Cystic Fibrosis

What this deck covers

Cystic Fibrosis in thirteen clinical teaching cases with worked explanations. Each case gives you a clinical vignette, four options, the correct answer, and an explanation of why each of the other three is wrong.

Diagnoses and management options tested

Across the thirteen cases you are asked to choose between options such as: Meconium ileus due to cystic fibrosis, Hirschsprung disease, Intestinal atresia, Malrotation with volvulus, Janeway lesions and allergic rhinitis, Osler nodes and nasal foreign body, Digital clubbing and nasal polyps, Paronychia and adenoid hypertrophy, Diagnostic of cystic fibrosis, Intermediate or borderline, requiring repeat testing, Normal result, CF excluded, Inconclusive due to the infant age, Class II — corrects protein misfolding, Class IV — improves channel conductance, Class V — increases protein quantity, Cystic fibrosis, CRMS / CFSPID, Cystic fibrosis carrier, Primary ciliary dyskinesia, Initiate a low-fat diet to reduce steatorrhoea, Ivacaftor (Kalydeco) monotherapy, Lumacaftor/ivacaftor (Orkambi), Tezacaftor/ivacaftor (Symdeko), Elexacaftor/tezacaftor/ivacaftor (Trikafta/Kaftrio), Oral ciprofloxacin alone, A single IV antipseudomonal agent.

Clinical pearls from this deck

• Meconium ileus is the earliest manifestation of CF, occurring in 15–20% of affected neonates — thick inspissated meconium obstructing the distal ileum. The soap bubble (Neuhauser) sign on abdominal radiograph is characteristic.
• Digital clubbing — loss of the normal Lovibond angle, which exceeds 180 degrees, with a positive Schamroth window test — develops by school age in most CF patients. Bilateral recurrent nasal polyps occur in 10–25%, and chronic pansinusitis is nearly universal.

The thirteen worked cases in this deck, with the images and the full explanation of every option, are part of Pediatric Case Review membership. See what is included.

More from this system: all metabolic and genetic decks and question sets · pediatric reference values.