Cistic Fibrosis Flashcards
1
Q
Cystic Fibrosis
A
- must be inherited from both parents
- Gi issues related to blockage of pancreatic ducts
- respiratory issues related to mucus production
- fat absorption results
2
Q
Diagnosis of CF
A
- 2 positive sweat chloride tests *
- newborn screening for elevated IRT, and DNA tests for 40 most common Cf mutations
3
Q
Clinical features
A
- chronic sino pulmonary disease, nasal polyps, digital clubbing
- GI abnormalities: steatorrhea, fat-soluble vitamin def, chronic pancreatitis, hepatic manifestations
- salt loss
4
Q
Genetics of CF
A
- Cl- channel in apical surface of exocrine glands
- inability to move Cl- out of cells and increased movement of Na+ into cells *
- there is a net water movement into cells (water can’t leave) – thick tenacious secretions
5
Q
Consequences of CF
A
- respiratory
- gastrointestinal
- pancreatic
- reproductive
- clubbing of nails
6
Q
Respiration Manifestations in CF
A
infection, chronic cough, wheeze, shortness of breath:
- thick secretions trap bacteria - infections
- air trapping - barrel-chest
- destruction of lung tissue - respiratory failure
- destruction of lung tissue - respiratory failure
7
Q
GI Manifestations in CF
A
intestinal obstruction, liver failure (cirrhosis)
- thick meconium obstructs GI tract ~ vomiting and abdominal distention
- obstruction of biliary system - cirrhosis
- intestinal obstruction ~ meconium ilius and DIOS (distal intestinal obstruction syndrome)
- requires surgical intervention
8
Q
pancreatic insufficiency
A
- affects digestive enzymes
- 85% of CF patients at birth
- diabetes mellitus
pancratic destruction –> insufficient digestive enzymes –> protein/fat maldigestion –> significant energy loss
9
Q
reproductive system
A
- absence of vas deferens in >95% male infertility
- femal fertility reduced due to thick cervical mucus
10
Q
Therapy
A
- pumonary: antibiotics, broncodilators
- nutritional: enzymes, vitamins ADEK, supplemental feeds
