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31 Cards in this Set
- Front
- Back
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what does the defective CF gene cause? |
improper na and cl |
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why does cf lead to infection |
mucus becomes thick and cannot be cleared, impaired host defense |
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what bacteria usually causes infection? |
pseudomonas |
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how can CF affect the pancreas and liver |
prevents the release of digestive enzymes and bile leading to malnourshment |
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how can CF lead to diabetes |
causes scarring of the pancreas and prevents insulin release, glucose is no longer under control
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what gene is defective |
CFTR-cystic fibrosis transmembrane conductance regulator |
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how does Cf affect the sweat glands? |
there is a greater loss of Na and Cl,salty skin same idea with lungs, cl does leave, neither does na or water |
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why does cf lead to infertility? |
prevents formation of vas deferenes, mucus clogs off tubes |
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what can happen in the GI tract from CF |
intussaception |
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how does CF affect bone density |
vitamin D isnt absorbed because liver is not producing bile, and vitamin d is fat soluble |
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how is cystic firbrosis diagnosed |
sweat test, genetic testing |
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treating CF starting with GI |
enzymes and high calories and insulin |
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treating cf with in the lungs |
clear mucus, anti inflammatory drugs, abx |
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what two agents are used to thin the mucus? |
dornase alfa (destroys dna from neutrophils) and hypertonic saline |
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what medication is best used for P aeruginosa infection? |
inhaled tobramycin |
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what is used to treat a patient colonized with P aeruginosa to help reduce inflammation |
azithromycin |
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are steriods okay to use? |
never use systemic, ICS only if they have asthma of COPD |
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most common CFTR mutation? |
delta F508 |
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describe the different classification of CFTR mutations |
class 1 no protein class 2 doesnt reach surface class 3 defect in regulation class 4 altered conductance class 5 not enough made |
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what classes of CFTR mutations usually have pancreatic issues and diabetes? |
1-3 |
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what is cascade testing? |
individual does a reproductive genetic test |
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what is population testing? |
screening for CF in those who do not have a family history of CF |
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most infections are caused by what baceteria? |
psuedomonas aerguinosa and MRSA and B cepacia
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what is responsible for most CF deaths? |
pulmonary insufficency |
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name another inhaled antibiotic that is used to maintain pseudomonas |
cayston/ aztreonam |
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other than azithromycin what drug can be used for inflammation? |
ibuprofen, but cant be used to much because of renal side effects in combo with aminoglycoside |
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what baceteria is associated with allergic bronchopulmonary asperigillosis |
aspergillus fumigatus |
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how is ABPA treated? |
systemic steriods |
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what does RICH stand for |
respect, information, connection, hope |
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what compound usually regulates the CFTR protein? |
cAMP |
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what two genes alter CF severity |
TGF b1 and MBL2 |
