Cystic Fibrosis and Bronchiectasis: Clinical Correlation Flashcards Preview

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Flashcards in Cystic Fibrosis and Bronchiectasis: Clinical Correlation Deck (23):
1

CF genetics

Auto recessive

CFTR mutation

IN US, DeltaF508

85% carry at least one copy and 50% homo

CFTR - membrane associated chloride channel and you get defective ion transport across and within epithelial cells

2

CFTR classes

Severe - pancreatic insufficiency and decreased sufficiency

Mild - pancreatic sufficiency

1 - stop codon
2 - doesn't fold right and can't get in golgi
3 - chloride channe lthere but it won't open
4- works but the defect is in the pore
5- transcription...not enough of it

3

Upper airways
Lungs
Panc
Liver
Intest
Resprodutive

CF

Chronic sinusitis

Recurrent infection, bronchiect

Pancreatic insuff, diabetes

Cirrhosies

Meconium ileus, distal intestinal obstruction

Infertility

4

Gold std dx

Sweat test

Chloride concentration is measured...higher is more indicative of CF (over 60)

5

Dx

One of these - over 1 typical phenotypic feautre, sibling, pos newborn screen

PLUS - elevated sweat, 2 ID'd CFTR genes, abnormal nasal potential diff test

6

Mucous clearance from the lung depends on

Hydrated airway surface fluid

7

Patho

Defective gnee

Def CFTR

Decreased chloride secretion and increased sodium absorption

Bornchial obnstruction

Infection

Inflammation

Bronchiectaiss

8

Broncheictasis

Acqwuired dz of major bronchi and bronchioles with permanent dilitation and destruction of bronchial walls

SUaully wit hchornic bac infection

9

Patho of bronchiect

Infection PLUS

Impaired mucociliary clearance, airway obstruction, or a defect in host defense

10

Bronch sx and signs

Cough, sputum, hemoptysis

Signs - crackles, wheezing/rhonchi, clubbing

11

Tx infeciton in CF

IV Abs to tx pseudomonas

Oral anti-psuedomonas ABs - approval of fluroquinos in children

INhaled ABs

Infection control practices

Surveillance of resp cultures

Pseudo erad protocols

Chronic suppression therapy

12

Tx inflammation

Oral CSs

Inhaled CSs

Azithromycin and ibuprofen (chronically)

13

Tx obstruction

Bronchodilators

Mucolytic agents - dornase

Rehydrating agents - hypertnic, asaline

Airway clearance

14

Nurtirion

Enteric coated pancreatic enzymes*****

May need supplements

15

Type 1 and 2

No synthesis

Block in processing

16

3,4,5

BLock in reg

Altered conductance

Reduced syn

17

2 types of problems

Reduced quantity (1,2,5) - corrector

Reduced function - 3,4 - need potentiator

18

Nroaml vs G551D

When protein binds cAMP, the chloride channel will open (normal)

in CF, might not bind the cAMP

19

Ivacaftor

Potentiator of CFTR

Facilitates increased CL transport by potentiating channel-open probability of G551D...potentiates binding of cAMP

20

Lumacaftor/Ivacvaftor

Indicated for CF homo for delta 508

21

Most CF is caused by

Processeing defect (golgi problem)

22

Lumacaftor

INcreases the amount of F508del-CFTR at the cell surface

If you add potnetiator, then it will help even more

23

Most important thing in CF

Monitoring lung function and try to intervene as early as possible