jan 17

Question 1

what is a vascular ring?

Answer 1

-congenital malformation of vessels that encircles/compresses the trachea and or esophagus

Question 2

what makes symptoms of a vascular ring worse?

Answer 2

activity (better with rest)

Question 3

presentation of a vascular ring?

Answer 3

biphasic stridor that improves with neck extension

Question 4

presentation of atlanto-axial instability:

Answer 4

weakness, gait changes, urinary/fecal incontinence, dizziness, vertigo, diplopia, upper motor neuron findings

Question 5

atlantoaxial instability is a common complication of…

Answer 5

down syndrome

Question 6

upper motor neuron findings are present in atlantoaxial instability TRUE OR FALSE

Answer 6

TRUE

Question 7

upper motor neuron findings are present in tethered spinal cord TRUE OR FALSE

Answer 7

FALSE

Question 8

tethered spinal cord is associated with down syndrome TRUE OR FALSE

Answer 8

FALSE

Question 9

tethered spinal cord is associated with…

Answer 9

spina bifida

Question 10

optic neuritis is a common manifestation of..

Answer 10

MS

Question 11

presentation of retinal detachment?

Answer 11

floaters or flashes of light and loss of peripheral vision

Question 12

What is Dubin-Johnson syndrome?

Answer 12

-conjugated hyperbilirubinemia due to defective liver excretion

Question 13

is Dubin-Johnson syndrome serious or benign?

Answer 13

benign

Question 14

Path of Gilbert syndrome?

Answer 14

Mildly decreased UDP-glucuronyltransferase conjugation and impaired bilirubin uptake -> mild jaundice with stress/fasting

Question 15

does gilbert syndrome have high UNconjugated or CONJUGATED bilirubin?

Answer 15

unconjugated

Question 16

Path of Crigler-Najar syndrome type I

Answer 16

ABSNET UDP-glucuronyltransferase -> jaundice, kernicteris and death within first few years of life

Question 17

is there high UNconjugated or CONJUGATED bilirubin in Crigler-Najar?

Answer 17

UNCONJUGATED

Question 18

presentation of pagets disease

Answer 18

bone pain, headaches, hearing loss, femoral bowing

Question 19

how to treat uric acid kidney stones

Answer 19

alkalisation of urine and low purine diet

Question 20

what drug can be used to alkalize the urine?

Answer 20

potassium citrate

Question 21

how do thiazide diuretics effect risk of uric acid kidney stones?

Answer 21

increase risk from increased uric acid in urine

Question 22

renal complications of acyclovir?

Answer 22

crystal-induced acute kidney injury -> renal tubular obstruction

Question 23

what drugs are associatd with acute interstital nephritis?

Answer 23

beta lactams

PPIs

Question 24

presentation of acute interstitial nephritis?

Answer 24

7-10 days after drug exposure -> skin rash, eosinophilia, pyuria

Question 25

when should routine vaccinations be given to premature infants?

Answer 25

based on chronological age (NOT CORRECTED AGE)

Question 26

beta blocker effects on liver?

Answer 26

prevent glucose production/glycogen breakdown

Question 27

what is clonidine?

Answer 27

alpha 2 agonist