S8) Cystic Fibrosis and Bronchiectasis Flashcards
What is bronchiectasis?
Bronchiectasis is the chronic dilatation of one or more bronchi wherein the bronchi exhibit poor mucus clearance and there is predisposition to recurrent/chronic bacterial infection
Identify 5 causes of bronchiectasis
- Post infective e.g. whooping cough, TB
- Immune deficiency e.g. hypogammaglobulinaemia, secondary - HIV
- Obstruction e.g. foreign body, tumour, extrinsic lymph node, compression = bronchus can’t be cleared = inflammation etc
- Inhalation of toxic chemicals/gases
- Secondary immune deficiency e.g. HIV, malignancy
- mucociliary clearance defects - CF
Identify 4 genetic causes of bronchiectasis
- Cystic fibrosis
- Primary ciliary dyskinesia
- Young’s syndrome (bronchiectasis, sinusitis, reduced fertility)
- Kartagener syndrome (bronchiectasis, sinusitits, situs inversus)
Identify 3 causative organisms of bronchiectasis
- Haemophilus influenzae
- Fungi e.g. aspergillus, candida
- Non-tuberculous mycobacteria
Describe the management of bronchiectasis
- Treat underlying cause - removed the mucus, underlying congenital problem, autoimmune?
- Physiotherapy for mucus clearance (⅔ times a day)
- Antibiotics according to sputum cultures
- Flu vaccine
- Bronchodilators // corticosteroids
- correct nutrient deficits
What is cystic fibrosis?
Cystic fibrosis is a multisystem, autosomal recessive disease occurring due to a mutation in the CFTR molecule which is characterised by thickened secretions
How does cystic fibrosis present?
- Meconium ileus – intestinal obstruction by sticky secretions (bilous vomiting & abdominal distension)
- Intestinal malabsorption – severe deficiency of pancreatic enzymes
- Recurrent chest infections
How does one confirm the diagnosis of Cystic fibrosis?
- 1/more characteristic phenotypic features
- History of CF in a sibling
- Positive newborn screening test result
- Increased sweat [Cl-]
- Identification of two CF mutations (genotyping)
Identify 4 common cystic fibrosis complications and describe how they are treated
- Respiratory Infections – aggressive therapy with physio and prophylactic antibiotics
- Low body weight – pancreatic enzyme replacement therapy, high calorie intake and extra supplements
- Distal Intestinal Obstruction Syndrome (DIOS)
- CF Related Diabetes
Describe the management of cystic fibrosis
- Avoid smoking
- Avoid other CF patients
- Avoid jacuzzis (pseudomonas)
- Annual influenza immunisation
- Sodium chloride tablets in hot weather / vigorous exercise
What is the aetiology of bronchiectasis?
- chronic inflammation
- can permanently damage elastic and muscular components of bronchial wall and causes fibrosis
- more risk of infection
- then more inflammation and cillary damage
- cycle
- primarily caused by pertussis and TB
- remember this infection and pus can also spread to the alveoli
what investigation do you use to determine for bronchiectatsis
CXR
- classic diagnosis is thickened walls and dilated bronchi - tram track signs
- need high resolution CT (it will show the stage of the situation too)
- lungs will be thickened and scarred
- signet ring sign - dilated bronchus next to pulmonary artery and in this case its BIGGER than the artery (should be smaller)
what are clinical symptoms of bronchiectasis
- chronic cough
- breathless on exertion
- chest pain
- fatigue
- nasal symptoms
- haemoptysis
- flem - see how much they are collecting over 24 hours
(mainly find out through history)
what are signs of bronchiectasis
- hyperaemia
- fever
- haemoptysis (cough up blood)
- fine crackles
- high pitched inspiratory peaks
- crackles and wheezing
what are some red flags in a history that can be a risk factor bronchiectasis
- severe chest infection earlier in life
- life long chest infections
- recurrent chest infections
- recurrent sinus infections
what are some pulmonary function tests
- obstructive airways:
- reduced forced expiratory volume
differentiate between chronic bronchitis and bronchiectasis
- chronic - white sputum, no history of intermitted fevers
- bronchiectasis - intermitted fevers, sputum is foul smelling
how do we define exacerbation in bronchiectasis
- symptoms for at least 48 hours:
- cough
- sputum volume
- breathlessness
- fatigue
- haemoptysis
how does CFTR malignancy cause CF
- transports chloride and bicarbonate and regulates ENAC channels
- enables Cl to be transported into airways
- it regulates sodium reabsorption into cells
- plays a role in the hydration of mucous
- if its defective it reduces hydration
- mucus becomes thick and can cause problems within the ciliary hair
basically:
- controls movement of chloride out cells, Na follows Cl for the electrochemical gradient, water follows Na, so if there is a problem with Cl transport then water wont move and mucus becomes thick
what is the classic presentation of people with CF
- meconium ileum - bowel is blocked due to mucus so intestinal obstruction = vomiting, abdominal distension
- intestinal malabsorption - mainly in infancy, due to blockage of exocrine glands
- recurrent chest infections
- newborn screening
CF lifestyle
- no smoking
- no other friends with CF - cross infection
- avoid people who are ill
- annual influenza immunisation
- sodium chloride tablets
- need nutritional support - better nutrition = better lungs
tram track signs
CT comparison of a normal lung and a lung with bronchiectasis
signet ring sign on a CT
CF can effect multiple systems in the body, name some problems it can cause
- chronic sinusitis
- oesophageal reflux
- chronic liver disease
- gall stones
- cardiac failure
- arthritis
