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Module 1 > CF: Clinical Aspects and Pathophysiology > Flashcards

CF: Clinical Aspects and Pathophysiology Flashcards

1
Q

Inheritance Pattern of CF and the carrier rate

A

Autosomal Recessive

1 in 25

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2
Q

Incidence of CF

A

1 in 2500 live births

(300 new cases/year in UK)

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3
Q

What is the function of the CFTR gene

A

Codes for CFTR protein which is a caMP-regulated Cl- and HCO3- channel at apical membrane of epithelial cells

Regulates ENaC too

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4
Q

How could CF lead to respiratory failure?

A

Decreased clearance of mucus

Increase infections and inflammation

Small airway plugging

Bronchiectasis

Respiratory failure

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5
Q

CF is a multi-organ disease. What are its other extrapulmonary presentations

A
  • Sinus infections and Nasal Polyps
  • Bile duct obstruction
    • Biliary cirrhosis
  • Exocrine pancreatic insufficiency
    • Malnutrition
  • Infertility
    • CBAVD in men
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6
Q

What are the 6 classes of CFTR mutations

A
  • I - no CFTR synthesis
  • II - Synthesis but no processing
  • III - Block in gating
  • IV - altered conductance
  • V - reduced synthesis
  • VI - increased recycling
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7
Q

About how much CFTR is enough to decrease sypmtoms

A

30%

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8
Q

What are the broad categories of therapies that have been developed to treat CF

A
  • CFTR protein correctors/potentiators
  • Mucolytics
  • Anti-inflammatories
  • Anti-microbial
  • Nutrition
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9
Q

What are the two approaches to restoring CFTR function

A
  • Potentiators
    • Increase ion flow through CFTR on membrant
  • Correctors
    • Corrects the misfolding of CFTR to increase its processing and delivery to membrane
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10
Q

What are the diagnostic tests for CF

A
  • Sweat Test
  • CFTR Genotyping
  • Stool Elastase
    • Pancreatic insufficiency
  • Nasal Potential Difference
    • Measure of ion transport
    • Only i specialist centres
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11
Q

What are the ranges for sweat test

A
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12
Q

What are some symptoms of CF

A
  • Cough with sputum
  • Wheeze
  • Recurrent Infections
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13
Q

What are some signs of CF

A
  • Nasal polyps
  • Finger clubbing
  • Malnutrition
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14
Q

What are the aims of modern CF management

A
  • Maintain pulmonary health
    • Asymptomatic
    • ↓Infections
    • Normal lung function
    • ↓Exacerbations
  • Normal growth/nutrition
  • QoL
  • Fulfill potential
    • Social
    • Academic
    • Financial
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15
Q

What can we use to assess respiratory disease

A
  • Physical Examination
  • Microbiology
  • Radiology
    • CXR
    • FRCT
    • Ventilation Scan
  • Physiology
    • Oximetry
    • Spirometry
  • Bronchoscopy
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16
Q

What is the advantage of spirometry in assessing CF disease

A
  • Reproducible measure of lung volumes
17
Q

What are some disadvantages of spirometry in assessing CF

A
  • Limited normal values for <5 years
  • Relatively insensitive
    • Normal LFT even with evolving CF disease
18
Q

OUtline how Lung Clearance Index is measured

A
  • Known concentration of inert gas inhaled
    • Until equilibrium
  • Patient exhales and inert gas concentration measured in exhaled breath
19
Q

What is a limitation of microbiology samples from CF and what is the implication of this?

A

Paediatric patients may not be able to expectorate

Could miss P**seudomonas aeruginosa antibodies -> produce biofilm and treatment wouldn’t work

20
Q

What pathogens are usually found in CF patients

A
  • Staphylococcus
    • ​Protocol: Babies given prophylactic flucoxacillin
  • Pseudomonas aeruginosa
21
Q

Features of Pseudomonas aeruginosa

A
  • Oppostunistic
  • Grame negative
  • Planktonic or biofilm form
22
Q

What is the role of bronchoscopy in CF

A
  • Microbiological samples
    • Non-expectorating patients
  • Clearnce of mucus plugs
  • Research
23
Q

What is a potential novel therapy against antibiotic resistance

A

Bacteriophages

24
Q

What are some features of bacteriophages

A

Virus that is highly specific to a bacteria

Self-amplifying

Self-limiting

No reported adverse effects

25
Q

What is the mechanism of action of bacteriophages

A
  • Attachment
    • Phage lands on target bacterium wall
  • Phage DNA
    • Is injected into bacterium by the sheathing of phage tail
  • Phage genome replication
    • Replication of DNA
    • Taking over bacterium’s metabolic machinery
  • Synthesis
    • Production of phage components
  • Assembly
  • Release
    • Phage enzyme breaks down peptidoglycan
    • Cell wall lysis
26
Q

What are some gastrointestinal complications of CF

A
  • Exocrine and endocrine pancreatic insufficiency
  • GORD
  • Meconium ileus
  • Constipation
  • Liver disease
27
Q

How do you treat GI involvement

A
  • Improve Fat absorption
    • PERT (Pancreatic Enzyme Replacement Therapy)
  • Increase intake
    • Calories
    • Fat
  • Supplemental Diet
    • DEKAs
    • Feeds/gastrostomy

Module 1 (16 decks)

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