Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

Advanced Pediatric Pharmacology Exam 1 > Cystic Fibrosis > Flashcards

Cystic Fibrosis Flashcards

1
Q

Life expectancy

A

1962: average survival age was 10 years
2011: median survival age is mid-30’s
Centers are treating patients in their 60s

2016 life expectancy is close to 40

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Clinical Course of CF (4)

A
  1. Patient specific
  2. Gene mutation dependent
  3. Patient/family compliance
  4. Gradual lung function decline
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Dx of CF (4)

A
  1. Meconium ileus
  2. Genetic testing
  3. Sweat Test: Cl- concentrations
  4. Classic Triad
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Pathology Overview (3)

A
  1. CFTR dysfunction alters ion permeability of cell membranes
  2. Inadequate secretin of fluid which alters physical chemical properties of secretions
  3. Imbalance of ions and water in intracellular areas
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Pathophysiology of Genetics (3)

A
  1. CFTR gene
  2. CFTR protein
    - -> ATP-binding cassette protein
    - -> 2 ATP hydrolysis domains
    - -> Function: cAMP-dependent and protein kinase C, Cl- channel
  3. Loss of function results in epithelial dysfunction
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Gene Mutations (5)

A
  1. Class I: defective protein production
  2. Class II: defective protein processing
  3. Class III: defective channel regulation
  4. Class IV: defective channel conductance
  5. Most common mutation is a 3-base pair deletion resulting in loss of phenylalanine at position 508
    - - changed 508 allele
    - - Earlier diagnosis, more pancreatic involvement
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Pathophysiology of the lower respiratory tract (3)

A
  1. Dehydrated mucous and reduced mucous clearance
  2. Loss of innate defense mechanisms
  3. Inflammation
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Clinical Manifestations (7)

A
  1. Decreased forced vital capacity (FVC) and forced expiratory volume in 1 sec (FEV1)
  2. Bronchiectasis, chronic hypoxia
    - increased cough
    - decreased appetite
    - decreased exercise intolerance
  3. Cough
  4. Chronic infection
  5. GERD
  6. Barrel chest
  7. Clubbing
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Pseudonomas Cascade and CF (6 steps)

A

A bacteria that is a major cause of lung infection in patients with CF

1st: Pseudonomas attrack cytokines IL-1, IL-8, LTB, TNF-alpha
2nd: Cytokines attract neutrophils
3rd: Neutrophils are unable to digest the bacteria
4th: Neutrophils die leaving proteolytic enzymes, oxidants, DNA, and actin in the airway where pseudonomas were
5th: Clogs airway and increases thickness/tenacity of mucous
6th: Bronchiectasis and Atelectesis occur; increased cough, decreased appetite and exercise intolerance

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Vicious Circle of Lung Disease (4 steps)

A

1st: CF gene mutations
2nd: CTFR protein dysfunction
3rd: Ion transport defects
4th: Altered airway secretions Infection Tissue damage Inflammation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Respiratory dysfunctions with CF (5)

A
  1. Persistent wheezing
  2. Chronic cough
  3. Thick sputum production
  4. Recurrent infections
  5. Clubbing of nail beds (Hypoxia)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Most common organisms that infect CF patients (3)

A
  1. Staphylococcus aureus (Including MRSA)
  2. Pseudomonas aeruginosa
  3. Haemophilus influenzae
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Other bacteria that infect CF (5)

A
  1. Proteus
  2. Klebsiella species
  3. Stenotrophomonas maltophilia
  4. Burkholderia cepacia
  5. Achromobacter xylosoxidans
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Fungus that infects CF

A

Aspergillus fumigatus

-Associated with allergic bronchopulmonary asp

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Pathophysiology of the Upper Respiratory Tract (3)

A
  1. Nasal Polyps
  2. Sinusitis
  3. Upper respiratory infections and colonization
    - P. aeruginosa
    - H. influenzae
    - Streptococci
    - Anaerobes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Pathophysiology of the Pancreas (6)

A
  1. 85 – 90% of CF patients will pancreatic insufficiencies and require supplementation
  2. Pancreatic Insufficiency is generally first symptom noticed
  3. Effects can be variable but are primarily due to defective Cl- HCO3- exchanger
  4. Decreased Na+ and HCO3- in the pancreatic duct
  5. Retention of enzymes
  6. Malabsorption of fat, protein, and nutrients
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Long term effects on the pancreas (6)

A
  1. Destruction of pancreatic tissue leading to…
  2. Fibrosis
  3. Fatty replacement
  4. Cyst formation
  5. Overtime may require insulin
  6. Pancreatitis
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Pathophysiology of the intestinal tract (7)

A
  1. 10 - 16% of CF cases are diagnosed with meconium ileus
  2. Dehydrated contents leads to obstruction and….
  3. GERD
  4. Distal intestinal obstruction syndrome (DIOS)
  5. Rectal prolapse
  6. Intussesception
  7. Appendiceal abscesses
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

Pathophysiology of the liver (4)

