Flashcards in Cystic Fibrosis Deck (26):
CF is inherited in what pattern?
Abnormal gene causes product of this protein?
Cystic fibrosis transmembrane conductance regulator (CFTR)
Gene mutations can cause these problems
Defective CFTF production, CFTR processing within cytoplasm, regulation, ion conduction
Signs and symptoms of CF?
Salty sweat, lung infections, sinus infections, shortness of breath, FTT, fatty stools, delayed puberty, infertility
Tissues affected by CF?
Airway, liver, sweat glands, reproductive, pancreas
Cycles of this cause permanent damage to respiratory system?
Inflammation and infection
Low volume hypothesis?
Excessive Na reabsorption and decreased Cl excretion causes dehydration of airway surface cells
Dehydration of airway surface cells causes
Compresses of ciliary and decreased ciliary elevator
Dehydration causes mucus layer compression of ciliary , and dried mucus forms hypoxic pockets of tissue that _______?
Can infections be cured? common types of infections in CF?
No they are controlled. S. aureus, MRSA, P. aeroginosa, Burkholderia, Aspergillus
Complications of pulomnary disease of CF?
atelectasis, pneumothorax, hemoptysis, nasal polyps
This is happens in about 1/4th of infants with CF?
Meconium ileus (thick and gooey stools)
90% of patients with CF develop this?
Part of sex organ that may be absent in males?
Virtually all males have this?
CF can produce DM via?
Fatty replacement of islet cells and decreased insulin production
Newborn screening for measures this which is high in pancreatic injury?
Immunoreactive trypsinogen IRT
This results on the sweat test is diagnosit for CF?
Treatments used for airway clearance?
PEP (positive expiratory pressure) VEST (high frequency chest wall oscillation) these need done twice a day
This chemical treatment thins mucus secretions?
Dornase alfa (human DNAse)
This treatment helps control infections by getting high concentrations in the lungs?
Inhaled tobramycin and aztreonam
This disease causes dilation of the bronchi and bronchioles secondary to airway wall thickening?
Causes of bronciestasis?
CF, recurrent infection, airway obstruction, immunodeficiency, airway injury, connective tissue abnormality,
Findings on high resolution CT scan on chest?
dilated lumen, tram track, extension into periphery, lack of tapering, focal air trapping
CXR of bronchiectasis findings?
interstitial markings increased, thickened airway walls, dilated lumen, hyperinflation