Pharmacology - Croup/Cystic Fibrosis Flashcards

(45 cards)

1
Q

croup is also known as….

A

laryngotracheobronchitis

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2
Q

what is croup

A

respiratory condition triggered by acute viral infection of the upper airway

barking cough
stridor (noisy breathing sound)
hoarseness

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3
Q

what organisms can cause croup

A

a lot of them - both viruses and bacteria

bacteria are worse

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4
Q

2 types of pharmacotherapy for croup

A

epinephrine
corticosteroid

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5
Q

how long do the effects of epinephrine last for croup

A

less than 2 hours
children need to be monitored for the return of symptoms!!

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6
Q

MOA of nebulized epinephrine for croup

A

stimulates alpha receptors and thus arterioles are constricted which decreases laryngeal edema

decreased stridor and less work to breathe!

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7
Q

what ROUTE of corticosteroids are preferred

A

oral/IM are preferred over inhaled

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8
Q

most commonly used corticosteroid for croup and why

A

dexamethasone

long t1/2

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9
Q

recent studies have shown _____ to be as effective as dexamethasone with even more rapid onset

A

nebulized budesonide

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10
Q

cystic fibrosis is autosomal ______

A

recessive

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11
Q

complications of CF

A

the thick secretions obstruct the pancreas which prevents the enzymes that digest to be released and absorb food

mucus can also block the bile duct into the liver, causing permanent liver damage

diabetes, osteoporosis, reproductive problems

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12
Q

about 70% of mutations in CF patients result from deleting ____ base paris in CFTR nucleotide sequence

A

3 pairs

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13
Q

CF mutation in delta 508 results in loss of what amino acid

A

phenylalanine

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14
Q

the most common CF mutation destabilizes _____

A

NBD1

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15
Q

how many mutation classes are there in CF

A

6

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16
Q

there are 6 classes of CF mutation.
state whether they are reduced QUANTITY of CFTR protein or reduced FUNCTION

A

class 3 and 4 are reduced function

all the others are reduced quantity

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17
Q

class ____ mutation is the most common CFTR mutation worldwide

A

II (F508del)

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18
Q

as the 6 classes go up (1 ->6) what happens to the CFTR-mediated chloride transport

A

class 6 has the most CFTR mediated chloride transport. just a stability defect

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19
Q

class 1 is ___ defect
class 2 is ____ defect
class 3 is ____
4..5..6..

A

class 1 - synthesis
class 2 - processing
class 3 - gating
class 4 - conductance
class 5 - splicing
class 6 - stability

20
Q

how is the skin affected by CF

21
Q

3 body areas for CF treatment

A

tracheobronchial tree
pancreas
GI tract

22
Q

what is pulmozyme

A

a mucus thinning drug that reduces the number of lung infections in CF patients and improves lung function

23
Q

what type of mutation is for ivacaftor for

24
Q

ivacaftor brand name

25
MOA of ivacaftor in CF
increases the probability of the channel opening therefore, increased chloride ion transport in class III mutation
26
AE of ivacaftor
abdominal pain, dizziness, upper RT reactions
27
tezacaftor/ivacaftor function
improved function of CFTR channel/protein
28
lumacaftor MOA
increases amount of CFTR channel at the cell membrane bc prevents misfolding of CFTR proteins (chaperone function)
29
lumacaftor/ivacaftor is both a ____ and ____ of the CFTR protein
corrector and potentiator
30
ivacaftor improves....
CFTR channel function
31
explain how tezacaftor/ivacaftor work together
ivacaftor - helps the proteins stay open longer tezacaftor brings more CFTR proteins to the cell surface
32
explain how lumacaftor/ivacaftor work together
ivacaftor helps the channel stay open longer lumacaftor brings more CFTR proteins to the cell surface - more channels!
33
______ is a CF drug that is a strong INDUCER of CYP3A4 and is also hepatotoxic
lumacaftor
34
what drug is produced in chinese hamster ovary cells
dornase alpha
35
dornase alpha (pulmozyme) is a highly purified solution of....
recombinant human DNase (an enzyme which selectively cleaves DNA)
36
MOA of dornase alpha (pulmozyme) in CF
hydrolyzes the DNA in the mucus of CF patients thus reduces the mucus viscosity in the lungs which improves clearance
37
AE of pulmozyme (dornase alfa)
change/loss of voice discomfort in throat chest pain red eyes
38
dornase alfa has been shown to reduce the risk of.......
respiratory tract infections requiring IV antibiotics
39
what is the primary molecular defect in cystic fibrosis patients
improper folding and degradation of CFTR protein before it reaches the membrane
40
how does pulmozyme improve respiratory symptoms in CF patients
breaks down DNA in the mucus to decrease viscosity
41
what inhaled antibiotic is used in CF to treat p. aeruginosa infection
tobramycin (p. aeruginosa is common in 10 yrs and up)
42
a patient with CF is homozygous for the delta F508 mutation. despite receiving standard mucolytic therapy, their lung function remains poor. which therapeutic strategy best addresses the ROOT CAUSE of the patient's condition
give lumacaftor/ifacaftor to enhance CFTR processing and function MUCOLYTICS ARE NOT SUFFICIENT!! (ie: dornase alpha, hypertonic saline. antibiotic not appropriate either)
43
patient has delta F508 mutation on both alleles. can the clinician ONLY give ifacaftor?
NO has no effect unless a gating mutation is present (class III) need corrector/combo therapy
44
dornase alfa is used in CF patients to REDUCE MUCUS VISCOSITY what patient is LEAST LIKELY to benefit from this drug
a newly diagnosed infant with minimal infection or inflammation -- they don't have a lot of that thick mucus yet!
45