Term
| Signs of Cystic Fibrosis include: |
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Definition
- Lung infections (pneumonia, bronchitis)
- Decreased growth and weight gain, normal appetite
- Coughing
- Wheezing
- Shortness of Breath |
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Term
| CF is a common genetic disease within the __ population of the United States |
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Definition
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Term
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Definition
- Dornase Alfa inhalation
- Acetylcysteine inhalation
- Azithromycin
- Pancrelipase |
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Term
| Dornase Alfa inhalation for CF helps to __ __ and reduce the chance of __ __. It is a ___. |
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Definition
- improve breathing
- reduce changes of lung infections
- DNase |
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Term
| Acetylcysteine inhalation is used for CF b/c it __ __ so it can be __ __. |
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Definition
| - dissolves mucus so it can be coughed up |
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Term
| Azithromycin is an ___ used in CF to treat certain infections |
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Definition
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Term
| Pancrelipase is an __ that helps __ __ for pts with CF or digestive disorders. |
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Definition
| - enzyme that helps digest food |
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Term
| Cystic Fibrosis affects what systems: |
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Definition
- Lungs
- Sinuses
- GI
- Liver
- Pancreas
- Endocrine system
- Reproductive system
- Secretory glands |
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Term
| With cystic fibrosis there is secretory gland dysfunction. There is defective __ and __ secretion, impaired __, and accumulation of __ and __. |
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Definition
- defective chloride and fluid secretion
- impaired flushing
- accumulation of mucous and obstruction |
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Term
| __ insufficiency occurs in 85-90% of CF patients resulting in malabsorption of fats, protein, and other nutrients. |
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Definition
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Term
| Dx tests for pancreatic insufficiency with CF: |
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Definition
- 72 hour fecal fat
- fecal elastase-1 |
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Term
| Tmt of pancreatic insufficiency: |
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Definition
- pancreatic enzyme supplementation
- improved release with H2 blocker or PPI
- Proprietary enzymes better than generic
- Taurine supplementation |
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Term
| Complication of pancreatic enzyme supplementation with CF: |
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Definition
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Term
| The sweat test measures the amount of __ and __that is excreted in sweat. __ reliable positive results on __ separate days is diagnostic for __ ___. Clinical presentation, family history, and patient age must be considered in the results. |
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Definition
- sodium and chloride
- Two
- two separate days
- Cystic Fibrosis |
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Term
| Most common life-threatening inherited disease: |
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Definition
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Term
| Is CF more common in males or females? |
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Definition
| equal b/w males and females |
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Term
| CF is caused by a single gene defect in chromosome __ on the __ __ __ __ gene. |
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Definition
- chromosome 7
- Cystic Fibrosis Transmembrane Regulator (CFTR) |
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Term
| CF mutation is on the __ __ __ __ gene on chromosome __. It is usually a ___defect. |
|
Definition
- cystic fibrosis transmembrane regulator (CFTR)
- chromosome 7
- deltaF508 |
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Term
| The Cystic Fibrosis Transmembrane Regulator (CFTR) conducts __ across cell membranes, __ regulates transepithelial __ transport, and regulates __ __ __ and __ channels. |
|
Definition
- conducts chloride across cell membranes
- downregulates transepithelial sodium transport
- regulates calcium-activated chloride and potassium channels |
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Term
| CFTR mutations affect epithelial cells, thus affecting: |
|
Definition
- Airways (lungs, sinuses)
- Pancreas (endocrine, exocrine)
- GI tract (liver, biliary system, intestines)
- Reproductive organs
- Skin |
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Term
| A __ base pair __ called __ is the most common mutation in cystic fibrosis this results in the deletion of a single __ __, __ at position 508. |
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Definition
- 3 base pair deletion called deltaF508
- amino acid, Phe |
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Term
| Having one copy of the deltaF508 mutation reduces water loss during __, greatly increasing the chance of survival |
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Definition
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Term
| CFTR mutation --> abnormal airway surface microenvironment --> thickened mucus, chronic infection, chronic inflammation --> damages airway ---> bronchiectasis, respiratory failure and often death |
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Definition
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Term
| With CF, the pancreas has diminished exocrine function resulting in __ and __. Treat this with __/__ and __. |
|
Definition
- malabsorption
- pancreatitis
- Creon/Pancrease
- ADEKS |
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Term
| The pancreas may hve poor endocrine function as well with CF leading to __ __. To screen for this do an __ __ __ __. Treat with __ and possibly oral hypoglycemics. |
|
Definition
- Diabetes Mellitus
- oral glucose tolerance test
- insulin |
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Term
| With CF, the intestins have __ and __ resulting in __, __, __, and __. Treat with __ __. |
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Definition
- malabsorption and sludging
- malnutrition, steatorrhea, constipation, and obstruction
- enzyme replacement |
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Term
| With CF pts, the liver has __ __ __ reulting in __ __ and ___. Treat this with an early ___. |
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Definition
- inadequate bile secretion
- biliary colic and cirrhosis
- early cholecystectomy |
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Term
| With CF pts, their lungs have poor __ __ of __ leading to __, __, and __. |
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Definition
- ciliary clearing of secretions
- infections, inflammation, and scarring |
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Term
| Lungs of CF pts are __, treat this problem with ___. |
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Definition
- hyperreactive
- bronchodilators |
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Term
| With CF, there is increased sputum viscosity, treat this with __ __. |
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Definition
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Term
| With lungs there is imparied ciliary function and reduced clearance of secretions leading to infections, inflammation, and and scarring Treat this with __ ___ |
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Definition
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Term
| common offending agents causing CF exacerbation: |
|
Definition
- Pseudomonas aeruginosa
- Staphylococcus aureus |
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Term
| Tmt for CF exacerbations: |
|
Definition
- Empiric IV tmt: Aminoglycoside, Antipseudomonal beta-lactam
- Inhaled antibiotics: Tobi Neb, Colistin, Colistimethate, Polymyxin B |
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Term
| aminoglycoside side effects: |
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Definition
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Term
| Infants can easily be dx with CF via a blood test. Elevated levels of __ indicate CF. Screening programs identify 10% of cases at birth. Screening is possible but not routine. |
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Definition
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