CF, D Kinder, DSA Flashcards
What are signs of acute exacerbation CF in lungs
cough, dyspnea, decreased exercise tolerance, fatigue, increased sputum production
What bacterial agent causes infections regularly in CF
pseudomonas
then Staph and MRSA
allergic: bronchopulmonary mycoses and nonTB mycobacterium
what does CF cause in sinuses
pan sinusitis and nasal polyposis
how does CF affect pancreas
acinar dilation from viscous obstruction
leads to exocrine pancreatic insufficiency in 1st year of life
have malabsorption especially of fat soluble vitamins
leads to DM by age 30
how does CF affect intestine
intestinal obstruction, constipation and chronic pain.
meconium ileus secondary to obstruction occurs 15% at birth
how does CF affect liver
bile duct obstruction leading to HSM, hematemesis in some with esophageal or gastric varices secondary to portal HTN
how does CF affect sweat gland
failure of Cl absorption from lumen into ductal lining cell,
marked elevation of Cl and Na in sweat
how does CF affect vas deferens
obstructed as a fetus and reabsorbed, almost all males are sterile
how does CF affect MSK
decreased bone density secondary to decreased absorption of Vit D, glucocorticioids and decreased exercise
What is the marker for pancreatic injury
immunoreactive trypsinogen
What is the confrimatory Dx for CF
sweat testing
how does CF present on PFT
obstruction
CF on CXR
hyperinflation
Tx for CF
oral antibiotics
IV antibiotics
What occurs to epithelium in CF
increase in Na absorption, thinning airway surface liquid lining impairing mucociliary clearance
Neutrophil response leading to inflammatory damage
Most common mutation in CF
F508 delta
what is the second most common mutation in CF
G542X
What is class I CF
protein not produced from nonsense mutation
What is class II CF
defective protein processing or traddicking
What is class III CF
protein is produced but channel is closed from physiologic stimuli
What is class IV CF
present in membrane and result in a channel that opens only partially in response to stimuli
What is class V mutation
normal CFTR but in reduced amounts because of defective splicing
When do the pulmonary Sx arise in CF
adolescence
What does cT show in CF
bronchiectasis
What bacteria can lead to sepsis in CF
Burkholderia infection
What is Ddx for respiratory CF
primary ciliary dyskinesia, immune deficiency or postinfectious bronchiectasis
