Lecture - CF As A Multisystem Disease Flashcards
1
Q
CF - Pathogenesis
A
- abnormal gene - abnormal CFTR - altered ion transport - abnormal mucus secretion - Exocrine duct obstruction, infection and inflammation - tissue destruction - organ destruction - resp failure
2
Q
CF- diagnosis
A
- Newborn screening: IRT + DNA analysis majority
- clinical features
- Sweat chloride test (CF > 60mmol)
- genomic screening
3
Q
CF - a multisystem disease
A
- Respiratory: - lung, nose and sinuses
- GI: pancreas, liver, biliary tree, small bowel
- Reproductive: testes/ vas deferens
- Fluid/electrolytes: sweat duct
4
Q
CF goals of management
A
- delay/prevent disease progression
- prevent cross infection CF organism
- optimise growth and nutrition
- maintain quality of life of child + family
- encourage development independence
- support transition to adult care
5
Q
Medical management of respiratory issues
A
- antibiotics
- mucolytics: pulmozyme, HTS
- physiotherapy + exercise
6
Q
Gastrointestinal management
A
- high calory, fat, protein diet
- pancreatic enzyme supplements
- Vitamins ADEK
7
Q
Other management
A
- management of complications
- strict infection control measures
