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Pulmonary Cystic Fibrosis Exam 1
Cystic Fibrosis Pulm Exam 1
35
Anatomy
Graduate
11/15/2011

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Term
Signs of Cystic Fibrosis include:
Definition
- Lung infections (pneumonia, bronchitis)
- Decreased growth and weight gain, normal appetite
- Coughing
- Wheezing
- Shortness of Breath
Term
CF is a common genetic disease within the __ population of the United States
Definition
Caucasian
Term
Therapies for CF (4):
Definition
- Dornase Alfa inhalation
- Acetylcysteine inhalation
- Azithromycin
- Pancrelipase
Term
Dornase Alfa inhalation for CF helps to __ __ and reduce the chance of __ __. It is a ___.
Definition
- improve breathing
- reduce changes of lung infections
- DNase
Term
Acetylcysteine inhalation is used for CF b/c it __ __ so it can be __ __.
Definition
- dissolves mucus so it can be coughed up
Term
Azithromycin is an ___ used in CF to treat certain infections
Definition
antibiotic
Term
Pancrelipase is an __ that helps __ __ for pts with CF or digestive disorders.
Definition
- enzyme that helps digest food
Term
Cystic Fibrosis affects what systems:
Definition
- Lungs
- Sinuses
- GI
- Liver
- Pancreas
- Endocrine system
- Reproductive system
- Secretory glands
Term
With cystic fibrosis there is secretory gland dysfunction. There is defective __ and __ secretion, impaired __, and accumulation of __ and __.
Definition
- defective chloride and fluid secretion
- impaired flushing
- accumulation of mucous and obstruction
Term
__ insufficiency occurs in 85-90% of CF patients resulting in malabsorption of fats, protein, and other nutrients.
Definition
Pancreatic insufficiency
Term
Dx tests for pancreatic insufficiency with CF:
Definition
- 72 hour fecal fat
- fecal elastase-1
Term
Tmt of pancreatic insufficiency:
Definition
- pancreatic enzyme supplementation
- improved release with H2 blocker or PPI
- Proprietary enzymes better than generic
- Taurine supplementation
Term
Complication of pancreatic enzyme supplementation with CF:
Definition
- fibrosing colonopathy
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.
Definition
- sodium and chloride
- Two
- two separate days
- Cystic Fibrosis
Term
Most common life-threatening inherited disease:
Definition
Cystic Fibrosis
Term
Is CF more common in males or females?
Definition
equal b/w males and females
Term
CF is caused by a single gene defect in chromosome __ on the __ __ __ __ gene.
Definition
- chromosome 7
- Cystic Fibrosis Transmembrane Regulator (CFTR)
Term
CF mutation is on the __ __ __ __ gene on chromosome __. It is usually a ___defect.
Definition
- cystic fibrosis transmembrane regulator (CFTR)
- chromosome 7
- deltaF508
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
Term
CFTR mutations affect epithelial cells, thus affecting:
Definition
- Airways (lungs, sinuses)
- Pancreas (endocrine, exocrine)
- GI tract (liver, biliary system, intestines)
- Reproductive organs
- Skin
Term
A __ base pair __ called __ is the most common mutation in cystic fibrosis this results in the deletion of a single __ __, __ at position 508.
Definition
- 3 base pair deletion called deltaF508
- amino acid, Phe
Term
Having one copy of the deltaF508 mutation reduces water loss during __, greatly increasing the chance of survival
Definition
cholera
Term
CFTR mutation --> abnormal airway surface microenvironment --> thickened mucus, chronic infection, chronic inflammation --> damages airway ---> bronchiectasis, respiratory failure and often death
Definition
Term
With CF, the pancreas has diminished exocrine function resulting in __ and __. Treat this with __/__ and __.
Definition
- malabsorption
- pancreatitis
- Creon/Pancrease
- ADEKS
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
Term
With CF, the intestins have __ and __ resulting in __, __, __, and __. Treat with __ __.
Definition
- malabsorption and sludging
- malnutrition, steatorrhea, constipation, and obstruction
- enzyme replacement
Term
With CF pts, the liver has __ __ __ reulting in __ __ and ___. Treat this with an early ___.
Definition
- inadequate bile secretion
- biliary colic and cirrhosis
- early cholecystectomy
Term
With CF pts, their lungs have poor __ __ of __ leading to __, __, and __.
Definition
- ciliary clearing of secretions
- infections, inflammation, and scarring
Term
Lungs of CF pts are __, treat this problem with ___.
Definition
- hyperreactive
- bronchodilators
Term
With CF, there is increased sputum viscosity, treat this with __ __.
Definition
dornase alfa
Term
With lungs there is imparied ciliary function and reduced clearance of secretions leading to infections, inflammation, and and scarring Treat this with __ ___
Definition
chest PT
Term
common offending agents causing CF exacerbation:
Definition
- Pseudomonas aeruginosa
- Staphylococcus aureus
Term
Tmt for CF exacerbations:
Definition
- Empiric IV tmt: Aminoglycoside, Antipseudomonal beta-lactam
- Inhaled antibiotics: Tobi Neb, Colistin, Colistimethate, Polymyxin B
Term
aminoglycoside side effects:
Definition
nephrotoxicity
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.
Definition
- trypsinogen
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