Bronchiectasis and Cystic Fibrosis Flashcards
What is bronchiectasis
Bronchiectasis - chronic dilatation of one or more bronchi
Bronchi exhibit poor mucus clearance and there is predisposition to recurrent or chronic bacterial infection
What sign is common on a CT of a patient with bronchiectasis
Signet ring sign
What are the symptoms of bronchiectasis
Chronic cough
Daily sputum production
Breathlessness on exertion
Intermittant haemoptysis
Nasal symptoms/upper resp symptoms
Chest pain
Fatigue
Wheeze - less common
Name the causes of bronchiectasis
Post infective - TB, whooping cough
Primary or secondary immune deficiency - hypogammaglobinaemia, HIV, malignancy
Mucocilary clearance defects - CF, primary ciliary dyskinesea, Youngs or Kartagener syndrome
Obstruction- foreign body, tumour, extrinsic lymph node
Toxic insult
Allergic bronchopulmonary aspergillosis
Rheumatoid arthritis
What organisms commonly cause bronchiectasis
Haemophilus influenzae
Psuedomonas aeruginosa
Strep pneumoniae
Fungi - aspergillus, candida
Nontuberculous mycobacteria
Staph aureus
What is the management for bronchiectasis
Physio/airway clearance
Sputum sampling
Exclude immunodeficiency
Treat identifiable causes
Flu vaccine
Pulmonary rehabilitation if MRC dyspnoea score ≥3
Establish MDT
How does a patient with an exacerbation of bronchiectasis present with
Deterioration in 3 or more of the key symptoms for at least 48hr
Cough
Sputum volume and/or consistency
Sputum purulence
Breathlessness and/or exercise intolerance
Fatigue
Haemoptysis
What is cystic fibrosis
Autosomal recessive condition affecting cystic fibrosis transmembrane regulator
CFTR is abnormal in cystic fibrosis resulting in thick, dehydrated body fluids in organs that have the CFTR
How is cystic fibrosis diagnosed
One or more of the characteristic phenotypic features, history of CF in a sibling or +ve new-born screening test
AND
Increased sweat chloride concentration or identification of two CF mutations
How can CF present
Meconium ileus - bowel blocked by sticky secretions so have intestinal obstruction soon after birth. Have bilious vomiting, abdominal discomfort and delay in passing meconium
Intestinal malabsorption - severe deficiency of pancreatic enzyme
Chest infections
New-born screening
Where can complications of CF occur in the body
Lungs - bronchiectasis, pneumothorax
Nasal/upper resp tract - chronic sinusitis
Pancreas - DM, pancreatic insufficiency
Gut - distal intestinal obstructive syndrome, oesophageal reflux
Liver - chronic liver disease, portal hypertension
Biliary tree - gallstones
Heart - cardiac failure
Joints and bones - arthritis, osteoporosis
Reproductive system - male infertility, congenital bilateral abscence of vas deferens
What lifestyle advice is given to patients with CF
No smoking
Avoid other CF patiens
Avoid friends/relatives with colds/infections
Avoid jacuzzis
Clean and dry nebulisers thoroughly
Avoid stables, compost and rotting vegetation
Annual influenza immunisation
NaCl tablet in hot weather/vigours exercise
How is CF managed
Holistic care
Maintaining lung health and nutritional state
Targeted therapies
Managing co-morbidities
Concordance with treatment
Name some features of cystic fibrosis
Chronic sinusitis
Nasal polyps
Repeated lower resp tract infections
Abnormal sweat secretions
Liver disease
Diabetes and pancreatic insufficiency
Finger clubbing
Steatorrhoea
Osteoporosis
Male infertility
Arthropathy and arthritis