CF, D Kinder, DSA

Question 1

What are signs of acute exacerbation CF in lungs

Answer 1

cough, dyspnea, decreased exercise tolerance, fatigue, increased sputum production

Question 2

What bacterial agent causes infections regularly in CF

Answer 2

pseudomonas
then Staph and MRSA
allergic: bronchopulmonary mycoses and nonTB mycobacterium

Question 3

what does CF cause in sinuses

Answer 3

pan sinusitis and nasal polyposis

Question 4

how does CF affect pancreas

Answer 4

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

Question 5

how does CF affect intestine

Answer 5

intestinal obstruction, constipation and chronic pain.

meconium ileus secondary to obstruction occurs 15% at birth

Question 6

how does CF affect liver

Answer 6

bile duct obstruction leading to HSM, hematemesis in some with esophageal or gastric varices secondary to portal HTN

Question 7

how does CF affect sweat gland

Answer 7

failure of Cl absorption from lumen into ductal lining cell,

marked elevation of Cl and Na in sweat

Question 8

how does CF affect vas deferens

Answer 8

obstructed as a fetus and reabsorbed, almost all males are sterile

Question 9

how does CF affect MSK

Answer 9

decreased bone density secondary to decreased absorption of Vit D, glucocorticioids and decreased exercise

Question 10

What is the marker for pancreatic injury

Answer 10

immunoreactive trypsinogen

Question 11

What is the confrimatory Dx for CF

Answer 11

sweat testing

Question 12

how does CF present on PFT

Answer 12

obstruction

Question 13

CF on CXR

Answer 13

hyperinflation

Question 14

Tx for CF

Answer 14

oral antibiotics

IV antibiotics

Question 15

What occurs to epithelium in CF

Answer 15

increase in Na absorption, thinning airway surface liquid lining impairing mucociliary clearance
Neutrophil response leading to inflammatory damage

Question 16

Most common mutation in CF

Answer 16

F508 delta

Question 17

what is the second most common mutation in CF

Answer 17

G542X

Question 18

What is class I CF

Answer 18

protein not produced from nonsense mutation

Question 19

What is class II CF

Answer 19

defective protein processing or traddicking

Question 20

What is class III CF

Answer 20

protein is produced but channel is closed from physiologic stimuli

Question 21

What is class IV CF

Answer 21

present in membrane and result in a channel that opens only partially in response to stimuli

Question 22

What is class V mutation

Answer 22

normal CFTR but in reduced amounts because of defective splicing

Question 23

When do the pulmonary Sx arise in CF

Answer 23

adolescence

Question 24

What does cT show in CF

Answer 24

bronchiectasis

Question 25

What bacteria can lead to sepsis in CF

Answer 25

Burkholderia infection

Question 26

What is Ddx for respiratory CF

Answer 26

primary ciliary dyskinesia, immune deficiency or postinfectious bronchiectasis