Term
| How many mutations cause CF, and which is most common? |
|
Definition
Over 2000 mutations identified
Delta 508 is most common |
|
|
Term
| Chromosome / Proetien affected in CF? |
|
Definition
Chromosome 7
CFTR Transport Protein |
|
|
Term
|
Definition
1. Pulmonary/pancreatic symptoms
2. Sweat Chloride Test
3.Genotyping
4. Newborn screening |
|
|
Term
| Which diagnosis method is the gold standard? |
|
Definition
|
|
Term
| What constitues a polsitive Sweat Chloride test? |
|
Definition
|
|
Term
| How many mutations must be present for a genotyping diagnoses |
|
Definition
|
|
Term
| What is one method newborns are screened? |
|
Definition
Immunoreactive trypsinogen (IRT)
+ Sweat test |
|
|
Term
| What are respiratory clinical characteristics of CF? |
|
Definition
- Recurrent infections
- Fast decline in pulm. fuct.
- Nasal polyps
- Chronic cough
- Obstructive lung disease
- Pulm. HTN
|
|
|
Term
What are GI clinical characteristics of CF? |
|
Definition
- Pancreatic insuf.
- weight loss
- DIOS
- Rectal prolapse
- GE reflux
|
|
|
Term
| What are genitourinary/ednocrine clinical characteristic of CF? |
|
Definition
- Diabetes
- Infertility M>F
- Menstrual irregularities
- Delayed puberty
|
|
|
Term
What are other clinical characteristic of CF? |
|
Definition
1. Cirrhosis
2. Cholethiasis
3. Anemia
4. Sweat gland abnormalities |
|
|
Term
| T/F CF affects the absorption of fat soluble vitamins |
|
Definition
True
b/c of pancreatic insufficency |
|
|
Term
| Whatg respiratory agents are use to treat CF? |
|
Definition
1. anitbiotics
2. bronchodialotors
3. mucolytics
4. antiinflammatory |
|
|
Term
| What is the primary way to identify an exacberation of CF? |
|
Definition
| Change in Sputum appearance |
|
|
Term
| The common bacterial pathogens of CF and how are they treated? |
|
Definition
P. Aeruginosa = AGLY + Beta lac.
B. cepacia = AGLY + Beta lac.
S. Aureus = Vanco, Linezolid, or Tigecycline
S. maltophila = tetracyclines or TMP/SMZ |
|
|
Term
| How often are CF patients seen, and what comprises empiric therapy? |
|
Definition
Every 3 months
Sputum culture
|
|
|
Term
| T/F CF affects all parts of ADME? |
|
Definition
| True; notable faster metabolization |
|
|
Term
| When prescribing a AGLY what is the therapuetic goal time or concentration for ACUTE infection |
|
Definition
|
|
Term
| T/F At high doses AGLY switch from one compartment to two compartment drug model |
|
Definition
|
|
Term
| Typical AGLY dosing for CF related P.aeruginosa or B. cepacia ACUTE infection? |
|
Definition
Torbamycin 7-15mg/kg/24hr IV (can be dosed BID, TID, QD)
Amikacrin 30-35mg/kg/24hr IV |
|
|
Term
| What are goal concentrations for tobramycin and amikacinin ACUTE CF infection? |
|
Definition
Tobramycin: Peak = 20-40mg/L; trough <1mg/L
Amikacin: Peak = 80-120mg/L; trough <10mg/L |
|
|
Term
| Typical Beta Lac dosing for CF related P.aeruginosa or B. cepacia ACUTE infection? |
|
Definition
Piperacillin: 350-600 q4-6hrs; max = 24g/day
Ticarcillin: 300-600 q4-6hrs max=24g/day
Ceftazidime: 150 q8h; max 6g/day
Cefepime 150 q8h; max 6g/day |
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Term
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|
Definition
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