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S2 Case 3 - asthma & COPD > Cystic Fibrosis: Case Study > Flashcards

Cystic Fibrosis: Case Study Flashcards

1
Q

What is done at birth?
1st

A

Heel prick test
= test for IRT levels
= immuno reactive trypsinin

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2
Q

What is trypsinin?

A

Pro-enzyme
= enzyme that is NOT active
Activated by trypsin in small intestine

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3
Q

What is then done if IRT levels come back elevated?

A

Sweat chloride test

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4
Q

What is the level of sweat chloride if CF?
2nd

A

> 60 mmol/L

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5
Q

What is then done if sweat test is elevated?
3rd

A

Genetic test to identify if genetic mutation is present

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6
Q

What is the pancreas’ endocrine function?

A

Release hormones
eg. insulin

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7
Q

What is the pancreas’ exocrine function?

A

Release enzymes

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8
Q

What happens to pancreas in CF patient?

A

Pancreatic duct blocked by mucus
= enzymes blocked
= accumulate

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9
Q

What happens if enzymes are stuck in pancreas?

A

Carbs, proteins + fats cannot be broken down
= nutrients cannot be absorbed

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10
Q

What therapy is immediately offered?

A

Early nutrition
Liquid multivitamins
Replacement of enzymes
Prophylactic antibiotics

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11
Q

What is an example of replacement of enzymes drug?

A

Creon

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12
Q

What is the problem with Creon?

A

Enteric coating
= need to be given with acidic food
= or protease will degrade the mouth
Babies can’t be given tablets

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13
Q

What do you monitor in patient’s with CF?

A

Height + weight
Pulmonary status
Oropharyngeal cultures
Glucose tolerance testing
Bone density

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14
Q

How is pulmonary status tested?

A

FEV1
= forced expiratory vol in 1s

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15
Q

Why is oropharyngeal cultures collected?

A

Testing for bacteria

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16
Q

Why is glucose tolerance tested?

A

Pancreas NOT working
= chance of developing diabetes

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17
Q

Why is bone density tested?

A

NO nutrition
= chance of osteoporosis

18
Q

What % of FEV1 is considered respiratory failure?

A

30%

19
Q

Why is inhalation of hypertonic saline sometimes given to CF patients?

A

Help liquify mucus
= can cough it up

20
Q

What is Dornase alfa?

A

Enzyme
Recombinant form of human deoxyribonuclease

21
Q

What does Dornase alfa do?

A

Breaks up cellular DNA from neutrophils
= decreases mucus viscosity

22
Q

How does cellular DNA of neutrophils end up in lungs?

A

Bacteria trapped in lungs
= neutrophils stimulated
= trapped
= die (apoptosis)
= DNA released
= increase viscosity of mucus

23
Q

What is Pseudomonas aeruginosa?

A

Gram negative
CF patients will eventually get it at one point

24
Q

What is the problem with Pseudomonas aeruginosa?

A

Difficult to treat
= resistant to most antibiotics

25
Q

What is often prescribed to treat Pseudomonas aeruginosa in CF patients?

A

Oral ciprofloxacin for 3 weeks
Nebulised tobramycin 300mg for 1 month
After negative, ciprofloxacin stopped BUT tobramycin continued for 1 month

26
Q

What is the problem with quinolones?

A

Caution in children
= joint problems

27
Q

Why are quinolones used then in CF paediatric patients?

A

CF outweighs the problems

28
Q

What type of antibiotic is ciprofloxacin?

A

Quinolone

29
Q

What type of antibiotic is tobramycin?

A

Aminoglycosides

30
Q

What type of antibiotic is azithromycin?

A

Macrolide antibiotic

31
Q

Why is azithromycin sometimes used as “500mg 3x a week”?

A

Has inflammatory properties at that dose + regime

32
Q

What is the normal level for 2-hr OGTT plasma glucose?

A

<11mmol/L
= higher diabetes/pancreas not working?

32
Q

What is the normal level for 2-hr OGTT plasma glucose?

A

<11mmol/L
= higher diabetes/pancreas not working?

33
Q

What is HbA1c?

A

Sugar stuck on Hb

34
Q

What is the normal level for HbA1c?

A

<6%

35
Q

What does microalbuminuria suggest?

A

Protein in urine
= signs of renal failure

36
Q

Which gene is mutated in CF?

A

CFTR

37
Q

What does CFTR code for?

A

Cl channels

38
Q

What happens when we sweat?

A

Release Na+, Cl- + H2O
Na channel = Na+ reabsorbed
Cl channel = Cl- reabsorbed = NORMAL person

39
Q

What happens because Cl- is NOT reabsorbed?

A

= Na+ NOT reabsorbed
= NaCl excreted out = salty sweat
= H2O follows salt
= dehydration

40
Q

What happens because H2O leaves in CF patients?

A

Mucus gets thick + sticky
= mucus cannot be cleared
= bacteria cannot be cleared
= stuck in lungs

S2 Case 3 - asthma & COPD (25 decks)

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