Term
| Name 4 systems that are affected in CF: |
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Definition
| pulmonary, digestive, swet glands, reproductive sys. |
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Term
| CF is caused by mutations in: |
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Definition
| single large gene on C'some 7 |
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Term
| the mutated gene in CF codes for: |
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Definition
| complex chloride channel in exocrine glands. |
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Term
|
Definition
| Cystic Fibrosis Transmembrane Regulator Protein: the chlorise channel involved in CF |
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Term
| what accounts for the differing severities of CF patients? |
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Definition
| CFTR gene is large and CF can result from any number of mutations in the 250 kilobases |
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Term
| more than ____ distinct sequence changes in the CFTR gene are assoc. with clinical disease. |
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Definition
|
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Term
| what is the genotype of the disease? |
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Definition
|
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Term
| deranged chloride transport leads to: |
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Definition
| thick, viscous secretions from all exocrine glands. |
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Term
| pulmonary pathophysiology: |
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Definition
| thich mucus secretions in the airways prevent cilia from transporting mucus. leads to bronchiectasis |
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Term
|
Definition
| destruction and widening of the large airways due to the mucus production |
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Term
| 5 pulmonary manifestations; |
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Definition
1. productive cough
2. chronic bronchitis
3. tachypnea
4. dyspnea
5. weight loss |
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|
Term
| digital manifestation associated with all obstructive pulmonary disorders: |
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Definition
|
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Term
| CF pts are colonized with 3 major pathogens that are impossible to get rid of: |
|
Definition
1. S. aureus
2. H. influenza
3. P. aureugenosa |
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Term
| what would been seen on the chest radiograph in CF? |
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Definition
| hyperinflation (as with any obstructive pulmon. path), diffuse interstitial disease, bronchiectasis, mucoid impaction |
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Term
| pulmonary function test results are similar to other obstructive airway diseases; these are: |
|
Definition
increased:TLC & FRC
decreased:VC & FEV1 |
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Term
| in CF which organ defect is reponsible for decreased fat and protein absorption |
|
Definition
| pancreatic deect in secreting pancreatic juices from the exocrine glands of the pancrease |
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Term
| in CF there is some times a rash aound the orifices of the body that is associated with: |
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Definition
|
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Term
| relate meconium ileus and CF: what are the s/s? |
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Definition
| CF babies would have very thick meconium and wouldn't be able to pass it; s/s - 1. failure to pass meconium within 48hrs 2. abdominal distension and vomiting |
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Term
| in 90-100% of patients older than 8mo with CF: |
|
Definition
| panopacification of sinuses |
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Term
| why are 95% of men with CF infertil |
|
Definition
| defects in sperm transport & vas deferens |
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Term
| female infertility in CF is 20% and is due to: |
|
Definition
| increase viscosity of vaginal secretions & ammenorrhea induced by malnutrition |
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Term
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Definition
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Term
| what percent of CF diagnoses are made after age 18? |
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Definition
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Term
| describe presentation of 18 yo CF patient that has gone unDx: |
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Definition
| gastrointestinal issues, DM & infertility |
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Term
| which test is the Gold standard fo DX of CF? |
|
Definition
| sweat chloride test >60mEq |
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Term
| genetic testing detects __% of CF csomes |
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Definition
|
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Term
| newborn screening allows detection of __% of CF newborns |
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Definition
|
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Term
| newborn screenings test for elevated blood levels of: |
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Definition
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Term
| newborn testing not valid after ____ since levels of IRT fall rapidly |
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Definition
|
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Term
| newborn screenings have a high rate of false ________ |
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Definition
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Term
| 7 points in Tx plan for CF |
|
Definition
1. Refer to specialist
2. bronchodilators
3. chest physiotherapy
4. saline nebulizers
5. anitinflammatory; ibuprofin
6. inhaled steroids
7. antibiotics |
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Term
| about __% of those who undergoe a lung xplant survive at least 1 yr |
|
Definition
|
|
Term
| avg life span for CF pts: |
|
Definition
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