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47 Cards in this Set
- Front
- Back
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True or false- CF is an autosomal dominant disorder and is the most common disorder of this time among caucasians?
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False: autosomal RECESSIVE
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When is CF most typically diagnosed?
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At or shortly after birth.
Note: if diagnosed later in adolescence its considered "milder" phenotype |
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CF is caused by mutations on a single gene on chromosome ___ and is known as _._._._
- This specifically function as regulated ____ channel at the epithelial surface |
Chromosome 7
- CFTR- Cystic Fibrosis Transmembrane Conductance Regulator - Chloride channel |
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True or false: there are 1500 mutations id'd in CFTR gene but not all of them = CF?
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True
Note: there are about 1200 associated with clinical disease |
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Classes of Genetic Mutations: 1-5-
which is the most severe? which is the most common? which is the most mild? |
1 is the most severe
2 is the most common --> delta F508 mutation 5 is the most mild |
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True or false: the delta F508 is the only mutation associated?
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False: F508 does correlate but other factors take part like TGF-beta 1 and MBL (mannose binding lectin)
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A major part of diagnosis involves increased ____ content in sweat gland secretions
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Sodium
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What Sx would a patient present with CF?
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Respiratory Sx
Failure to thrive steatorrhea meconium ileus |
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Diagnosis:
- there MUST be clinical Sx consistent with CF in at least ___ organ system(s) AND evidence of ____ dysfunction - Gold standard for Dx is ____ shown by >____mmol/L on ____ occasions? |
- One organ system; CFTR
-Sweat chloride test- >60 mmol/L on 2 occasions |
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In the sweat test, what is applied to the skin?
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- Pilocarpine, a cholinergic agent that causes sweating
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>85% of mortality from CF is due to ____ Dz indicated by what type of Sx?
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- pulmonary
-Sx = lung destruction due to dehydrated & thickened secretions, infection, exaggerated inflammatory response - spontaneous pneumothorax, hemoptysis, respiratory failure |
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Life expectancy today is __-__ years
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40-50 years
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Pancreatic/GI Tract Involvement:
1. Intestinal- obstruction due to ____ deficiencies. - Sx include |
1. enzyme
-GERD, meconium ileus (destruction of small intestine due to electrolyte transportation deficits), distal intestinal obstruction syndrome |
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Pancreatic/GI Tract Involvement:
2. Pancreatic- - (Most/Least) common GI complication - ____ malabsorption leads to maldigestion of nutrients |
- most common (99% with delta F508 mutation have it)
-fat malabsorption (leads to decreased enzymes) |
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Pancreatic/GI Tract Involvement:
3. Hepatobiliary - usually diagnosed by age ____ - can lead to ____-HTN, ____varices, hyper____ism |
- 20
-portal HTN; esophageal varices, hypersplenism |
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Pancreatic/GI Tract Involvement:
Medications and Tx - _____ ____ supplementation - ____ to ____ lipase units per 1 gm ingested fat |
- pancreatic enzyme
-500-4000 lipase units |
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CREON
- releases enzymes in the ____ at a pH >5.5 - taken how? - can they substitute? - Dosing per CF foundation based on ___ ___/kg per meal- max of ____/kg/day |
- duodenum
- swallow whole with meals or sprinkle contents on nonalkaline food (applesauce, jelly) - DO NOT SUBSTITUTE - lipase units; max of 10,000 lipase units per day |
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Which of the following pancreatic enzymes are NOT on the market today?
A. ZENPEP B. Pancreaze C. Pancrease D. Creon |
C. Pancrease
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Vitamins Suplementation
- all patients should be taking ____ - additional ___ soluble vitamins - Vitamin ___ 5 mg PO 2x/wk if ____ ___ prolonged |
- multivitamin
- fat soluble vitamins - Vit K, if PT time prolonged |
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Surgery options
- for meconium ileus- usually don't respond to ____ - _____ transplant |
- enemas
- liver txp |
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Respiratory Tract Involvement:
- Lab tests- often have decrease in ___ and ___ and increased ___ volume |
- decreased FEV, FEV1; increased tidal volume
(due to viscous secretions) |
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Respiratory Tract Involvement:
- medications: - ____/____ (dornase alpha) - MOA? - Improved ___ - __ mg inhaled via ____ daily - Recommendation: grade ___ for children > ___ with mod-severe. Grade __ for mild |
- DNase I/ Pulmozyme
- MOA: endonuclease; cleaves long strands of denatured DNA released by degenerating neutrophils- liquefied CF sputum - FEV1 - 2.5 mg; nebulizer -A; >6 yrs; B for mild |
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Respiratory Tract Involvement:
- Medcations: Hypertonic Saline MOA: liquefies mucus by pulling ___ from airway to surface layer - dose: ___ mL of ___% saline inhaled via nebulizer ____ daily -used in combo? |
- H20
- 4 mL; 7%; twice daily - yes, combo with DNase I in pts >6 yrs (alone for 6 mo - 6 yrs) |
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Respiratory Tract Involvement:
Medications- Albuterol - MOA? - Improved ___ - Grade ___ recommendation |
- Beta-2 adrenergic receptor agonist
- FEV1 -B |
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Respiratory Tract Involvement:
Medications: - True or false: the CF foundation recommends the chronic use of inhaled steroids in all patients |
- False!
