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10 Cards in this Set
- Front
- Back
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Cystic fibrosis gene abnormality |
AR pattern, on chromosome 7, mutation on CFTR gene |
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Kartagener's syndrome |
Immotile ciliary syndrome - causes bronchiectasis |
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Most common causes of bronchiectasis |
Developed countries - Cystic fibrosis Underdeveloped countries - Tuberculosis |
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Frequent cause of bronchiectasis exacerbation |
Pseudomonas aeruginosa |
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Diagnosis of cystic fibrosis |
Best initial test is a sweat chloride test, if >60mmol/L do DNA analysis of CFTR gene mutation for confirmation Pilocarpine is used to stimulate glands Supportive diagnostic tools: CXR- bronchiectasis (diffuse) - tram track lines, diffusely PFT- obstructive pattern Stool: low chymotrypsin and elastase (pancreatic insufficiency) HRCT |
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C. F. treatment |
Chest physiotherapy, airway clearance techniques Exacerbations: ATB (pseudomonas coverage : IV penicillin (oxacillin, methicillin) or aminoglycoside (gentamicin)) Dornase alpha nebulizer - mucolytic Oral supplementation of vitamins ADEK and pancreatic enzymes Liver transplant if cirrhosis Screen and treat DM Lung transplant |
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Thyroiditis |
Subacute granulomatous thyroiditis (deQuervain) =post viral Subacute lymphocytic thyroiditis =post partum Chronic lymphocytic thyroiditis = hashimotos Chronic fibrous thyroiditis = riedel's Radiation induced thyroiditis Amiodarone induced thyroiditis |
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Interstitial lung disease |
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Drugs that cause ILD |
Many - website pneumotox can be assessed for all information Most common: bleomycin, amiodarone, methotrexate, nitrofurantoin |
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Treatment of IPF |
Supportive: oxygen, pulmonary rehabilitation, opiates Pirfenidon and nintendanib: block TGF-beta which stimulates fibrin production and deposition in the lungs |
