann thrombasthenia

Question 35

___________ syndrome is a defect in platelet plug formation secondary to a defect or deficiency of GpIb. GpIb is a platelet surface protein which binds directly to vWF, which then binds to subendothelial collagen, to form the platelet plug. Clopidogrel does not affect GpIb expression.

Answer 35

Bernard-Soulier syndrome.

Question 36

galactosemia is a result of a deficiency in what enzyme?

Answer 36

galactose-1-phosphate uridyltransferase.

Laboratory findings include: direct hyperbilirubinemia, elevated transaminases, prolonged prothrombin and partial thromboplastic times, hypoglycemia, metabolic acidosis, a positive urine test for reducing substances, and a negative urine test specific for glucose.

Question 37

Alpha-iduronidase is the enzyme most commonly associated with ?

Answer 37

Hurler’s Syndrome

Question 38

Beta-glucosidase is the enzyme most commonly associated with ?

Answer 38

Gaucher’s Disease

Question 39

Cystathionine synthase is the enzyme most commonly associated with ?

Answer 39

Homocysteinuria

Question 40

Phenylalanine hydroxylase is the enzyme most commonly associated with ?

Answer 40

Phenylketonuria (PKU)

Question 41

Ornithine transcarbamylase is the enzyme deficiency most commonly associated with ?

Answer 41

Ornithine Transcarbamylase Deficiency

Question 42

presents radiographically as subcortical white matter lesions similar to multiple sclerosis, but with a much more rapid onset and more confluent appearance.

???

Answer 42

progressive multifocal leukoencephalopathy (PML);

d/t JC virus

Question 43

HIV+ pt. well-controlled. CD4 count 400. S/Sx of fever, cough, chest pain, with consolidation in the right lower lobe. What is the bug that caused this?

Answer 43

S. pneumoniae

Question 44

Patients with HIV have increased susceptibility to fungal pneumonias, like Coccidiomycosis, at a CD4 cell count of what?

Answer 44

CD4 cell count <100/μl.

Question 45

Anything that decreases blood flow to the kidneys causes what type of AKI?

Answer 45

pre-renal AKI

Question 46

What are the renal labs of pre-renal AKI?

Answer 46

Urine Osmol > 500

Urine Sodium < 20

BUN:Cr > 20

FENa < 1

Question 47

What is a post mastectomy complication that leads to a “winged scapula” that is not an injury to the long thoracic n.?

Answer 47

Lymphedema of the arm.

Pt. with “winged scapula” cannot abduct their arm.

Question 48

EEG showing the characteristic triphasic spike-wave complexes and CSF positive for protein 14-3-3 ==> ???

Answer 48

Creutzfeldt-Jakob Disease (CJD), a prion disease that causes rapidly progressive dementia and ultimately death

Question 49

__________ are a Gram-positive anaerobes that grow as cocci in 6.5% NaCl and are bile esculin positive.

Answer 49

Enterococci spp.

Question 50

What nerve is the EFFERENT limb of the cremasteric reflex?

Answer 50

genitofemoral nerve

In males, the “genito” portion of the nerve travels with the spermatic cord to innervate the cremaster muscle and the anterior scrotal skin. The cremaster muscle is responsible for the elevation of the scrotum seen during the cremasteric reflex.

Question 51

What junction is located where the ureter joins the bladder?

Answer 51

uretrovesical junction (UVJ) is located where the ureter joins the bladder and is the second most common site of obstruction leading to prenatal hydronephrosis.

Question 52

The _____________________ is located where the pelvis of the kidney meets the ureter and is the most common site of obstruction leading to hydronephrosis in fetal life, infants, and children

Answer 52

ureteropelvic junction (UPJ)

The UPJ is the last portion of the ureter to canalize in development, which may contribute to the relatively increased incidence of UPJ obstruction.

Question 53

deficit of dopaminergic actvity within the nigrostriatal pathway is responsible for ???

Answer 53

the symptoms of Parkinson’s disease and other parkinsonian syndromes.

Question 54

What enzyme needs the same cofactors are required by pyruvate dehydrogenase, which converts pyruvate to acetyl-CoA?

Answer 54

branched-chain α-keto acid dehydrogenase complex (BCKD)

BCKD is responsible for the metabolism of the branched chain amino acids leucine, valine, and isoleucine, as well as odd-numbered fatty acids.

As a result, these metabolites build up in the system. Signs and symptoms of maple syrup urine disease include encephalopathy and a characteristic “Maple Syrup” smell in the urine.

Question 55

Psychiatric effects include paranoia, hallucinations, euphoria, and aggressive and erratic behavior with a diminished or seemingly absent sensation of pain. Heart rate and blood pressure are often elevated.

What drug-intoxication?

Answer 55

phencyclidine (PCP, also known as “angel dust”).

Complications of PCP use include seizures, rhabdomyolysis, and subsequent depressed level of consciousness that can progress to coma.

Question 56

PUD affecting the greater curvature is more likely to be related to ?

Answer 56

NSAIDs, tumors, and infections.

H. pylori = first part of duodenum!

Question 57

treat bradycardia and hypotension in cases of beta-blocker ingestion/overdose?

Answer 57

High-dose glucagon