Shared Flashcard Set

Details

Cystic Fibrosis
CF
70
Anatomy
4th Grade
02/02/2013

Additional Anatomy Flashcards

 


 

Cards

Term
Newborn screening: only identifies infants at risk for CF; high value necessitates genetic testing
Definition
Immunoreactive Trypsinogen (IRT)
Term
80-90% of patients with this have CF
Definition
Meconium Ileus
Term
~20% of patients with this present with Meconium Ileus
Definition
Cystic Fibrosis
Term
diagnostic gold standard; 2nd confirmatory test required
Definition
Sweat Chloride test
Term
Cystic Fibrosis: Genetics
Definition
Autosomal recessive disease
Term
most severe form of cystic fibrosis
Definition
Class 1
Term
F508
Definition
Class 2 mutation
Term
usually pancreatic sufficient
Definition
Class 4
Term
most common genetic mutation in CF
Definition
F508
Term
normally regulates sodium and chloride transport across the cell membrane
Definition
CFTR
Term
impaired chloride transport --> dehydrated cell surface liquid --> mucus plugging --> organ dysfunction
Definition
Defective CFTR
Term
thickened mucus --> decreased mucus clearance --> bacterial overgrowth and colonization ---> plugging, increased cough, SOB --> decreased O2, salts, lung scarring--> eventual mortality
Definition
Lung Disease (CF)
Term
Other lung complications in CF
Definition
Pneumothorax, Pleurisy
Term
Thick secretions in bile ducts and pancreas
Definition
GI Disease
Term
In utero blockage of vas deferens... thickened cervical mucus or malnutrtion
Definition
Reproductive Disease
Term
Thick secretions in bile ducts and pancreas
Definition
GI Disease
Term
Recommend genetic counseling
Definition
Reproductive Disease
Term
preferred CF-specific vitamin
Definition
AquADEK
Term
~50-75% of CF patients
Definition
Bone Disease
Term
Consider if T/Z score is equal to or less than -2.0
Definition
bisphosphonates
Term
Zenpep, Creon, Pancreaze, ultresa, Viokase (DO NOT SUBSTITUTE)
Definition
Pancreatic Enzyme Replacement Therapy (PERT)
Term
Order of Respiratory Meds: #1
Definition
Albuterol (MDI or neb)
Term
Order of Respiratory Meds: #2
Definition
Hypertonic saline, then Pulmozyme (Vest simultaneously)
Term
Order of Respiratory Meds: #3
Definition
inhaled antibiotic
Term
Order of Respiratory Meds: #4
Definition
steroid neb or inhaler
Term
t-piece Nebulizer
Definition
drugs: as recommended by your physician
Term
sidestream nebulizer (use with 50 psi compressor, i.e., MobilAire)
Definition
Drugs: Pulmozyme (dornase alpha); Albuterol (salbutamol)
Term
Pari LC Plus (use with 25 psi compressor, i.e., PulmoAide)
Definition
Drugs: Colmycin (steril colistimethate sodium, USP); Albuterol; Pulmozyme; Pulmicort (budesonide)
Term
Pari LC Star (use with 25 psi compressor, i.e. PulmoAide)
Definition
Drugs: Colymycin, Albuterol, Pulmozyme, Pulmicort
Term
Altera with eFlow technology
Definition
Drugs: Cayston
Term
a substitute for the Pari-LC Plus nebulizer that allows for rapid administration of meds
Definition
E-Rapid
Term
increases hydration of airway surface liquid; not FDA approved for CF
Definition
Hyptertonic Saline
Term
Mucolytic; breaks up long DNA strands, decreases mucus viscosity
Definition
Pulmozyme (dornase alfa)
Term
Pulmozyme
Definition
may cause increased cough, wheeze, hoarseness, skin rash, fever, HA, malaise
Term
CF Anti-inflammatory therapies
Definition
Ibuprofen and Azithromycin
Term
studies show benefit for nutritional status and fewer pulmonary infections; monitor for atypical mycobacterium
Definition
Azithromycin
Term
yearly at minimum
Definition
cultures
Term
