cystic fibrosis Flashcards
1
Q
how do most people present initially with CF?
A
- meconium ileus
- Guthrie test
2
Q
how is the diagnosis made?
A
- sweat test
- >60 mmol of Cl in 100mg
3
Q
what does a CXR look like in CF?
A
- overinflation of lungs
- hilar enlargements
- bronchiectatic changes (hilar enlargement/ ring shadows)
- consolidation
CXR can be normal in early stages
4
Q
what are the stools like in CF?
why do CF patients get malabsorption?
what signs may you see of malabsorption?
A
- frequent, bulky, greasy
- pancreatic exocrine insufficiency
- protuberant abdomen, decreased muscle mass and poor growth
5
Q
how is the respiratory side managed?
A
- clear secretions
- prevent and treat infections
- regular chest physio
- prolonged antibiotic therapy
6
Q
the GI effects?
A
- pancreatic enzyme replacement
- supplementary vitamins (A,D,E)
- high energy and protein diet
7
Q
what other more broad effects are there of CF?
A
- diabetes in adolescence
- most males are azoospermic, unimpaired sexual function
- delayed puberty
