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Respiratory > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

What is the prevalence of cystic fibrosis?

A

The commonest inherited, life-shortening, disorder amongst the northern european population

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2
Q

How is cystic fibrosis inherited?

A

As an autosomal recessive trait

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3
Q

What is CFTR?

A

The gene that codes for ion channels that conduct chlorine

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4
Q

How does cystic fibrosis happen?

A

A mutation of the CFTR gene

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5
Q

How is the severity of CF measured?

A

By classing the mutations as class 1 being the most severe, decreasing in severity to class 5

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6
Q

What happens in class 1 mutations?

A

There is no synthesis of the CFTR protein

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7
Q

What is the function of the CFTR proteins?

A

Active transport channel for chloride

Regulates liquid volume on the epithelial surface which allows cilia to function normally

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8
Q

What are the common presentations of CF in infants and young children?

A

Recurrent chest infection

Failure to thrive

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9
Q

What are the common presentations of CF in older children and adults?

A

Recurrent chest infections
Nasal polyps and sinusitis
Male infertility

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10
Q

What are the less common presentations of CF in infants and young children?

A 
Rectal prolapse 
Pseudo-Bartter's syndrome 
Anaemia 
Oedema 
Hypoproteinaemia
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11
Q

What are the less common presentations of CF in older children and adults?

A

Acute pancreatitis
Liver disease
Pseudo-bartter’s syndrome
Atypical mycobacteria

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12
Q

What are the cardinal features of CF?

A

Pancreatic insufficiency

Recurrent bronchopulmonary infection

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13
Q

How is the pancreatic insufficiency treated?

A

Using pancreatic enzyme replacement therapy (PERT)
High energy diet
H2 agonist or proton pump inhibitor

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14
Q

How is respiratory tract infection prevented in early years?

A 
Segregation 
Airway clearance and adjunction 
Mucolytics 
Prophylactic antibiotics 
Annual influenza vaccination
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15
Q

In early years how much of infection is eradicated?

A

80-90% of cases

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16
Q

What are the common respiratory pathogens in early years of CF?

A

Staphylococcus aureus

Haemophilus influenzae

17
Q

What are the common respiratory pathogens in later years of CF?

A

Pseudomonas aeruginosa

18
Q

What are the less common respiratory pathogens of CF?

A

Burkholderia cepacia
Stenotrophomonas maltophilia
Alcaligenes xylosoxidans
Atypical mycobacteria

19
Q

How can CF manifest in the GI tract?

A

Dysmotility

Co-exisent disease

20
Q

What does GI dysmotility cause?

A

Meconium ileus
Gastro-oesophageal reflux
Distal intestinal obstruction
Constipation/rectal prolapse

21
Q

What types of GI co-existent diseases can occur with CF?

A

Crohn’s disease

Celiac disease

22
Q

How can CF manifest apart from its clinical features and its GI manifestations?

A 
Hepatopathy 
Upper airway polyps and sinusitis 
Diabetes 
Osteopenia 
Arthropathy 
Heat exhaustion 
Bilateral absence of vas deferens 
Vaginal candidiasis
23
Q

What are some airway clearance techniques?

A

Percussion and drainage
Autogenic drainage
Active cycle of breathing

24
Q

What are the airway clearance adjuncts?

A

PEP mask
Cornet/Flutter
High frequency chest wall oscillation

25
Q

What is the estimated life expectancy of CF patients born after 2000?

A

40 years and above

26
Q

How is CF in adults divided?

A

80% recurrent pulmonary infections and pancreatic insufficiency
15% recurrent pulmonary infections
5% GI problems only

27
Q

What is changed in patients with persistent respiratory infections?

A

Less mucociliary clearance
More bacterial adherence
Less endocytosis of bacteria

28
Q

What does colonisation of pseudomonas aeruginosa do?

A

Reduce life expectancy by 10 years due to a rapid decline in lung function

29
Q

What is the treatment of recurrent respiratory tract infections?

A

Treat early and very aggressively with antibiotics - oral for Staph, haemophilus and pneumococcus. IV for pseudomonas, stenotrophomonas and burkholderia

30
Q

What are the two types of antibiotics used in treatment?

A

Beta-lactams

Aminoglycosides

31
Q

When should a lung transplant be done?

A

Rapidly deteriorating lung function
FEV1 < 30% predicted
Life threatening exacerbations
Estimated survival <2 years

32
Q

What drug can be used with Celtic mutation CF patients to treat the primary defect in CF?

A

Ivacaftor

33
Q

What gene in the celtic mutation?

A

G551D

Respiratory (27 decks)

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