jan 25 Flashcards

1
Q

presentation of an acute episode of hereditary angioedema?

A
  • cutaneous swelling (no urticaria or pruritis)
  • colicky abdominal pain, vomiting, diarrhea
  • laryngospasm, airway obstruction
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2
Q

management of episode or hereditary angioedema?

A

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

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3
Q

first line treatment for pseudotumour cerebri?

A

acetazolamide

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4
Q

isoniazid is associated with which type of anemia?

A

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

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5
Q

causes of microcytic anemia?

A

iron deficiency
thalasemias
sideroblastic

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6
Q

what type of anemia does thalasemias cause?

A

microcytic

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7
Q

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

A

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

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8
Q

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

A

antibiotics AND renal/bladder ultrasound

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9
Q

what causes mortality in Friedreichs ataxia?

A

cardiac dysfunction - hypertrophic cardiomyopathy -> arythmias

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10
Q

presentation of friedreichs ataxia?

A

-progressive gait ataxia and dysarthria

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11
Q

what age does friedreichs ataxia usually present?

A

adolescence or young adulthood

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12
Q

path of friedreichs ataxia?

A

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

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13
Q

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

A

hepatic hydrothorax (pleural effusion)

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14
Q

path of hepatic hydrothorax?

A

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

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15
Q

hepatic hydrothorax usually occur on the ___ side

A

right - due to weaker diaphgram

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16
Q

does crigler-najar result in conjugated or unconjugated hyperbilirubinemia?

A

UNconjugated

17
Q

when does galactosemia present?

A

first few days of life

18
Q

presentation of galactosemia?

A
  • poor feeding and vomiting

- jaundice, hepatomegaly, cataracts (galactose accumulation)

19
Q

high galactose may impair…

A

leukocyte function -> increased risk of E coli sepsis

20
Q

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

A

the high levels of galactose impair leukocyte defense

21
Q

what causes the unconjugated hyperbilirubinemia in galactosemia?

A

galactose accumulation in RBCs -> lysis

22
Q

lab findings in galactosemia?

A
  • increased bilirubin, AST and ALT
  • low glucose
  • metabolic acidosis
    • urine reducing substance
23
Q

class I antiarythmics block __ channels

A

Na

24
Q

flecainamide is a class …

A

1C

25
Q

flecainamide exhibits use dependence T or F

A

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

26
Q

initial management of uncomplicated lumbosacral radiculopathy (sciatica)?

A
  • activity modification

- NSAIDs

27
Q

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

A
  • those with SIGNIFCANT/progressive or bilateral neurologic defects
  • suspected malignany or epidural abcess
28
Q

how does respiratory alkolosis effect Ca in the blood?

A

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

29
Q

which form of ca is active?

A

ionized calcium

30
Q

difference between esophageal dysphagia and oropharyngeal dysphagia?

A

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

31
Q

work up for OROPHARYNGEAL dysphagia?

A

videofluroscopic modified barium swallow

32
Q

etiology of retinitis pigmentosa?

A

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

33
Q

when does retinitis pigmentosa present?

A

symptom onset may start at age 10 and up

34
Q

clinical features of retinitis pigmentosa?

A
  • night blindness
  • progressive visual field loss
  • decreased visual acuity
35
Q

fundoscopy findings of retinitis pigmentosa?

A
  • retinal vessel attenuation
  • optic disc pallor
  • abnormal retinal pigmentation
36
Q

the cornea apperas normal in Vit A deficiency TRUE OR FALSe

A

FALSE - it will appear very dry

37
Q

clinical manifestations of x-linked agammaglobulinemia?

A
  • recurrent sinopulmonar infections and GI infections
  • chronic enterovirus infection
  • small of absent lymph tissues
38
Q

presentation of acute phenytoin toxicity?

A

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

39
Q

exam findings of acute angle glaucoma?

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