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Respiratory > Bronchiectasis and CF > Flashcards

Bronchiectasis and CF Flashcards

1
Q

What is youngs syndrome?

A

Thickened mucosal secretions

Leads to azoospermia and recurrent sinobronchial infection

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2
Q

What is allergic bronchopulmonary aspergillosis?

A

Exaggerated response of the immune system to the fungi aspergillus

Most commonly occurs in asthma and CF patients

Causes airway inflammation and bronchiectasis

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3
Q

What is bronchiectasis?

A

Chronic infection of bronchi and bronchioles causes airways to become thickened, inflamed and irreversibly damaged

This leads to dilatation of airways and impaired mucociliary transport

This leads to frequent infections

Vicious circle

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4
Q

What are the main organisms causative of exacerbations of bronchiectasis?

A

H.influenzae
Strep.pneu
Staph.a
Pseudomonas.aer

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5
Q

Causes of bronchiectasis

A

Idiopathic in 50% of cases

Mechanical obstruction

Post-infective bronchial damage

Granulomatous disease (TB, sarcoidosis)

Diffuse disease of lung parenchyma (idiopathic pulmonary fibrosis)

Immune overresponse (abpa, post-transplant)

Immune deficiency

Mucociliary defect (CF, youngs, Kartageners)

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6
Q

Symptoms of bronchiectasis

A 
Persistent cough
Occasionally haemoptysis
Originally yellow green sputum 
More severe - persistent copious purulent dark green sputum and persistent halitosis 
Breathlessness 
Chest pain, malaise, fever, weight loss
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7
Q

Signs of bronchiectasis

A

Clubbing
Coarse creps (usually at bases) which shift with coughing
Wheeze if asthma, COPD or ABPA

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8
Q

Signs on chest X-ray of bronchiectasis

A

Tram lines shadows (dilated Bronchi seen as parallel lines radiating from hilum to diaphragm)
and ring shadows - thickening of bronchial walls
Fibrosis, atelectasis, pneumonic consolidations

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9
Q

Other investigations in bronchiectasis

A

HRCT - extent of disease (best diagnostic test - dilated bronchi with thickened walls)
Sputum culture - specificity
Spirometry - obstructive problem
Bronchoscopy - to locate haemoptysis and exclude obstruction
CF sweat test
Aspergillus precipitans

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10
Q

Management of bronchiectasis

A

Postural drainage
Antibiotics
Bronchodilators in COPD, asthma, CF and ABPA

Inhaled corticosteroids (fluticasone) has been shown to reduce inflammation and volume of sputum but does not affect frequency of exacerbations or lung function

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11
Q

What is Kartageners syndrome?

A

Triad of primary ciliary dyskinesia, situs inversus (dextrocardia) and abnormal frontal sinuses
And infertility

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12
Q

What is cystic fibrosis?

A

Alteration in viscosity and tenacity of mucus produced at epithelial surfaces

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13
Q

Where does cf affect?

A 
Lungs
Pancreas
Gastrointestinal tract 
Hepatobiliary
Reproductive tracts
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14
Q

Prevalence of CF

A

1 in 2500 live births

1 in 25 carrier (autosomal dominant inheritance)

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15
Q

Pathology of CF

A

Mutation on chromosome 7 in CFTR (CF transmembrane conductance regulator)
3 base pairs at position 508 - loss of phenylalanine

No CFTR - chloride channel therefore decreased chloride conductance and increased resorption of sodium

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16
Q

Significance of pseudomonas aeruginosa in CF

A

Frequent infecting agent

Some evidence CFTR has role in normal uptake and processing of it

17
Q

Respiratory effects of CF

A 
Lungs born normal 
Frequent respiratory infections
Increased mucosal viscosity 
Disrupted clearance
Salt content inactivates defensins - natural antimicrobial peptides

Vicious cycle of infection and inflammation
Bronchiectasis, resp failure and death

18
Q

Lung signs in CF

A

Coarse crackles
Clubbing
Cyanosis
Often chronic sinusitis

SOB and haemoptysis with bronchiectasis

19
Q

What is a worrying sign in CF

A

Chest pain - could be a mucus plug - lead to pneumothorax

20
Q

What happens eventually in respiratory with CF?

A

Respiratory failure and cor pulmonale (right heart failure due to pulmonary hypertension)

21
Q

GI effects of CF

A

85% - pancreatic dysfunction - decreased enzymes - malabsorption - steatorrhea and failure to gain weight

Meconium ileus (MI equivalent syndrome in adult) - 10% of children born with it 
- constipation and mass, colicky pain
22
Q

What else can CF patients get following pancreatic dysfunction

A

11% get diabetes

23
Q

Other GI CF symptom

A

Gall stones and biliary cirrhosis due to abnormalities of biliary tract

24
Q

Reproductive effects of CF

A

Nearly all male are infertile due to absent vas deferens

Female okay - near normal fertility - but some have cervical mucus abnormalities

25
Q

Other effects of CF

A

Overheating - excess loss of salt in sweat

Rashes

Nasal polyps

Arthropathy

Later osteoporosis and amyloidosis

26
Q

Diagnosis of CF

A

Family history

DNA analysis

Sweat test - pilocarpine iontophoresis
Chloride > 60 mmol/L - diagnostic
30-59 - possible atypical CF
Chloride usually > sodium

27
Q

Investigations in CF

A

Chest X-ray - bronchiectasis and hyperinflation

Bloods

28
Q

How do infant cf screening

A

Increased immunoreactive trypsin activity in blood taken on 6 day guthrie heal prick

29
Q

Treatment of CF

A

Vaccinations

Pulmonary rehab and Chest physio

Oxygen when needed

Antibiotics (staph a - flucloxacillin, h.inf and strep.p - amoxicillin)
Pseudomonas - ceftazidime or ciproflaxin

DNAse nebulised
Amiloride - sodium transport inhibitor
Nebulised saline - improves clearance by drawing water in periciliary layer

Corticosteroids for inflammatory response

Nutrition and high calories

30
Q

Antibiotic treatment for pseudomonas - cf and bronchiectasis

A

Oral Ciprofloxacin or IV antibiotics

31
Q

Chromosome for CF

A

Chromosome 7

32
Q

When do symptoms of bronchiectasis arise

A

Usually after an acute respiratory illness

Respiratory (7 decks)

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