Cystic Fibrosis

Question 1

CF is inherited in what pattern?

Answer 1

Autosomal recessive

Question 2

Abnormal gene causes product of this protein?

Answer 2

Cystic fibrosis transmembrane conductance regulator (CFTR)

Question 3

Gene mutations can cause these problems

Answer 3

Defective CFTF production, CFTR processing within cytoplasm, regulation, ion conduction

Question 4

Signs and symptoms of CF?

Answer 4

Salty sweat, lung infections, sinus infections, shortness of breath, FTT, fatty stools, delayed puberty, infertility

Question 5

Tissues affected by CF?

Answer 5

Airway, liver, sweat glands, reproductive, pancreas

Question 6

Cycles of this cause permanent damage to respiratory system?

Answer 6

Inflammation and infection

Question 7

Low volume hypothesis?

Answer 7

Excessive Na reabsorption and decreased Cl excretion causes dehydration of airway surface cells

Question 8

Dehydration of airway surface cells causes

Answer 8

Compresses of ciliary and decreased ciliary elevator

Question 9

Dehydration causes mucus layer compression of ciliary , and dried mucus forms hypoxic pockets of tissue that _______?

Answer 9

Breed bacteria

Question 10

Can infections be cured? common types of infections in CF?

Answer 10

No they are controlled. S. aureus, MRSA, P. aeroginosa, Burkholderia, Aspergillus

Question 11

Complications of pulomnary disease of CF?

Answer 11

atelectasis, pneumothorax, hemoptysis, nasal polyps

Question 12

This is happens in about 1/4th of infants with CF?

Answer 12

Meconium ileus (thick and gooey stools)

Question 13

90% of patients with CF develop this?

Answer 13

Pancreatic insufficiency

Question 14

Part of sex organ that may be absent in males?

Answer 14

Vas deferens

Question 15

Virtually all males have this?

Answer 15

Azospermia

Question 16

CF can produce DM via?

Answer 16

Fatty replacement of islet cells and decreased insulin production

Question 17

Newborn screening for measures this which is high in pancreatic injury?

Answer 17

Immunoreactive trypsinogen IRT

Question 18

This results on the sweat test is diagnosit for CF?

Answer 18

> 60mmol/cl

Question 19

Treatments used for airway clearance?

Answer 19

PEP (positive expiratory pressure) VEST (high frequency chest wall oscillation) these need done twice a day

Question 20

This chemical treatment thins mucus secretions?

Answer 20

Dornase alfa (human DNAse)

Question 21

This treatment helps control infections by getting high concentrations in the lungs?

Answer 21

Inhaled tobramycin and aztreonam

Question 22

This disease causes dilation of the bronchi and bronchioles secondary to airway wall thickening?

Answer 22

Bronchiecstasis

Question 23

Causes of bronciestasis?

Answer 23

CF, recurrent infection, airway obstruction, immunodeficiency, airway injury, connective tissue abnormality,

Question 24

Findings on high resolution CT scan on chest?

Answer 24

dilated lumen, tram track, extension into periphery, lack of tapering, focal air trapping

Question 25

CXR of bronchiectasis findings?

Answer 25

interstitial markings increased, thickened airway walls, dilated lumen, hyperinflation

Question 26

Bronchiectasis management?

Answer 26

Treat underlying disease, antibiotics as needed, chest physiotherapy, bronchodilators, DNAse, surgical resection, lung transplantation