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Respiratory > Bronchiectasis and cystic fibrosis > Flashcards

Bronchiectasis and cystic fibrosis Flashcards

1
Q

What is bronchiectasis?

A
  • Chronic irreversible dilatation of one or more bronchi
  • Pathological condition can be caused by many diseases or be idiopathic
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2
Q

What happens to the bronchioles in bronchiectasis?

A
  • Results in abnormally enlarged bronchi
  • Deformed bronchi exhibit poor mucus clearance
  • Predisposition to recurrent or chronic bacterial infection
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3
Q

What is the aetiology of bronchiectasis?

A
  • Variety of underlying causes, with a common underlying mechanism of chronic inflammation
  • Pertussis and TB used to be most common causes
  • Now underlying congenital conditions are primary cause in UK
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4
Q

What does inflammation of the bronchi result in?

A
  • Destruction of elastic and muscular components of bronchial wall and peribronchial fibrosis + scarring
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5
Q

What are the radiological findings in a patient with bronchiectasis?

A
  • CXR - usually abnormal but may be normal in early disease
  • Classic abnormality: dilated bronchi with thickened walls (tram-track sign)
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6
Q

What is the gold-standard diagnostic investigation for bronchiectasis?

A
  • High resolution CT
  • Demonstrates bronchial dilation bigger than the adjacent blood vessel
  • Bronchial wall thickening
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7
Q

What is the signet ring sign?

A
  • Dilated bronchus and accompanying pulmonary artery branch are seen in cross section
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8
Q

What are the very common symptoms of bronchiectasis?

A
  • Chronic cough
  • Daily mucopurulent sputum production - can vary in quantity, colour, and consistency
  • Accompanying halitosis
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9
Q

What are the common symptoms of bronchiectasis?

A
  • Breathlessness on exertion
  • Intermittent haemoptysis
  • Nasal symptoms
  • Chest pain
  • Fatigue
  • Symptoms not very specific - history and risk factors are key to diagnosis
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10
Q

What is a less common symptom of bronchiectasis?

A
  • Wheeze
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11
Q

What are the clinical signs of bronchiectasis?

A
  • Hypoxaemia in advanced cases
  • Fever
  • Haemoptysis (usually mild)
  • Fine crackles (rales)
  • High-pitched inspiratory squeaks
  • Rhonchi
  • Sometimes can hear both crackles and wheezing
  • Systemic signs e.g. weight loss
  • Clubbing of digits is less common
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12
Q

What are some of the causes of bronchiectasis?

A
  • Post infective - pertussis, TB, measles
  • Mucociliary clearance defects - cystic fibrosis
  • Immune deficiency - hypogammaglobulinaemia
  • Idiopathic causes
  • Alpha-1 antitrypsin deficiency
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13
Q

What is the vicious cycle of bronchiectasis?

A
  • Bronchial dilation
  • Leads to mucous accumulation, impaired ciliary function, increased risk of infection
  • Infection leads to inflammation and loss of bronchial elastic fibres and smooth muscle
  • More dilatation
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14
Q

What are the common organisms that cause infection in bronchiectasis?

A
  • Haemophilus influenzae
  • Pseudomonas aeruginosa
  • Moraxella catarrhalis
  • Stenotrophomonas maltophilia
  • Fungi - aspergillus, candida
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15
Q

What makes it more likely that a patient has bronchiectasis?

A
  • Asthma (particularly lifelong) without any great objective evidence
  • ‘COPD’ but without risk factors or diminished breath sounds
  • Severe chest infection earlier in life, lifelong/recurrent chest infections
  • Sputum culture positive for common organisms such as haemophilus/pseudomonas/atypical mycobacterium
  • Pt has IBS/rheumatoid arthritis
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16
Q

What are the pulmonary function tests used to investigate bronchiectasis?

A
  • Initial and follow up spirometry
  • Obstructive airways disease evidenced by reduced FEV1 or FEV1/FVC ratio of <70%
  • Elevated RV/TLC ratio consistent with air trapping
  • Reduced capacity for CO in severe disease
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17
Q

How is bronchiectasis differentiated from chronic bronchitis?

A
  • Bronchitis caused by mucous gland hyperplasia, hypersecretion rather than airway dilatation or scarring
  • Bronchitis due to tobacco smoke and air pollutants rather than infection
  • Chronic bronchitis causes cough and a small amount of white sputum production but no intermittent fevers/haemoptysis
18
Q

How is bronchiectasis managed?

A
  • Physio/airways clearance - daily clearance is essential for treatment success
  • Sputum sampling - routine culture and Non Tuberculosis Mycoplasma
  • Exclude immunodeficiency/treat identifiable causes
  • Consider long-term therapies at future visits
  • Annual flu & routine vaccinations
  • An established MDT is key
19
Q

Which organisms do we need to vaccinate bronchiectasis patients against?

A
  • Haemophilus influenzae
  • Streptococcus pneumoniae
20
Q

How do we define exacerbation of bronchiectasis?

A
  • A person with bronchiectasis with deterioration in 3 or more key symptoms for at least 48 hours
  • Cough
  • Sputum volume and/or consistency
  • Sputum purulence
  • Breathlessness and/or exercise tolerance
  • Fatigue
  • Haemoptysis
21
Q

What is cystic fibrosis?

