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Flashcards in Bronchiectasis and CF Deck (32)
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1

What is youngs syndrome?

Thickened mucosal secretions
Leads to azoospermia and recurrent sinobronchial infection

2

What is allergic bronchopulmonary aspergillosis?

Exaggerated response of the immune system to the fungi aspergillus

Most commonly occurs in asthma and CF patients

Causes airway inflammation and bronchiectasis

3

What is bronchiectasis?

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

4

What are the main organisms causative of exacerbations of bronchiectasis?

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

5

Causes of bronchiectasis

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)

6

Symptoms of bronchiectasis

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

7

Signs of bronchiectasis

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

8

Signs on chest X-ray of bronchiectasis

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

9

Other investigations in bronchiectasis

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

10

Management of bronchiectasis

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

11

What is Kartageners syndrome?

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

12

What is cystic fibrosis?

Alteration in viscosity and tenacity of mucus produced at epithelial surfaces

13

Where does cf affect?

Lungs
Pancreas
Gastrointestinal tract
Hepatobiliary
Reproductive tracts

14

Prevalence of CF

1 in 2500 live births
1 in 25 carrier (autosomal dominant inheritance)

15

Pathology of CF

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

16

Significance of pseudomonas aeruginosa in CF

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

17

Respiratory effects of CF

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

Lung signs in CF

Coarse crackles
Clubbing
Cyanosis
Often chronic sinusitis

SOB and haemoptysis with bronchiectasis

19

What is a worrying sign in CF

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

20

What happens eventually in respiratory with CF?

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

21

GI effects of CF

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

What else can CF patients get following pancreatic dysfunction

11% get diabetes

23

Other GI CF symptom

Gall stones and biliary cirrhosis due to abnormalities of biliary tract

24

Reproductive effects of CF

Nearly all male are infertile due to absent vas deferens

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

25

Other effects of CF

Overheating - excess loss of salt in sweat

Rashes

Nasal polyps

Arthropathy

Later osteoporosis and amyloidosis

26

Diagnosis of CF

Family history

DNA analysis

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

27

Investigations in CF

Chest X-ray - bronchiectasis and hyperinflation

Bloods

28

How do infant cf screening

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

29

Treatment of CF

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

Antibiotic treatment for pseudomonas - cf and bronchiectasis

Oral Ciprofloxacin or IV antibiotics