Bronchiectasis and Cystic Fibrosis Flashcards

1
Q

What is bronchiectasis

A

Bronchiectasis - chronic dilatation of one or more bronchi

Bronchi exhibit poor mucus clearance and there is predisposition to recurrent or chronic bacterial infection

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2
Q

What sign is common on a CT of a patient with bronchiectasis

A

Signet ring sign

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3
Q

What are the symptoms of bronchiectasis

A

Chronic cough

Daily sputum production

Breathlessness on exertion

Intermittant haemoptysis

Nasal symptoms/upper resp symptoms

Chest pain

Fatigue

Wheeze - less common

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4
Q

Name the causes of bronchiectasis

A

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

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5
Q

What organisms commonly cause bronchiectasis

A

Haemophilus influenzae

Psuedomonas aeruginosa

Strep pneumoniae

Fungi - aspergillus, candida

Nontuberculous mycobacteria

Staph aureus

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6
Q

What is the management for bronchiectasis

A

Physio/airway clearance

Sputum sampling

Exclude immunodeficiency

Treat identifiable causes

Flu vaccine

Pulmonary rehabilitation if MRC dyspnoea score ≥3

Establish MDT

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7
Q

How does a patient with an exacerbation of bronchiectasis present with

A

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

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8
Q

What is cystic fibrosis

A

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

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9
Q

How is cystic fibrosis diagnosed

A

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

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10
Q

How can CF present

A

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

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11
Q

Where can complications of CF occur in the body

A

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

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12
Q

What lifestyle advice is given to patients with CF

A

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

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13
Q

How is CF managed

A

Holistic care

Maintaining lung health and nutritional state

Targeted therapies

Managing co-morbidities

Concordance with treatment

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14
Q

Name some features of cystic fibrosis

A

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

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