Note: grade B recommendation use high dose ibuprofen in pts >6 yrs with FEV1 >60% |
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True or false: Inhaled anticholinergics, cromolyn, leukotriene modifiers, N-acetylcysteine do not have sufficient evidence of efficacy?
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True
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Chest Physiotherapy- to promote ___ and includes ___ and ____ techniques
Supplemental ____ is reserved for pts with chronic hypoxemia |
- secretion clearance; breathing and coughing
- oxygen |
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Respiratory Tract Involvement:
Role of Vaccinations: - yearly ___ in pts >6mo - _____ vaccine due to favorable risk-benefit profile |
- influenza
- pneumococcal |
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Potential Pathogens involved in airway:
- name a few involved - which one is the WORST to have? |
- S. aureus
- H. influenza - P. aeruginosa - Burkholderia capecia - the worst is burkholderia! |
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Data shows greater improvement of lung function when ______ are added to bronchodilators
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- antibiotics
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When should you give PO antibiotics?
When should you give IV antibiotics? |
- mild and pathogens are sensitive
- severe and bacteria are resistant |
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What type of antibiotics can you give PO? For how long?
- aerosolized ____ or ____ for P. aeruginosa |
- Bactrim, dicloxacillin, Amox-Clav, Cephalexin, macrolide
"BD-MAC".. BD is a MAC - for 14-21 days - Tobi or colistin for P. aerug |
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Clearance of hydrophillic abx (Aminoglycosides, PCNs, cephs) are INCREASED/DECREASED in CF patients?
Do you need LARGER/SMALLER more frequent dosing? |
- increased
- larger |
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Aminoglycosides:
- ____ has best activity against Pseudomonas - start dose __-___% higher than normal - ____ daily dosing |
- Tobramycin
-30-35% - once daily |
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Vanco: do you have to increase the dose compared to a non-CF patient?
Cipro: - Adults need (Higher/Lower/Same) dose as non-CF patient - Children need (Higher/Lower/Same) dose as non-CF patient |
NO!
- Adults - same - Children need HIGHER dose (40 mg/kg/day) |
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How often should sputum cultures be obtained?
- Lungs appear ______ on radiograph - _.____ and _.____ persist for years once est in airways |
- monthly
- hyperinflated - P. aeruginosa, B. cepacia |
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True for False:
1. Routine PO broad spectrum ABX is recommended 2. Prophylactic anti-staph abx is not supported 3. Chronic PO meds directed towards Pseudomonas supported |
1. False
2. True 3. False- NEBULIZED not PO!!!! |
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Many CF centers treat recently detected Pseudomonas with ____ or _____ for 28 days
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- Tobramycin; quinolone for 28days
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Tobi:
-Route -class - dose: ___ mg in 5 mL sterile water via jet _____ BID x ___days on ___ days off - Improves ____ |
- inhaled
- Aminoglycocide - 300 mg; nebulizer; 28 on, 28 off - FEV1 |
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Colistin
- route: -class - benefit over TOBI? |
- nebulized
- antipseudomonal polypeptide - no shown benefit |
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Azithromycin
- Class - MOA - used in what type of infections? - ___ - ___ mg __ times weekly or ___ mg daily - Improves ____ and reduces ___ |
- macrolide
- antimicrobial, anti-inflammatory activity - Pseudomonas- Grade B - 250-500 3x/week; 250 mg daily - FEV1; exacerbations |
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FEV1
- Normal: - Mildly impaired - Moderately impaired - Severely impaired |
- Normal >90%
- Mild = 70-89% - Mod = 40-69% - Severe = <40% |
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Infection with ______ associated with accelerated decline in pulm function and its presence is a contraindication to ____ ____
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- Burkholderia; lung transplant
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Drugs used against Burkholderia
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- Bactrim, Doxyclycline, Ceftazidime, meropenem or a combo of 2 or more
Burkhold is a "Bad Dog Crap Master" |
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About ___% of males are sterile
Females experience ____ irregularities |
- 95%
- mentrual (abnormal cervical mucus production) |
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Hematological abnormalities due to distrubances in ___ regulation and ___ availability
- DECREASED/INCREASED HCT and serum ferritin? - DECREASED/INCREASED carboxyhemoglobin |
- EPO; Iron
- decreased - increased |
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Gene therapy:
- correction of _._._._. through homologous replacement - Splicesome-mediated RNA transsplicing to correct ___-____ - Inserting normal _._._._. copies to airway cells |
- CFTR
- delta F508 - CFTR |
