Session 7: Cystic Fibrosis and Bronchiectasis Flashcards
What is cystic fibrosis?
Autosomal recessive disease leading to a mutation in CFTR. Multisystem disease with thickened secretions.
How to diagnose CF.
Phenotypic features like a history of CF or a positive new born screening result.
Also a sweat test to check for increased sweat chloride concentrations.
Can also be done by demonstration of abnormal nasal epithelial transport.
How may CF present in newborns?
Meconium ileus
Intestinal malabsorption
Recurrent chest infections
Explain meconium ileus as a presentation of CF.
CF infants bowel might be blocked by sticky secretions. Leading to intestinal obstruction, bilious vomiting, abdo distension and delay in passing meconium.
Why do infants with CF present with intestinal malabsorption?
Main cause is du to a severe deficiency of pancreatic enzymes.
Signs and symptoms of CF.
Chronic sinusitis.
Recurrent lower resp tract infections.
Finger clubbing
Abnormal sweat secretions
Male infertility
Gall stones, liver disease and portal hypertension.
Common CF complications.
Recurrent resp infections
Low body weight
Distal intestinal obstruction syndrome
CF related diabetes
Management of resp infections due to CF.
Aggressive therapy with physio and antibiotics.
Often receiving prophylactic antibiotics to maintain health.
Explain cause of low body weight in CF.
Consequence of pancreatic insufficiency leading to intestinal malabsorption.
Management of low body weight due to CF.
Pancreatic enzyme replacement therapy.
High calorie intake and often extra supplements.
NG or PEG feeding
What is distal intestinal obstruction syndrome? (DIOS)
Due to intestinal contents in the distal ileum and proximal colon which are thick and dehydrated.
Mainly due to no pancreatic enzymes, salt deficiency or hot weather.
What’s the difference between DIOS and constipation?
DIOS is a faecal obstruction in ileo-caecum.
Constipation is whole bowel.
How to diagnose DIOS.
Palpable right iliac fossa mass.
Abdo x-ray
Management of CF in general.
No smoking
Avoid other CF patients
Avoid people with infections
Avoid jacuzzies (pseudomonas)
Keep things clean
Avoid fungi
Immunisation for influenza
NaCl tablets
What is bronchiectasis?
Chronic dilation of one or more bronchi with poor mucus clearance of the bronchi and recurrent or chronic bacterial infections.
What is Young’s syndrome?
A triad of bronchiectasis, sinusitis and reduced fertility.
What is Kartagener syndrome?
Triad of bronchiectasis, sinusitis and situs inversus.
Explain signet ring sign.
Dilated bronchus and accompanying pulmonary artery.
