Term
What are the predominant features of cystic fibrosis in the advance stages |
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Definition
| Excessive bronchial secretions, bronchial obstruction, and hyperinflation of the lungs |
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Term
The major pathologic/structural changes associated with cystic fibrosis: |
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Definition
o Excessive production and accumulation of thick, tenacious mucus in the tracheobronchial tree
o Partial bronchial obstruction (mucous plugging)
o Hyperinflation of the alveoli
o Total bronchial obstruction (mucous plugging)
o Atelectasis |
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Term
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Definition
| an autosomal recessive gene disorder caused by mutations in a pair of genes located on chromosome 7 |
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Term
There are over ____ different mutations of CF |
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Definition
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Term
The abnormal expression of CTFR results in abnormal transport of ____ and ____ across many types of epithelial surfaces, including surfaces, including those lining the bronchial airways, intestines, pancreas, liver ducts, sweat glands, and vas deferens |
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Definition
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Term
Patients with CF have trouble digesting ____ and ____ |
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Definition
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Term
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Definition
| a blockage of the intestine |
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Term
Most ___ with CF are infertile and In ____ it is uncommon to be infertile |
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Definition
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Term
| CF is a ___ gene disorder |
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Definition
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Term
Cystic Fibrosis affects ____children and adults in the U.S. |
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Definition
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Term
The predicted median life expectancy is ___ years |
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Definition
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Term
Death is usually caused by ____ |
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Definition
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Term
What is the gold standard Diagnostic test for cystic fibrosis and what does it measure? |
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Definition
| Sweat test; it measures the amount of sodium and chloride in the patient’s sweat |
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Term
In children, a sweat chloride concentration greater than __ mEq/L is considered to be a diagnostic sign of the disease… In adults it’s ___ mEq/L |
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Definition
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Term
An immunoreactive trypsinogen test (IRT) may be ordered as an initial test for |
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Definition
o Babies who are not creating enough sweat to perform a sweat test
o Infants with meconium ileus
o Children and adults with signs and symptoms associated with cystic fibrosis and pancreatic dysfunction |
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Term
During an IRT test, a blood sample is analyzed ___ for a specific protein called ____ |
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Definition
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Term
What does the stool fecal fat test measure? |
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Definition
| the amount of fat in the infants stool and the percentage of dietary fat that is not absorbed by the body |
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Term
Nasal electrical potential difference- |
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Definition
| the amount of energy required to move an electrical charge from one point to another in the nasal passages |
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Term
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Definition
| a sample of blood is given to test the DNA of the patient for the chromosome that carries cystic fibrosis |
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Term
It is estimated that genetic testing can confirm cystic fibrosis in about ___ to ___ % of the patients tested |
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Definition
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Term
The two prenatal tests used to look for CF are ____ in the first trimester or by ____in the second or third trimester |
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Definition
| chorionic villus biopsy; amniocentesis |
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Term
A spontaneous pneumothorax is greater than ___ in adults with CF. When a patient with CF has a pneumothorax, there is a ___ chance that it will recur |
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Definition
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Term
Typical findings on a chest x-ray: |
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Definition
o Translucent (dark) lung fields
o Depressed/flattened diaphragms
o Right ventricular enlargement
o Areas of atelectasis and fibrosis |
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Term
The primary goals of cystic fibrosis management are to |
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Definition
| prevent pulmonary infections, reduce the amount of thick bronchial secretions, improve airflow, and provide adequate nutrition |
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