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11 Cards in this Set
- Front
- Back
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Cystic Fibrosis
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mutation in CFTR gene on long arm of chromosome 7
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Clinical presentations that may indicate CF
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respiratory - Bronchitis, chronic cough, recurrent pneumonia, nasal polyps
Staph or Pseudomonas pneumonia chronic pulmonary disease GI - meconium ileus, failure to thrive, steatorrhea, rectal prolapse, intestinal blockages, pancreatitis, hepatic disease other - digital clubbing, dehydration, diabetes |
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Diagnosis of CF
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phenotypic features
or sibling with CF or positive newborn screen And Lab evidence (high Cl is sweat, gene analysis) |
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Sweat Chloride Testing
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at least 48 hours old
ionophoresis and pilocarpine must not cross the heart sweat collecteed for 30 mins must collect 75 mg must be repeated |
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Cystic Fibrosis and GI problems
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pancreatic insufficiency
malnutrition meconium ileus diabetes liver disease bowel obstruction GERD |
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measuring pancreatic function
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fecal elastase levels
72 hour fecal fat collection |
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Nutrition intervention for CF
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assess and monitor growth
supplement pancreatic enzymes calorie supplements fat soluble vitamins |
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pathogenesis of lung disease in CF
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bronchial obstruction -> infection -> inflammation -> bronchiectasis
alveoli are spared |
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Complications of CF Lung disease
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airway obstruction
atelectasis bronchiectasis pneumothorax massive hemoptysis pulmonary hypertension cor pulmonale |
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Treating CF Lung disease
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improve chest clearance
antimicrobial anti-inflammatory |
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CF and the Pancreas
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pancreatic duct block -> undigested fat in stool -> deficiencies which lead to night blindness, skin disorders, rickets, and excessive bleeding
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