A
  1. CTFR located on the apical surfaces of the cells lining the bile ducts (interhepatic and extrahepatic)
  2. Results in bile duct obstruction
  3. Ultimately can lead to biliary cirrhosis
  4. Diagnosed with increasing liver enzymes
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Long-term effects on the liver (6)

A
  1. Cholelethiasis
  2. Cirrhosis
  3. Portal hypertension
  4. Esophageal varices
  5. Cholestasis
  6. Hypersplenism
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

Pathophysiology on male reproductive system (3)

A
  1. 95% are sterile
  2. Delayed puberty
  3. Aspermia results from obstruction of the epididymis, vas deferens, and seminal vesicles
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Pathophysiology on female reproductive system (4)

A
  1. 20% are infertile
  2. Delayed puberty
  3. Reduced fertility from abnormal cervical mucous
  4. Menstrual irregularities can occur
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Pathophysiology on sweat glands (3)

A
  1. High Na+ and Cl- concentrations
  2. Loss of ability to reabsorb sodium (Na concentrations > 100 mM)
  3. Typically only a problem in hot climates
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Pathophysiology on bones and joints (3)

A
  1. Arthritis
  2. Osteopenia/osteoporosis
  3. Vitamin D deficient
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Hematologic Pathophysiology (2)

A
  1. Anemia

2. Decreased erythropoetin

26
Q

Keys to success with CF treatment (4)

A
  1. Compliance = Big Issue
    - It is easy to slack when patients are healthy
  2. Therapies can be difficult to adhere to
  3. Teenagers often struggle and do not take their medications
  4. Nutrition is a major component
27
Q

Unique pharmacokinetic characteristics with CF (3)

A
  1. Hyper-metabolic
  2. Increased Vd for water soluble drugs
    - Penicillins, theophylline, aminoglycosides
    - Change in body composition
  3. Enhanced total body clearance
28
Q

Mechanical devices for pulmonary physiotherapy (2)

A
  1. Positive Expiratory Pressure (PEP) Device

2. High frequency Chest Wall Oscillation (HFCWO)

29
Q

Pulmonary physiotherapy Physical therapy (4)

A
  1. Postural draining
  2. Percussion
  3. Vibration
  4. coughing
30
Q

Inhaled Agents: Mucolytics (2)

A
  1. Recombinant Human DNAase

2. Dornase alfa (Pulmozyme)

31
Q

Inhaled agents for airway obstruction

A

B2-Agonists

32
Q

Inhaled agents for airway clearance

A

Hypertonic saline (7% or 3%)

33
Q

Inhaled antibiotics (3)

A
  1. Tobramycin (TOBI)
  2. Aztreonam (Cayston)
  3. Colistimethate (Colistin)
34
Q

Oral Anti-Inflammatory Agents (4)

A
  1. If FEV1 > 60% it is recommended for patients 6+ to slow the loss of lung function decline (Fair evidence, level B
  2. Oral Ibuprofen: Doses: 15 – 30 mg/kg q12h
    (Most studies doses were ~ 20 mg/kg)

Other anti-inflammatory agents aren’t typically recommended

  1. Oral Corticosteroids
    - Some benefit but increase in side effects is also seen
  2. Inhaled Corticosteroids
    - Do see used for other co-morbidities (i.e. asthma symptoms)
35
Q

Oral Ibuprofen (Dose, Cmax target and 2 ADEs)

A

Dose: 15-30mg/kg q12h

Cmax target: 50 – 100 mg/L

Adverse Effects:

  1. Abdominal pain
  2. Epistaxis
36
Q

IV Antibiotic treatment (3)

A

Treat based on suspected organism and sensitivities from sputum culture; Also consider antifungals

  1. Macrolide Antibiotics
    - Azithromycin 500 mg TIW = most common dose and frequency
  2. Bactrim
  3. Minocycline (Stenotrophamonas)
37
Q

Macroline antibiotics for IV antiobiotic tx (4)

A
  1. Place in therapy: patients with chronic PSA infection
  2. Recommended for anti-inflammatory and antibiotic properties
  3. Studies have shown significant improvement in FEV1 (3 – 6%)
  4. Azithromycin 500 mg TIW = most common dose and frequency
38
Q

Pancreatic Enzyme Treatments (5 and 3 available products)

A
  1. Lipase, Protease, Amylase
  2. Dosing is based on lipase content (max 2,500 Units/kg per meal)
  3. Take with meals and snacks
  4. Capsules can be opened and mixed with food or liquids
  5. Use insulin for cystic fibrosis related diabetes

Available Products (not interchangeable)

  • PancreazeTM
  • Creon
  • ZenpepTM
39
Q

Pancreatic Enzyme Replacement Pearls (5)

A
  1. Do no crush/chew
  2. Not all products created equally (DAW)**
  3. Do not allow prolonged exposure to alkaline foods
    - i.e. pudding
  4. May empty in applesauce, jelly, or non-alkaline substances
  5. Give with meals
40
Q

Fat soluble vitamin supplementation (4)

A
  1. Vitamin A
  2. Vitamin D
  3. Vitamin E
  4. Vitamin K
41
Q

Nutrients affected with CF that may need supplementation (4)

A
  1. Calcium
  2. Iron
  3. Zinc
  4. Beta carotene
42
Q

Distral Intestinal Obstructive Syndrome (what it is and two treatments)

A

DIOS: one the of many abdominal manifestations of cystic fibrosis. In older children or young adults with cystic fibrosis, the distal small bowel may become obstructed with mucofaeculent material in the distal ileum and right colon.

