Bronchiectasis and Cystic Fibrosis Flashcards
Describe bronchiectasis. (4)
Chronic dilation of one of more bronchi so it appears bigger than the adjacent blood vessel, bronchial wall thickening, and poor mucus clearance.
Describe the diagnosis of bronchiectasis. (2)
Signet ring sign on high resolution CT.
Describe symptoms of bronchiectasis. (8)
Often non-specific
Chronic cough and sputum production
Breathlessness, chest pain, haemoptysis, fatigue
Wheeze less common.
Describe the causes of bronchiectasis. (10)
Common:
Post infective - TB, pertussis toxin
Immune deficiency
Muco-ciliary clearing defects - CF, Young’s syndrome, Kartagener Syndrome.
Less common:
Obstruction - foreign body, tumour
Toxic insult - vomit aspiration, toxin inhalation
Describe Young’s Syndrome. (3)
Sinusitis, bronchiectasis and reduced fertility.
Describe Kartagener Syndrome. (3)
Sinusitis, bronchiectasis and situs invertus.
Describe 4 common organisms in the sputum of a bronchiectasis patient.
Haemophilus influenzae
Pseudomonas aeruginosa
Streptococcus Pneumonia
Non-tuberculus mycobacterium
When should you ask “could this be bronchiectasis?” (5)
“Asthmatics” with common severe chest infections, nasal/ear symptoms, IBD, RA, positive sputum culture.
Describe the management of bronchiectasis (5)
Physio and airway clearance Routine sputum sampling Exclude immunodeficiency Treat identifiable cause Consider daily abx and flu vaccines
Describe a bronchiectasis exacerbation. (7)
Deterioration in 3 or more key symptoms in 48 hours: cough, sputum volume / consistency / purulence, breathlessness, fatigue, haemoptysis.
Describe the pathophysiology of cystic fibrosis. (5)
Autosomal recessive defect in chromosome 7
CF transmembrane conductance regulator mutation
Ineffective cell surface Cl- excretion and increased ENaC activity (pumping Na back in) .
Dehydrated bodily fluid in eg lungs, liver, GI.
Describe the diagnosis of CF. (5)
One or more phenotypic features OR history of a sibling OR postive newborn screening
AND increased sweat chloride test OR genotyping showing mutations.
Describe the presentation of CF. (4)
Meconial delay at birth.
Intestinal malabsorption due to pancreatic enzyme deficiency
Chest infections common
New born screening.
Describe CF complications relevant to the lungs. (5)
Bronchiectasis, pneumothorax, Resp failure, haemoptysis.
Describe CF complications relevant to the Upper respiratory tract. (2)
Chronic sinusitis, nasal polyps.
Describe CF complications relevant to the Pancreas (2)
Diabetes, pancreatic insufficiency
Describe CF complications relevant to the Gut (1)
Oesophageal reflux.
Describe CF complications relevant to the Liver (2)
Chronic liver disease, portal hypertension.
Describe CF complications relevant to the Biliary tree. (1)
Gallstones
Describe CF complications relevant to the Heart (1)
Cardiac failure
Describe CF complications relevant to the Bones (2)
Arthritis, osteoporosis
Describe CF complications relevant to the Reproductive system. (2)
Males congenitally infertile due to bilateral absence of Vad Deferens.
Describe th lifestyle advice given to a patient with CF. (8)
No smoking
Avoid sites of infection - jacuzzis (pseudomonas), other CF patients, friends with colds, clean nebs properly, stables/rotting veg (aspergillus).
Annual flu jab
NaCl tablets for exercise in hot weather.
Describe the management for CF. (3)
Maintain lung health and nutrition
Genotype specific therapies
Managing other co-morbidities - diabetes, liver disease.
