Term
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Definition
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|
Term
| Defective processing (CFTR mutation) |
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Definition
|
|
Term
| Defective Regulation (CFTR mutation) |
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Definition
|
|
Term
| Defective Conductance (CFTR mutation) |
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Definition
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Term
| Cystic Fibrosis Signs and Symptoms |
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Definition
| very salty-tasting skin; persistent coughing, at times with phlegm, frequent lung infections, wheezing or shortness of breath, poor growth/weight gain in spite of a good appetite, frequent greasy, bulky stools difficulty in bowel movements |
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Term
|
Definition
| Sweat Chloride Test; CFTR mutational analysis; Nasal Potential Difference (NPD) |
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Term
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Definition
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Term
|
Definition
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|
Term
| Nasal Potential Difference |
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Definition
| Assessment of voltage across nasal epithelium, correlates with the transport of sodium and chloride across cell membranes |
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Term
| associated with increased pulmonary inflammation, has a negative effect on lung function; mucoid strains --> alginate production |
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Definition
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|
Term
| 17 closely related highly virulent bacterial species; easily become resistant to antibiotics during txt |
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Definition
| Burkholderia Cepacia Complex |
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|
Term
| chronic infection associated with the development of an immune response against the organism predicts more exacerbations, but not more progression in decline of lung function |
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Definition
| Stenotrophomonas Maltophilia |
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Term
| lung disease resulting from hypersensitivity to aspergillus species characterized by: impaired mucociliary clearance, mucoid impaction and airway obstruction, pulmonary infiltrates |
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Definition
| Allergic Bronchopulmonary Aspergillosis (ABPA) |
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|
Term
| Step 1 of managing Cystic Fibrosis |
|
Definition
| Open the Airway (Beta 2 agonists, anticholinergics) |
|
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Term
|
Definition
| Hydrate Secretions (7% and 3% hypertonic saline for inhalation) |
|
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Term
|
Definition
| Thin Secretions (Pulmozyme (rhDNAse/Dornase alpha) |
|
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Term
|
Definition
| Mobilize Secretions- airway clearance |
|
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Term
|
Definition
| Reduce Inflammation (inhaled steroids, anti-inflammatory agents) |
|
|
Term
|
Definition
| Chronic Suppressive Therapy (inhaled abx, oral abx, tobramycin...) |
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Term
| Multisystem Organ Involvement |
|
Definition
| pancreatic insufficiency and malnutrition, CF related diabetes mellitus, hepatobiliary, GI conditions, bone and joint disease, mental disorder |
|
|
Term
| pancreatic insufficiency and malnutrition |
|
Definition
| fecal fat excretion, weight loss/poor weight gain, fat-soluble vitamin deficiency, flatus, abdominal discomfort |
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Term
|
Definition
| Fibrosis, cholestasis, cirrhosis; portal hypertension; liver failure |
|
|
Term
| Gastrointestinal Conditions |
|
Definition
| Distal intestinal obstruction syndrome; Epigastric pain, heartburn, dyspepsia |
|
|
Term
|
Definition
| Low bone mineral density, Episodic arthritis, Hypertrophic pulmonary osteopathy |
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