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Jamie Respiration Pathology > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

How does cystic fibrosis occur?

A

Inheriting a mutation to the CFTR gene

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2
Q

What is the function of CTFR?

A

Provide active transport for chloride

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3
Q

How does cystic fibrosis present in infants and young children?

A

Recurrent chest infections

Failure to thrive

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4
Q

How does cystic fibrosis present in older children and adults? (3)

A

Recurrent chest infections
Sinusitis
Male infertility

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5
Q

When is screening for cystic fibrosis usually performed?

A

Newborn bloodspot on day 5 (Guthrie test)

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6
Q

What are the 2 cardinal features of Pancreatic insufficiency?

A 
Abnormal stools (pale/orange, oily and very foul)
Failure to thrive (growth)
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7
Q

What are the 2 Cardinal features of CF?

A

Pancreatic insufficiency

Recurrent bronchopulmonary infection

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8
Q

Name at least 2 treatment methods (4) for pancreatic insufficiency?

A

Enteric coated enzyme pellets
High energy diet
Fat soluble vitamin and mineral supplements
H2 agonist or proton pump inhibitors

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9
Q

Name at least 2 outcomes (4) for recurrent bronchopulmonary infections?

A

Pneumonitis
Bronchiectasis
Scarring
Abscesses

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10
Q

Name 3 treatments given to children with CF pre-infection?

A

Mucolytics
Prophylactic antibiotics
Influenza vaccination

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11
Q

What are the 2 most common organisms for CF in early years?

A

Staphylococcus aureus

Haemophilus influenzae

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12
Q

What is the most common organism for CF in later years?

A

Pseudomonas aeruginosa

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13
Q

What are the 3 aims for treating a chronic infection for CF in intermediate years and what drugs are given for these aims?

A

Suppress bacteria load (azithromycin)
Treat infective exacerbations (ibuprofen)
Reduce inflammation (prednisolone)

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14
Q

What is the function of mucolytics?

A

To reduce viscosity of sputum

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15
Q

Name at least 2 airway clearance techniques? (3)

A

Percussion and drainage
Autogenic drainage
Active cycle of breathing

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16
Q

Name at least 2 airway clearance adjuncts? (3)

A

PEP (positive expiratory pressure) mask
Cornet/flutter
High frequency chest wall oscillation

17
Q

What is other feature (not the cardinal 2) for CF that can occur in adults?

A

Gastrointestinal problems

18
Q

What is last resort treatment for CF?

A

Lung transplant

19
Q

Name at least 3 contraindications to lung transplants?

A 
Other organ failure
Recent malignancy (5 years)
Peripheral vascular disease
Drug/smoking/alcohol dependency
Systemic infection (active)
Microbiological issues

Jamie Respiration Pathology (15 decks)

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