8 - bronchiectasis and cystic fibrosis

Question 1

what is bronchiectasis?

Answer 1

chronic dilatation of one or more bronchi. they exhibit poor mucus clearance and there is predisposition to recurrent or chronic bacterial infection

Question 2

what are the causes of bronchiectasis?

Answer 2

POST INFECTIVE
whooping cough, TB

IMMUNE DEFICINECY

GENETIC/ MUCOCILIARY CLEARANCE DEFECTS
cystic fibrosis
primary ciliary dyskinesia
Youngs syndrome (bronchiectasis, sinusitis, reduced fertility)
Kartagener syndrome (brochiectasis, sinusitis, situs inversus)

OBSTRUCTION
foreign body, tumour, extrinsic lymph node

Question 3

what are the common organisms that cause bronchiectasis?

Answer 3

Haemophilus influenzae 
Pseudomonas aeruginosa 
moraxella catarrhalis 
fungi - aspergillus, candida 
non-tuberculous mycobacteria

less common = staphylococcus aureus (CF)

Question 4

what is the management of bronchiectasis?

Answer 4

treat underlying cause 
physiotherapy - mucus clearance 
Abx according to sputum cultures 
supportive - flu vaccine, bronchodilators 
pulmonary rehab

Question 5

what is cystic fibrosis?

Answer 5

autosomal recessive disease leading to mutations in the CFTR.
Causes a multisystem disease (resp and GI mainly) and thickened secretions

Question 6

how is a cystic fibrosis diagnosis made?

Answer 6

history of CF in a sibling
positive new-born screening test

AND

increased chloride in sweat
identification of two CF mutations
abnormal nasal epithelial ion transport

Question 7

how does cystic fibrosis present?

Answer 7

MECONIUM ILEUS
new-born CF infant the bowel may be blocked by sticky secretions. also may show bilious vomiting, abdominal distention and delay in passing meconium

INTESTINAL MALABSORPTION
more than 90% due to deficiency in pancreatic enzymes

RECURRENT CHEST INFECTIONS
NEWBORN SCREENING

Question 8

what are the features of cystic fibrosis?

Answer 8

• chronic sinusitis
• repeated lower respiratory chest infections
• liver disease, portal hypertension, gallstones
  steatorrhea
• distal intestinal obstruction syndrome
• nasal polyps
• abnormal sweat (high in sodium and chloride)
• pancreatic insufficiency, diabetes
• finger clubbing
• osteoporosis
• male infertility

Question 9

what are the common cystic fibrosis complications?

Answer 9

1. respiratory infections - abx and physio
2. low body weight - monitor, pancreatic enzyme replacements, high calories intake, supplements, NG PEG feeds
3. distal intestinal obstruction syndrome (DIOS)
4. CF related diabetes

Question 10

what is distal intestinal obstruction syndrome? (DIOS)

Answer 10

+ DIOS - faecal obstruction in ileo-caecum
+ constipation - faecal obstruction in the whole bowel

+ intestinal contents in distal ileum and proximal colon (thick and dehydrated faeces)
+ insufficient prescription of pancreatic enzymes or non-compliance
+ palpable right iliac fossa mass
+ diagnosis: symptoms, palpable right iliac fossa mass, AXR that shows faecal loading at junction of small and large bowel

Question 11

what lifestyle advice is given?

Answer 11

no smoking
avoid other CF patients
avoid friends/ relatives with colds/ infections
avoid jacuzzis (pseudomonas)
clean and dry nebs thoroughly
avoid stables, compost or rotting vegetation (aspergillus)
annual influenza immunisation
sodium chloride tabs in hot water/ exercise