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22 Cards in this Set
- Front
- Back
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Be able to calculate odds future kids will have CF; be heterooxygote or homozygous carries** and calc probable carrier state
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Know it, look it up
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Be able to calculate odds that siblings, aunts, uncles w/ cf will have kid w/ cf |
Know it
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Whats most common gene mutation in CF:
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Delta F508
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A child of 2 carries has what chance of having CF?
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¼; AR
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What’s incidence of CF in US?
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1/3,000 for whites; 1/10,000 non white; 1/6,500 hispanic
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What are methods used for newborn screening?
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IRT (pancreatic enzyme precursor) and DNA testing or IRT/IRT approach; then sweat. Sweat < 30 in < 3 mo
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What happens to lungs and pancreas in CF?
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Endobronchitis and bronchiectasis; and exocrine insufficiency and CF related Diabetes
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What is mucoid PSA due to ?
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Release of exopolysaccharide
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What’s a frequent complx of cf?
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Nasal polyposis
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What’s very common in males w/ CF
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Sterility/ failure of vas deferens to develop
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What vitamin deficiency occur w/ CF in infancy and what’s complx?
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Vite E- hemolytic anemia; vit K: bleeding diathesis; zinc: acrodermatitis
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What electrolyte imbalances are abnl ininfancy?
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**hypochloremic, Metabolic alkalosis; Hyponatremia: dehydration, FTT; Hypoproteineinemia: kwashiorkor, false negative
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How do you dose panc enzyme replacement?
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500-2500 lipase units/kg/meal; fat sol vitamin supplements; hi cal diet
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What pft finding first occurs in cf lung disease?
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Peripheral airways drop: FEF 25-75, then FEV1
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What are recommended chronic lung therapies of CF? (> 6)
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Inhaled tobi; dornase; HS; macrolides, Ibprofen; albuterol
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What are complx of CF? and whats most common complx of CF in adolescents with h/o wt loss?
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Sterility, nasal polyposis, portal HTN, meconium ileus, rectal prolapse, hypochloremia; Most common complx: CFRD
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What’s indication of lung transplant?
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FEV1 < 30%;
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What’s dx criteria of sweat cl for CF?
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Greater than 60 mm/L
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How do you dx pancreatic insufficiency w/ fecal elastase?
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Fecal elastase < 100 microg/g stool
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How do you deal w/ first dx of PSA?
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inhaled TOBI w/ or w/o CIPRO; for acute inpt: 2 antbx
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How do you deal w/ chronic PSA infx?
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Inhaled TOBI for a while
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What’s dose of CF enzyme replacement?
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500-2,500 units lipase/kg/day; limit: 2500 U/ kg /meal or < 10,000 Units/kg/day to decrease fibrosing colonopathy |
