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15 Cards in this Set
- Front
- Back
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Hallmarks of CF |
salty skin appetite poor weight gain coughing wheezing lung infections endobronchial disease |
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Clinical signs |
obstruction of tissue with tubular epithelial strucuts median age is 40 years mucus in lungs and sinuses |
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When the CFTR isnt workin |
water is driven into the cell because the chloride isnt driven out so Na comes in tand the mucous becomes much thicker can also be associated with damages in the mucocilliary escaltor |
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How to relieve mucos |
Chest physiotherapy pneumatic vest internal vibration positive pressure devices exercise ion channel approahces; hydrating mucus by putting a hypertonic saline, dnase |
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Endobronchial disease |
neutrophilic inflammation to try and get rid of the mucus and infection, leaving their proteases behind whcih can cause bronchiectasis (become like tube socks, cant keep them open to trap mucus) and damage give DNAase to chop up the NDA but not working too crude |
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Recombinant human DNase |
no reactions too neutrophul released DNA and f actin was digested and decreased the viscosity pulmozyme |
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Controlling drugs |
macrolide has anti inflammatory properties even if they dont actual target a bacteria ibuprofen to decrease neutrophils |
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Endobronchial colonization |
pseudomonas increased in biofilm and then becomes the mucoid alginate strain which is nearly impossible to remove other organisms MRSA, burkholderai, stenotrophmonas, atypical mycobacteria, fungi |
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Fighting infections |
antistaphylococcal and antipseudomonal inhaled aminoglyoside tobramyocine aztreonam |
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Mgmt goals |
active participation in self managment promote adequate growth prevent complications with immunizations, regular airway clearance, mucoactive agents, anti inflammatories |
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Therapeutc approaches |
5 classes of mutations defective production I defective processing II defective regulation III defective conductance IV reduced amounts V |
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F508 del |
combo of II and III classes everyone has deletions but homozygous is rarer |
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G551D |
class III |
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Ivacaftor |
imrpoved lung function and use swaet chloride as an outcome specific for the G551D mutation |
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Lumacaftor |
CFTR correctors works in the F508 deletion to suppress the misfolding of the protein due to the phenylalaine substitution |
