jan 25

Question 1

presentation of an acute episode of hereditary angioedema?

Answer 1

• cutaneous swelling (no urticaria or pruritis)
• colicky abdominal pain, vomiting, diarrhea
• laryngospasm, airway obstruction

Question 2

management of episode or hereditary angioedema?

Answer 2

-C1 inhibitor concentrate, bradykinin antagonist, or kallikrein inhibitor
(most resolve on their own but treat to prevent laryngeal swelling)

Question 3

first line treatment for pseudotumour cerebri?

Answer 3

acetazolamide

Question 4

isoniazid is associated with which type of anemia?

Answer 4

aquired sideroblastic (microcytic) anemia due to its anti-pyroxidine effects

Question 5

causes of microcytic anemia?

Answer 5

iron deficiency
thalasemias
sideroblastic

Question 6

what type of anemia does thalasemias cause?

Answer 6

microcytic

Question 7

what may. be increased in someone with iron deficiency anemia?

Answer 7

platelets - a reactive thrombocytosis may occur in response to low red blood cell count

Question 8

A child <2 years old has a UTI. What is indicated?

Answer 8

antibiotics AND renal/bladder ultrasound

Question 9

what causes mortality in Friedreichs ataxia?

Answer 9

cardiac dysfunction - hypertrophic cardiomyopathy -> arythmias

Question 10

presentation of friedreichs ataxia?

Answer 10

-progressive gait ataxia and dysarthria

Question 11

what age does friedreichs ataxia usually present?

Answer 11

adolescence or young adulthood

Question 12

path of friedreichs ataxia?

Answer 12

trinucleotide repeat -> loss of function mutation in frataxin -> degeneration of spinal tracts (dorsal columns, spinocerebellar)

Question 13

what type of lung complication may occur in patients with cirhosis and ascites?

Answer 13

hepatic hydrothorax (pleural effusion)

Question 14

path of hepatic hydrothorax?

Answer 14

transudative pleural effusion caused by small defects in the diagphram which allow peritineal fluid to pass through

Question 15

hepatic hydrothorax usually occur on the ___ side

Answer 15

right - due to weaker diaphgram

Question 16

does crigler-najar result in conjugated or unconjugated hyperbilirubinemia?

Answer 16

UNconjugated

Question 17

when does galactosemia present?

Answer 17

first few days of life

Question 18

presentation of galactosemia?

Answer 18

• poor feeding and vomiting

- jaundice, hepatomegaly, cataracts (galactose accumulation)

Question 19

high galactose may impair…

Answer 19

leukocyte function -> increased risk of E coli sepsis

Question 20

why are babies with galactosemia at increased risk for E coli sepsis?

Answer 20

the high levels of galactose impair leukocyte defense

Question 21

what causes the unconjugated hyperbilirubinemia in galactosemia?

Answer 21

galactose accumulation in RBCs -> lysis

Question 22

lab findings in galactosemia?

Answer 22

• increased bilirubin, AST and ALT
• low glucose
• metabolic acidosis
• • urine reducing substance

Question 23

class I antiarythmics block __ channels

Answer 23

Na

Question 24

flecainamide is a class …

Answer 24

1C

Question 25

flecainamide exhibits use dependence T or F

Answer 25

TRUE - higher heart rate -> higher amount of blocked channels -> QRS widening

Question 26

initial management of uncomplicated lumbosacral radiculopathy (sciatica)?

Answer 26

- activity modification | - NSAIDs

Question 27

which patients presenting with lumbosacral radiculopathy (sciatica) require MRI?

Answer 27

- those with SIGNIFCANT/progressive or bilateral neurologic defects - suspected malignany or epidural abcess

Question 28

how does respiratory alkolosis effect Ca in the blood?

Answer 28

Alkalosis leads to dissociation of H from albumin, leading to an increase in Ca bound albumin -> decreased ionized Ca

Question 29

which form of ca is active?

Answer 29

ionized calcium

Question 30

difference between esophageal dysphagia and oropharyngeal dysphagia?

Answer 30

oropharyngeal may have difficulty initiating swallow, coughing, nasal regurgitation whereas esophageal does not

Question 31

work up for OROPHARYNGEAL dysphagia?

Answer 31

videofluroscopic modified barium swallow

Question 32

etiology of retinitis pigmentosa?

Answer 32

-genetic mutation -> loss of photoreceptors -> progressive retinal degerenation

Question 33

when does retinitis pigmentosa present?

Answer 33

symptom onset may start at age 10 and up

Question 34

clinical features of retinitis pigmentosa?

Answer 34

- night blindness - progressive visual field loss - decreased visual acuity

Question 35

fundoscopy findings of retinitis pigmentosa?

Answer 35

- retinal vessel attenuation - optic disc pallor - abnormal retinal pigmentation

Question 36

the cornea apperas normal in Vit A deficiency TRUE OR FALSe

Answer 36

FALSE - it will appear very dry

Question 37

clinical manifestations of x-linked agammaglobulinemia?

Answer 37

- recurrent sinopulmonar infections and GI infections - chronic enterovirus infection - small of absent lymph tissues

Question 38

presentation of acute phenytoin toxicity?

Answer 38

Cerebellar dysfunction: horizontal nystagmus, ataxia, dysmetria, slurred speech, etc.

Question 39

exam findings of acute angle glaucoma?

Answer 39

- conjunctival injection - corneal edema - palpable firmness of eyeball - fixed, mid-dilated pupil