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56 Cards in this Set
- Front
- Back
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In CF, what is the main dysfunction that leads to mucosal obstruction and altered fluid viscosity?
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CF transmembrane regulator chloride channel dysfunction
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What happens to sodium and chloride in CF?
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decreased chloride secretion and increased sodium absorption
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This complication of CF is characterized by thick mucus secretions, imparied mucus clearance, chronic airway infection and colonization, obstruction, and an exaggerated neutrophil-dominated inflammatory response
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pulmonary disease
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The nutritional status of CF patients can be maximized by what methods?
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pancreatic enzyme replacement
vitamin and nutritional supplementation |
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This non-pharm therapy is necessary for all CF patients to clear secretions and control infection
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airway clearance therapy
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What are the 3 situations that would indicate a need for antibiotic therapy?
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1. early eradication and delay of colonization
2. suppression of bacterial growth once colonization occurs 3. reduction of bacterial load in acute overgrowth |
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In severeCF exacerbations, what antibiotic regimen is commonly used?
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IV antipseudomonal B-lactam + aminoglycoside
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Drug administration to CF patients usually require more drug for these reasons
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1. CF patients have larger volume of distribution
2. CF patients have higher total body clearance |
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Titration of pancreatic enzymes is based on what 3 conditions?
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control of steatorrhea
stool output abdominal symptoms |
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CF related diabetes results from what defect?
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insulin insufficiency
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How does CF cause small bowel obstruction?
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abnormally thick meconium
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how does CF cause distal intestinal obstruction?
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fecal impaction in terminal ileum and cecum
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how does CF cause maldigestion?
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obstruction of pancreatic ducts due to thick pancreatic secretions
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how does CF cause hepatobiliary disease?
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bile duct obstruction from abnormal bile composition and flow
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how does CF cause delayed puberty?
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female - increased viscosity of cervical mucus
male - absence of vas deferins w/ resultant obstruction |
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How dose CF cause bone disease
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malabsorption of vit D, K, and calcium
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how does CF cause anemia?
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impared erythropoietin regulation, nutritional factors, or chronic inflammation
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What is used to diagnose CF?
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2 seperate elevated sweat chloride conctrations of 60 meq/L or greater via pilocarpine iontrophoresis
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What are the symptoms of pulmonary CF?
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chronic cough
sputum production decreased exercise tolerance recurrent respiratory tract infections |
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What are the symptom of GI CF?
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foul-smelling stool
flatulence abdominal pain intestinal obstruction decreased bowel movements |
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What the symptoms of the nutrional state of CF?
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poor weight gain
voracious appetite dry skin, rash visual distrubances |
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What are the symptoms of CF related diabetes?
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weight loss
increased thirst frequent urination |
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What are the signs of CF?
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obstructive airway disease
failure to thrive salty taste to skin hepatobiliary disease recurrent pancreatitis |
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What vitamins are expected to be low in CF due to maldigestion?
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A, D, E, K
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In CF, glucose intolerance can be evaluated by blood glucose between what values after oral glucose tolerance test
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140-200 mg/dL
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In CF, CF related diabetes can be evaluated by blood glucose between what values after fasting?
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126 mg/dL
200 mg/dL after oral glucsoe tolerance test |
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Hepatobiliary disease can be evaluated using what lab test?
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elevated:
AST, ALT AlkPhos glutamyltranferase bilirubin |
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What are the expected PFTs in pulmonary CF?
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low FEV1
low FVC increase RV |
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what are the different types of airway clearance therapy?
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chest physiotherapy (CPT)
oscillating positive expiratory pressure (OPEP) intraapulmonary percussive ventilation (IPV) high-frequency chest compression (HFCC) |
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What is the typical caloric requirement for CF patients?
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110-200% RDA
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This agent can be administered subsequently after albuterol to reduce sputum viscosity and enhance clearance
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dornase alfa
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what is the dose for dornase alfa?
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2.5mg 1-2x daily via neb
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This is sometimes used as an add on mucolytic agent or for sputum induction
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hypertonic saline for inhalation 7% or 3.5%
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What is the dose for hypertonic saline for inhalation?
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4 ml 1-4x daily via neb
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Patients with recurrent wheezing or dyspnea who have demonstrated improvement with albuterol should consider using this agent
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laba
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this agent may attenuate reactive airways and reduce inflammation, but clear benefits in CF has not been established
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ICS
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these agents can be used for CF patients with reactive airways or allergic rhinitis symptoms
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montelukast
antihistamines intranasal steroids |
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This agent should only be used as added short courses in acute exacerbations or for treatment of allergic response to aspergillus
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systemic corticosteroids
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This agent, at high doses, has been shown to slow progression of CF with mild lung disease
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IBU
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what is the dose of IBU?
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20-30 mg/kg BID
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This antibiotic can be used as an antiinflammatory agent in CF
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azithromycin
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What is the dose of azithromycin in CF therapy
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40kg or above: 500 mg TIW
<40kg: 250 mg TIW |
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When should an acid-fast bacillus sputium culture be obtained in patients taking azithromycin?
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prior to initiation of therapy then q6months
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What should recent onset or mild symptoms of respiratory infection be treated with?
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oral or inhaled antibiotics for 14-21 days
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What should P. aeruginosa infection be treated with?
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oral flouroquinolones + inhaled tobramycin or colistin
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What should an MSSA infection be treated with?
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oral augmentin, dicloxacillin, first or second generation cephalosporin, bactrim or clindamycin
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What should MRSA infection be treated with?
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oral bactrim, clindamycin, minocycline, or linezolid
inpatient: vancomycin |
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What should H. flu infection be treated with?
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aumentin, cephalosporin or bactrim
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what should S. maltophilia be treated with?
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bactrim or minocycline
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what antibiotics are prefered due to it's excellent bioavailability?
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flouroquinolones, bactrim, minocycline, and linezolid
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What is the initial dose of pancreatic enzymes?
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500-1000 units of lipase/kg/meal
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Howare pancreatic enzymes administered?
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titrated at 2 to 3 week intervals in increments of 150-250 units of lipase/kg/meal
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What is the risk of using high doses of pancreatic enzymes (over 2500 units/kg/meal)
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fibrosing colonopathy
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Are pancreatic enzymes absorbed in an acidic or basic environment?
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basic
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What is used to slow the progression of liver disease?
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ursodiol 15-20mg/kg/day
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What is used to treat distal intestinal obstruction syndrome?
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oral or nasogastric administration of PEG
or enemas |
