Acute and chronic respiratory illness Flashcards

1
Q

Bronchiectasis

  • Definition/ pathology
  • Presentation
A

Irreversible dilatation of the bronchial tree leading to excess mucus production.

Can be a result of:

  • Cystic fibrosis
  • Primary ciliary dyskinesia
  • Severe pulmonary infections
  • Airway obstruction (foreign body, tumour)

Presentation

  • Chronic, mucus cough
  • Persists after infection
  • Recurrent purulent lung infections
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2
Q

Which protein is most commonly affected in cystic fibrosis

A

Cystic fibrosis transmembrane regulator (CFTR)

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3
Q

Cystic fibrosis is inherited in a _____ manner on chromosome _____ primarily affecting the _____ gene

A

Autosomal recessive inheritance

  • Chromosome 7
  • CFTR gene
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4
Q

Complications of CF

A

Chronic lung disease

Recurrent airway infections

Pancreatic insufficiency

  • Steatorrhoea
  • Vitamin K deficiency
  • Failure to thrive

Infertility

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5
Q

Signs of CF

A

Chronic resp disease

  • Chronic, productive cough
  • Finger clubbing
  • Barrell chest
  • Recurrent LRTIs

Pancreatic insufficiency

  • Meconium ileus
  • Failure to thrive

Bilteral atresia of vans deferens

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6
Q

Diagnosis of CF

A

At birth
- Newborn blood spot test

Chloride sweat test
- >60 mmol/L

Genetic testing

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7
Q

Most common mutation in CF

A

Delta-F508

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8
Q

Reproductive complication in males with CF

A

Bilateral vans deferens atresia

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9
Q

Common bacterial infections in CF (6)

A
  • Staphylococcus aureus
  • Haemophilus influenza
  • Klebsiella pneumoniae
  • Escherichia coli
  • Burkhodheria cepacia
  • Pseudomonas aeruginosa
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10
Q

Management of meconium ileus in CF

A

Enema/ osmotic laxative

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11
Q

Management of respiratory infections in CF

A

Prophylactic antibiotics

Bronchodilators/ chest physio

Inhaled mucolytic (mannitol, hypertonic saline)

Oxygen/ ventilation

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12
Q

Most common cause of pnuemothorax in children is…

A

Chest wall trauma

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13
Q

Presentation of pneumothorax

A

Dyspnoea

Chest pain

Shoulder pain (referred)

Signs

  • Hyperresonance on percussion
  • Reduced air entry
  • Subcutaneous emphysema
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14
Q

Management of spontaneous pneumothorax

A

If <2cm rim, <20% volume= supplemental oxygen

> 20% volume, >2cm rim
- Needle aspiration/ in-dwelling tube

Consistent leak/ failure of lungs to re-expand= surgery

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15
Q

Tension pneumothorax must be treated with…

A

Immediate needle decompression

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16
Q

What type of kids are likely to get a primary spotaneous pnuemothorax?

A

Tall, thin, athletic boys

17
Q

What percentage of CF patients have pancreatic exocrine dysfunction?

A

85%

18
Q

Incidence of CF in Caucasians

A

1 in 2500

19
Q

The carrier rate for the CF gene in Caucasians is ____

A

1 in 25

20
Q

Life expectancy of a newly diagnosed CF patient is _____ to ___ years

A

50-55