cystic fibrosis Flashcards
cystic fibrosis
inherited disease
most lethal inherited disorder in the US
affects about 1 in 2000 individual mostly caucasian european dissent
organ envolved in CF
lungs- bronchiectasis
pancreas- exocrine insufficiency
elevated sweat chlorides
etiology for CF
autosomal recessive gene
working tower in utero treatments
person afflicted with gene must receive two CF genes
person have and not display any symptoms: carrier for disorder
if two carriers have offspring 1 in 4 will contract CF
What is probable of Caucasion carry gene
1 in 16 or 1 in 25 carry gene
main problem on anatomy level is ?
Cf contain codes which regulate flow of ions through glands that secrete fluid
patients can’t regulate their salt concentration
pnacreatic insufficiency
pancreatic ducts become obstructed and increase saline content
organ becomes smaller and fibrotic
pantients expriences recurrent pancreatitis
pulmonary disease
most serious clear their own secretion
recurrent pulmonary infection
patient can’t clear their own secretion: increase quantity and viscosity
the retain secretion become a place for infection
bronchiectasis
the airway undergo structural changes resulting in permanent dilation
cause by chronic plugging
hyperactivity
bronchospasm is common in these patients
medical history
diagnosed early
usually before age 2
most physician are alert to the disorder and it is one of the rule outs when patients present recurrently
features of CF
recurrent pulmonary infection
chronic cough and wheezing
as the disease progresses, bronchieactisis and digital clubbing become events
what happens in pancreas
does produce enzymes that would digest food so patient need to take oral supplements
what happens in sweat glands in CF
elevated sweat glands chloride
physical examination
usually abnormal with in few years of diagnosis
respiratory distress and use of accessory muscles
chronic productive cough
digital clubbing and cyanosis
breath sounds in CF
coarse crackle and rhonchi
wheezing