oral antipseudomonal antibiotics
Definition
Ciprofloxacin
Term
inhaled antipseudomonal antibiotics
Definition
Tobi (tobramycin); Cayston (aztreonam) Colistimethate
Term
may cause coughing, wheeze, sore throat, hoarseness; little-to-no systemic absorption
Definition
Tobraymycin inhalation
Term
SEs: cough, bronchospasm, hoarseness; supplied by only a few specialty pharmacies in the US
Definition
Cayston (aztreonam lysine)
Term
IV Antipseudomonal Antibiotics
Definition
penicillins, cephalosporins, carbapenems, tobramycin, ciprofloxacin, aztreonam
Term
Piperacillin-Tazobactam (Zosyn)
Definition
Penicillin Antipseudomonal IV antibiotic
Term
Ceftazidime, Cefepime
Definition
Cephalosporin Antipseudomonal IV antibiotics
Term
meropenem, doripenem
Definition
Carbapenem Antipseudomonal IV antibiotics
Term
first choice among carbapenems for Antipseudomonal
Definition
Meropenem (merem)
Term
30 mg/kg/day IV required for CF children due to increased clearance
Definition
Ciprofloxacin
Term
oral antibiotics for MRSA
Definition
high dose septra, clindamycin, minocycline, doxycycline, linezolid
Term
Bactrim is the DOC; Cefepime or Timentin; Respiratory quinolones, minocycline are alternatives; resistant to aminoglycosides and carbapenems
Definition
Stenotrophomonas maltophilia
Term
Bactrim is DOC; Respiratory quinolones, minocycline are alternatives; may be resistant to aminoglycosides, piperacillin, ceftazidime
Definition
Burkholderia cepacia
Term
Treats and prevents CF liver disease
Definition
Ursodiol
Term
Ursodiol side effects
Definition
constipation, N/V, diarrhea, backache, dizziness, rash ,cough, pharyngitis/bronchitis
Term
MOA: decreased biliary cholesterol concentration --> breakdown of gallstones; increases bile flow, protects liver by decreasing toxicity of bile acids
Definition
Ursodiol
Term
Usodiol monitoring
Definition
LFTS; dose titration dependent upon LFTs
Term
Appetite stimulant
Definition
Periactin (cyproheptadine)
Term
Periactin side effects
Definition
drowsiness, diarrhea, N/V
Term
MOA: histamine, serotonin, and acetylcholine antagonist; likely alters serotonin activity in the appetite center of the hypothalamus
Definition
Periactin
Term
Dual mechanism of action in CF; improved enzyme efficacy: decreased bicarb production by the pancreas inhibitors release of enzymes from microspheres
Definition
PPIS and H2 blockers
Term
Pancreatic duct blockage and scarring, loss of beta cells prevents insulin secretion; insulin resistance may develop over time
Definition
CF-Related Diabetes (CFRD)
Term
Not appropriate in CF
Definition
oral anti-diabetic agents
Term
Metformin in CF
Definition
CI in hypoxia due to risk of lactic acidosis; GI side effects
Term
Thiazolidinediones in CF
Definition
risk of hepatotoxicity
Term
Sulfonylureas in CF
Definition
may bind and inhibit the CFTR
Term
Primary treatment of CFRD
Definition
insulin therapy; results in increased BMI, improved FEV1, improved survival
Term
Indicated for CF patients > 6 years old with G441D mutation
Definition
Kalydeco (Ivacaftor)
Term
Kalydeco contraindications
Definition
strong CYP3A4 inducers
Term
Kalydeco side effects
Definition
URI, stomach ache, HA, rash, diarrhea, dizziness
Term
Kalydeco
Definition
CFTR potentiator; facilitates opening of chloride channel
Term
Cost $25,000/month; additional studies underway for other gating mutations and for use in combination with VX809
Definition
Kalydeco
Term
CFTR Corrector; facilitates movement of the CFTR protein to the cell surface
Definition
VX809
Supporting users have an ad free experience!