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3 Cards in this Set
- Front
- Back
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Pathogenesis of CF |
AR disease due to defect in CFTR gene (cystic fibrosis transmembrane conductance regulator protein) on chromosome 7 - CFTR located on all exocrine tissues - defective CFTR protein prevents chloride moving out of cells > Na hyperabsorption > osmotic pull of water into cells > dehydration of extracellular surfaces > thick viscous secretions easily amenable to colonization and infection |
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What other systems are affected in cystic fibrosis? |
- Respi: nasal polyps, otitis media, asthma, bronchiolitis, recurrent pneumonia, bronchiectasis - GI: pancreatic enzyme insufficiency with fat & protein malabsorption, diabetes, meconium ileus, distal I/O syndrome, focal biliary cirrhosis & portal HTN, cholelithiasis - Reproductive: male subfertility due to defective sperm transport - MSK: osteoporosis - ENT: sinus disease, nasal polyposis |
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What organisms commonly colonise respi tract in CF? |
- Haemophilus influenzae - Staphylococcus aureus - Pseudomonas - Burkholderia cepacia complex |