A
  • Autosomal recessive disorder
  • Predominant mutation is Phe508del on long arm of chromosome 7
  • Abnormal function of Cystic Fibrosis Conductance Regulator - has a key role in maintaining lung epithelial function
22
Q

What is the CFTR protein?

A
  • Transmembrane protein that transports Cl- and HCO3- and regulates Na+ absorption into cells via ENAC channels
  • Enables Cl - to be transported out of cells into airways - helps to control movement of water
  • Plays critical role in hydration of mucus at surface of airway track
23
Q

What happens once the CFTR protein stops working properly?

A
  • Reduced airway surface liquid hydration
  • Thick and sticky mucus
  • Muco-ciliary clearance is impaired
24
Q

How can mutations affect the CFTR protein?

A
  • Manufacture of CFTR protein
  • Transportation of CFTR
  • CFTR processing function
  • CFTR stability at cell membrane
25
Q

Outline the effects of the Phe508del mutation?

A
  • Defective intracellular processing and trafficking
  • Decreased stability, which drastically reduces quantity of CFTR protein at apical surface of epithelial cells
  • Also exhibits defective channel gating, which further limits anion transport
26
Q

How is GF diagnosed?

A
  • One or more of the characteristic phenotypic features
  • Or history of CF in a sibling
  • Or a positive newborn screening test result

AND
- An increased sweat chloride concentration - SWEAT TEST
- Or identification of 2 CF mutations - genotyping/EXTENDED GENOTYPING

27
Q

What are the main CF clinical presentations?

A
  • Meconium ileus
  • Intestinal malabsorption
  • Recurrent chest infections
  • Newborn screening
28
Q

What is a meconium ileus?

A
  • Affects 15-20% of newborn CF infants
  • Bowel is blocked by sticky secretions
  • Bilious vomiting, abdominal distension, and delay in passing meconium
29
Q

What is intestinal malabsorption?

A
  • Over 90% of CF individuals are affected
  • Evident in infancy
  • Due to severe deficiency of pancreatic enzymes
  • Secondary to blockage of exocrine glands
30
Q

What kind of CF do patients who are diagnosed late have?

A
  • Mutation associated with residual CFTR function
  • Or heterozygous CFTR mutations - one severe + one mild
  • Called atypical CF
  • Need a high index of suspicion to diagnose
  • Consider in cases of recurrent idiopathic pancreatitis, recurrent sinusitis and lung infections, infertility, allergic bronchopulmonary aspergillosis
31
Q

What are the CF complications of the lungs?

A
  • Bronchiectasis
  • Pneumothorax
  • ABPA
  • Haemoptysis
  • Respiratory failure
32
Q

What are the CF complications of the nasal/upper respiratory tract?

A
  • Chronic sinusitis
  • Nasal polyposis
33
Q

What are the CF complications of the GI tract?

A
  • Pancreatic insufficiency
  • Distal Intestinal Obstruction Syndrome
  • Oesophageal reflux/oesophagitis
  • Chronic liver disease
  • Portal hypertension
  • Gallstones
34
Q

What are the CF complications of the heart?

A
  • Cardiac failure
35
Q

What are the CF complications of the joints and bones?

A
  • Arthritis
  • Osteoporosis
36
Q

What are the CF complications of the reproductive tract?

A
  • Male infertility
  • Congenital bilateral absence of the vas deferens (CBAVD)
37
Q

What lifestyle advice is given to patients with CF?

A
  • No smoking
  • Avoid other CF patients
  • Avoid friends/relatives with colds/infections
  • Avoid jacuzzies (pseudomonas)
  • Clean and dry nebulisers thoroughly
  • Annual influenza vaccine
  • Pneumococcal vaccine
  • Avoid compost, stables, rotting vegetation
38
Q

What is the clinical management of CF?

A
  • Complex - led by CF specialist centres/MDTs
  • Holistic care/multisystem organ focus
  • Up to date pneumococcal and haemophilus influenza vaccines
  • Key is maintaining lung health
  • Optimal nutritional state - pancreatic status, vitamin status, weight/BMI
39
Q

Outline the role of nutrition in cystic fibrosis?

A
  • Earliest manifestations of disease related to GI and nutritional derangements
  • Impaired nutritional status:
  • Pancreatic insufficiency
  • Chronic malabsorption
  • Chronic inflammation leading to increased energy expenditure
  • Increased energy requirements of breathing
  • Suboptimal nutrient intake related to impaired taste, fatigue, inflammatory mediated anorexia
40
Q

How can lung function be improved in CF?

A
  • Better nutritional status associated with better lung function
41
Q

What are the 6 classes of CFTR mutation?

A
  • No protein production
  • Protein made but never gets to cell membrane
  • Protein gets to membrane but doesn’t work at all
  • Protein made but only partially active
  • Protein expressed at gene level but substantial reduction in mRNA and/or protein synthesis
  • Protein gets to membrane but partially unstable
42
Q

What is the cystic fibrosis survival like?

A
  • Medications now being developed that target underlying disease mechanisms, not just complications
  • Improvement of life-expectancy has been achieved due to:
  • Augmenting airway clearance
  • Aggressively treating infection
  • Correcting nutrition deficits
  • New drugs

Respiratory (21 decks)

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