Cystic Fibrosis Flashcards Preview

Clinical Medicine- Pulmonary > Cystic Fibrosis > Flashcards

Flashcards in Cystic Fibrosis Deck (26):
1

CF is inherited in what pattern?

Autosomal recessive

2

Abnormal gene causes product of this protein?

Cystic fibrosis transmembrane conductance regulator (CFTR)

3

Gene mutations can cause these problems

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

4

Signs and symptoms of CF?

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

5

Tissues affected by CF?

Airway, liver, sweat glands, reproductive, pancreas

6

Cycles of this cause permanent damage to respiratory system?

Inflammation and infection

7

Low volume hypothesis?

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

8

Dehydration of airway surface cells causes

Compresses of ciliary and decreased ciliary elevator

9

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

Breed bacteria

10

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

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

11

Complications of pulomnary disease of CF?

atelectasis, pneumothorax, hemoptysis, nasal polyps

12

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

Meconium ileus (thick and gooey stools)

13

90% of patients with CF develop this?

Pancreatic insufficiency

14

Part of sex organ that may be absent in males?

Vas deferens

15

Virtually all males have this?

Azospermia

16

CF can produce DM via?

Fatty replacement of islet cells and decreased insulin production

17

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

Immunoreactive trypsinogen IRT

18

This results on the sweat test is diagnosit for CF?

>60mmol/cl

19

Treatments used for airway clearance?

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

20

This chemical treatment thins mucus secretions?

Dornase alfa (human DNAse)

21

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

Inhaled tobramycin and aztreonam

22

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

Bronchiecstasis

23

Causes of bronciestasis?

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

24

Findings on high resolution CT scan on chest?

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

25

CXR of bronchiectasis findings?

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

26

Bronchiectasis management?

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