  1. Polyethylene glycol 3350
    - 17 gm Miralax
    - 230 gm GoLytely
    - May require daily dosing
  2. Gastrograffin enemas
    - Not routine
43
Q

Tx for liver impairment

A

Ursodiol

  1. Reduces cholesterol, possibly reduces cytotoxicty of bile
  2. Improves liver function tests
44
Q

Treatments for lung transplant

A

Immunosupressants (CSA, prednisone)

45
Q

Treatment for nasal congestion (3)

A
  1. Saline Irrigation
    - Saline spray (OTC) products
  2. Nasal Corticosteroids
  3. Surgery
46
Q

CFTR Gene Potentiators (Mechanism of action and 2 drugs)

A

Mechanism: Only effective in certain mutations

Available agents

  1. Kalydeco
  2. Orkambi
47
Q

CF Foundation Recommendations for mild-severe CF

A
  1. Vitamin A,D,K,E supplements
  2. Pancreatic enzymes
  3. Dornase alfa
  4. Hypertonic Saline inhalations
  5. Inhaled B2-agonists
48
Q

When is inhaled trobamycin reocommended (2)

A

Mild to moderate disease: Chronic therapy may be appropriate in patients with colonized PSA infection

Moderate to severe disease:Recommended for all patients with PSA infection

49
Q

When is oral ibuprofen recommended?

A

only for moderate-severe disease for patients > 6 yrs w/ and FEV1 > 60%

50
Q

When is azithromycin recommended?

A

only for moderate-severe disease for patients > 6 with chronic PSA infection

51
Q

When are inhaled corticosteroids recommended?

A

Not recommended in patients who do not have concurrent asthma or ABPA symptoms

52
Q

When are oral corticosteroids recommended?

A

Not recommended in patients w/o asthma or ABPA

53
Q

Background of CF exacerbations (4)

A
  1. CF lung disease is characterized by chronic progression of disease and symptoms but also has intermittent episodes of acute worsening of symptoms (acute pulmonary exacerbations)
  2. CF exacerbations are an important marker for disease severity
  3. CF exacerbations increase with age
  4. Annual rate of exacerbations is associated with 2 and 5 yr survival
54
Q

Clinical Features of CF exacerbations (7)

A
  1. Decreased FEV1
  2. Cough
  3. Hemoptysis
  4. Increased sputum (production, volume)
  5. Shortness of breath
  6. Fever
  7. Anorexia, decreased appetite, weight loss
55
Q

CF Exacerbation Precipitating Factors

A

Infection:

  • Clonal expansion of existing strains, increased bacterial density
  • Viral infection
  • New colonizing organism
56
Q

Organisms that can lead to CF exacerbation

A
  1. S. aureus
  2. P. aeurginosa
  3. B. capecia complex (Associated with significant rapid decline, necrotizing pneumonia, and death)
  4. H. influenzae
  5. S. maltophilia
  6. A. xylosoxidans
  7. (Aspergillus, non-tuberculous mycobacterium)
57
Q

CF Exacerbation Maintenance Treatments (5)

A
  1. Pulmonary treatments
  2. Pancreatic Enzymes
  3. Physical Therapy
  4. Increase the time and frequency (twice to four times daily)
  5. Insufficient evidence to show that IV + inhaled antibiotics are beneficial or harmful
58
Q

IV antibiotics for CF Exacerbation

A
  1. S.Aeurus: Base treatment on susceptibilities
    - Vancomycin (MRSA)
  2. P. aeruginosa
    - 2x coverage is recommended
    - Antipseudomonal beta-lactams
    - Aminoglycosides: Once daily dosing is preferred
  3. Duration of therapy: 14 – 21 days
59
Q

Inhaled Tobramycin Use, Dosing, Types

A

Use: Approved for use in CF patients with P. aeruginosa

Dosing: 300 mg q12h x28 days

Types:
Requires specific nebulizer and air compressor:
-PARI LC PLUS Reusable Nebulizer
- DeVilbiss Pulmo-Aide air compressor

60
Q

PK of Inhaled Tobramycin (2)

A
  1. Sputum concentrations: high after 10 minutes but decreased to 14% after 2 hrs
  2. Systemic absorption: serum concentration ~ 1 ug/mL 1 hr after infusion
61
Q

Side effects of inhaled tobramycin

A
  1. Tinnitus

2. bronchospasms

62
Q

Lyophilized Aztreonam (Cayston) (Use, Dosing, and 5 common S/E)

A

Use: Tx for pulmonary lung infection

Dosing: 75 mg q6h x28 days
-Requires unique nebulizer system

Most common side effects

  1. Cough
  2. Nasal congestion
  3. Wheezing
  4. Pyrexia (fever)
  5. Pharyngeal pain

Advanced Pediatric Pharmacology Exam 1 (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions