Cystic Fibrosis Flashcards
1
Q
important CFTR mutation
A
- delta F508
- loss in both alleles
2
Q
cystic fibrosis genetic pattern
A
- autosomal recessive
3
Q
respiratory manifestations of CF
A
- infection
- inflammation
- mucous
- airflow obstruction
4
Q
GI tract manifestations of CF
A
- meconium ileus
5
Q
sweat glands manifestations of CF
A
- salty sweat
6
Q
liver and pancreas manifestations of CF
A
- plugging of small bile and exocrine ducts
- pancreatic insufficiency
- cystic fibrosis related diabetes
7
Q
reproductive organs manifestations of CF
A
- infertility
8
Q
bone manifestations of CF
A
- osteopenia/osteoporosis
9
Q
treatment of meconium ileus
A
- enemas
- surgical
10
Q
meconium ileus associated with
A
- atretic segments of intestine
- microcolon
11
Q
other CF related GI diseases
A
- microbladder/gallstones
- focal biliary cirrhosis -> liver failure
- GERD
- altered motility
12
Q
result of pancreatic insufficiency
A
- decreased production of pancreatic/digestive enzymes
13
Q
treatment of pancreatic insufficiency
A
- pancreatic enzyme replacement therapy
- fat soluble vitamins
14
Q
treatment of CF related diabetes
A
- insulin
15
Q
treatment of CF
A
- IV antibiotics
- chest physical therapy
- airway clearance
