Bronchiectasis, CF & small airways flashcards
19 high-yield MRCP Part 1 cards from the Intellect Medicos Respiratory Medicine course. Try to answer each question before you open it — then study them properly with spaced repetition, which brings each card back just before you would forget it.
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What is the gold-standard investigation for bronchiectasis?
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High-resolution CT (HRCT) of the chest.
Why? HRCT shows dilated, thick-walled bronchi that fail to taper. A chest X-ray may show tram-tracks and ring shadows but is insensitive.
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What is the "signet ring" sign on HRCT?
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A dilated bronchus larger than its accompanying pulmonary artery — a sign of bronchiectasis.
Why? Normally the bronchus and artery are about the same size; the ring is the bronchus and the "stone" is the artery.
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What is the most common organism isolated in non-CF bronchiectasis?
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Haemophilus influenzae.
Why? Pseudomonas aeruginosa is also common and is linked with more severe disease and faster decline.
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What is the mainstay of long-term non-drug management in bronchiectasis?
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Daily airway clearance physiotherapy (e.g. postural drainage).
Why? Clearing retained secretions breaks the "vicious cycle" of infection, inflammation and airway damage.
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What is the interventional treatment of choice for massive haemoptysis in bronchiectasis?
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Bronchial artery embolisation.
Why? The bleeding comes from hypertrophied bronchial arteries. Surgery is kept for localised disease or failure of other treatment.
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Kartagener's syndrome is the triad of bronchiectasis, chronic sinusitis and ______.
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situs inversus
Why? It is a subtype of primary ciliary dyskinesia: immotile cilia cause poor mucus clearance and random left–right organ positioning.
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What is the screening test for primary ciliary dyskinesia?
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Nasal nitric oxide (markedly reduced).
Why? Diagnosis is confirmed by ciliary function testing (beat frequency/pattern) and electron microscopy showing absent dynein arms.
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What structural ciliary defect causes primary ciliary dyskinesia, and how is it usually inherited?
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Absent or defective dynein arms; usually autosomal recessive (e.g. DNAH5, DNAI1 mutations).
Why? Without dynein arms cilia cannot beat, so mucociliary clearance fails and sperm are immotile.
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What distinguishes Young's syndrome from Kartagener's syndrome?
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In Young's syndrome cilia are NORMAL; infertility is due to obstructive azoospermia from thick epididymal secretions.
Why? Young's = sinopulmonary disease (bronchiectasis, sinusitis) + obstructive azoospermia + normal cilia, no situs inversus.
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What is the triad of yellow nail syndrome?
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Yellow, thickened, slow-growing nails; lymphoedema; recurrent pleural effusions.
Why? It is also associated with bronchiectasis.
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Which gene is affected in cystic fibrosis, and what is the commonest mutation?
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CFTR gene (chromosome 7); the commonest mutation is delta F508 (F508del).
Why? CFTR is a chloride channel; faulty CFTR causes thick secretions in lungs, pancreas, gut and vas deferens. Inheritance is autosomal recessive.
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What is the diagnostic test for cystic fibrosis, and what result is diagnostic?
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Sweat test: sweat chloride > 60 mmol/L.
Why? Defective CFTR stops chloride reabsorption in sweat ducts, so sweat is salty.
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Which organism most commonly colonises the lungs of adults with cystic fibrosis?
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Pseudomonas aeruginosa.
Why? Staph aureus and H. influenzae dominate in childhood; Burkholderia cepacia is feared because it can contraindicate transplantation.
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What IV antibiotic combination is classically used for a Pseudomonas exacerbation of cystic fibrosis?
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Ceftazidime plus tobramycin (an anti-pseudomonal beta-lactam + an aminoglycoside).
Why? Two anti-pseudomonal agents are combined to improve killing and reduce resistance. Long-term nebulised tobramycin is used as suppression/prophylaxis.
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What must be monitored during IV tobramycin treatment, and why?
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Serum drug levels (and renal function), because aminoglycosides are nephrotoxic and ototoxic.
Why? Aminoglycosides have a narrow therapeutic index; high trough levels predict toxicity.
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How does ivacaftor work in cystic fibrosis?
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It is a CFTR potentiator: it increases the opening (gating) of CFTR channels at the cell surface.
Why? It is combined with CFTR correctors (elexacaftor and tezacaftor) as Kaftrio, recommended by NICE for people aged 2 and over with at least one F508del mutation.
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What is the "exam favourite" cause of bronchiolitis obliterans in adults?
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Lung transplantation (chronic lung allograft dysfunction).
Why? It presents with a persistent fall in FEV1 (> 20% from baseline) and fixed airflow obstruction.
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What is the key imaging investigation for bronchiolitis obliterans, and what does it show?
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HRCT (with expiratory views): mosaic attenuation and air trapping.
Why? Spirometry shows fixed (irreversible) obstruction, which distinguishes it from asthma.
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Name two drugs linked with bronchiolitis obliterans in the course notes.
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Penicillamine and gold.
Why? Both are older DMARDs; rheumatoid arthritis itself, Sjögren's and systemic sclerosis are also associations.