Session 6- Cystic fibrosis

Question 1

What is cf

Answer 1

Autosomal recessive disease caused by mutations in he CFTR leading to a multiply stem disease most commonly affecting respiratory and GI tract

Question 2

How can we diagnose CF

Answer 2

• family history
• increased sweat chloride concentration
• demonstrations of abnormal nasal epithelium ion transpose

Question 3

Meconium ileus

Answer 3

In 15-20% of new bowel is blocked by sticky secretions

Question 4

signs of meconium ileus

Answer 4

intestinal obstruction soon after birth, bilious vomiting, abdominal distension and delay in passing meconium

Question 5

main CF clinical presentations

Answer 5

1. meconium ileus
2. intestinal malabsorption
3. recurrent chest infections
4. newborn screening

Question 6

lung CF pulmonary complications

Answer 6

bronchiectasis
pneumothorax
ABPA
haemoptysis
end stage- resp failure

Question 7

CF complications with nasal/ upper resp tract

Answer 7

chronic sinusitis

nasal polypsosis

Question 8

bronchectasis

Answer 8

pathological condition characterised by irreversibly dilated bronchi with associated mucous accumulation, impaired muco-cilary function, inflammation and recurrent infections

Question 9

clinical signs of bronchectasis

Answer 9

pulse ox may reveal hypoxaemia

fever

haemoptysis

fine crackles

high pitched inspiratory crackles

rhonchi

Question 10

what is the most common causative pathogen for pneumonia in CF patients

Answer 10

t in people with CF their respiratory flora is altered and Pseudomonas
aeruginosa becomes a major pathogen.