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CF Flashcards

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1
Q

What are the regulatory functions of CFTR?

A

Ion transport (Cl, bicarbonate, Na)
Pulmonary inflammation
Bacterial adherence
Mucus rheology

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2
Q

What ion reuptake is inhibited in CF?

A

chloride

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3
Q

What is a class I defect in CF?

A

Most severe

Don’t make the regulator/protein

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4
Q

What is a class II defect in CF?

A 
Most common in this class and overall -
 deltaF508
Protein is immature
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5
Q

What is a class III defect in CF?

A 
CFTR protein synthesized and placed in membrane but is defunct and energy does not connect
G551D most common in this class
Secondary messenger activation and regulation dysfunctional
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6
Q

What is a class IV defect in CF?

A

CFTR protein is synthesized and placed in membrane but chloride ion flow is reduced

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7
Q

What is a class V defect in CF?

A

CFTR synthesized and processing is partially defective

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8
Q

What is a class VI defect in CF?

A

CFTR synthesized and placed in membrane but other ion flow is reduced

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9
Q

How do we diagnose CF?

A 
CF sx in one or more organ systems
Evidence of CFTR dysfunction
Prenatal testing
Newborn screens
Sweat chloride test
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10
Q

How do we classify an abnormal sweat test?

A

> 60 no matter the age

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11
Q

How do we classify an intermediate sweat test?

A

< 6 months: 30-59

> 6 months: 40-59

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12
Q

How do we classify a normal sweat test?

A

< 6 months: < 30

> 6 months: < 40

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13
Q

What are gastrointestinal presentations of CF?

A 
Meconium ileus
Failure to thrive
Steatorrhea
Low protein problems (Edema, hypoproteinemia, anemia)
Electrolyte variability
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14
Q

Which classes tend to have pancreatic insufficiency?

A

Class I and II

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15
Q

What test is used to test for pancreatic insufficiency?

A

Fecal elastase testing

Less than 200 indicates insufficiency

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16
Q

What are the general principles for a pancreatic enzyme replacement therapy (PERT)?

A

Enzymes - lipase, protease, amylase
Dose based on lipase unit per weight or fat intake
Coating dissolves in alkaline environments

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17
Q

What are the wt based dosings for < 4 yo in PERT?

A

1000 units/kg/meal

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18
Q

What are the wt based dosings for > 4 yo in PERT?

A

500 units/kg/meal

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19
Q

What is the max units per meal?

A

2500

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20
Q

What kind of meal should PERT be given with?

A

Fatty snacks

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21
Q

What are the fat based dosings for PERT?

A

2000 units per 120mL of feedings
Max 2500 units/kg/feeding
Max daily dose of 10,000 units/kg

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22
Q

What vitamins must be started with PERT in every patient?

A

Fat soluble vitamin replacement

ADEK

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23
Q

What are the RFs for CF-related diabetes?

A 
Advanced age
Female
Pancreatic insufficiency
Increased insulin resistance
Decreased insulin production
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24
Q

What is the treatment for CR-related diabetes?

A

Insulin

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25
What is the intestinal manifestation of CF?
``` GERD Meconium ileus DIOS Constipation Intussesception Small bowel bacterial overgrowth Rectal prolapse ```
26
What causes GERD in patients with CF?
Decreased LES tone Increased intra-abdominal pressure Hyperinflated lungs Chest physiotherapy
27
What is the dosing of PPIs in CF?
0.2-2 mg/kg/d | 1-2 x daily
28
What are the classifications of meconium ileus?
Simple | Complex (perforation, atresia, meconium peritonitis)
29
How is a meconium ileus diagnosed?
Contrast enema (usually treats it as well)
30
What is common in patients with h/o of meconium ileus?
Distal intestinal obstruction syndrome (DIOS)
31
What is distal intestinal obstruction syndrome?
Acute blockage of the ileum?
32
What is treatment for complete DIOS?
Surgery
33
What is the treatment for partial DIOS?
``` Rehydration Osmotic laxatives (PEG, Gastrografin, electrolyte solution) ```
34
What are hepatobiliary problems in patients with CF?
Biliary cirrhosis Liver disease Neonatal cholestasis
35
What is the treatment of biliary cirrhosis?
Ursodiol 30 mg/kg/d divided BID
36
How can we tell if a patient with CF has liver disease?
Slight elevations in alkaline phosphatase
37
When do we not administer ursodiol?
T bilirubin < 2
38
What are risk factors for neonatal cholestasis?
H/o meconium ileus | Prolonged parenteral nutrition
39
What is the treatment for neonatal cholestasis?
Ursodiol 30 mg/kg/d divided 2-3 times daily
40
What is the pulmonary presentation of CF?
``` Recurrent cough Persistent cough Prolonged wheezing Recurrent bronchiolitis Productive cough ```
41
How do we treat reactive airway diseases in CF?
Bronchodilators
42
How can we tell if there is worsening lung disease in CF?
Reduction in smooth muscle support system | Collapsing muscle worsens obstruction
43
What are the causes of chronic pulmonary issues in CF?
``` Chronic airway obstruction Bacterial colonization of clogged airways Chronic medications (bronchodilators, Beta-agonists) ```
44
What drugs are used for improving mucus clearing in CF?
Dornase alfa | Hypertonic saline
45
How does dornase alfa work?
Cleaves DNA released during neutrophil death
46
What is the only proven treatment to decrease hospital length of stay d/t exacerbations?
Dornase alfa
47
What is the dosing for dornase alfa?
2. 5 mg QD | 2. 5 mg QOD
48
How is hypertonic saline useful in CF patients?
Hydrates mucus in lungs decreasing viscosity
49
What is a SE of hypertonic saline in CF patients?
May cause bronchospasm with administration
50
What is the dosing of hypertonic saline in CF patients?
4 ml BID
51
What inhaled abx are used as maintenance medications in CF?
Tobramycin | Azithromycin
52
What is dosing of inhaled tobramycin in CF patients?
300 mg per inhalation twice daily for 28 days | 1 month on, 1 month off course
53
What is decreased when inhaled azithromycin is used?
Inflammatory response | Bacterial load in airways
54
What is inhaled azithromycin dosing?
250-500mg PO TIW
55
What are the targeted therapies for CF?
Ivacaftor | Lumacaftor
56
What is ivacaftor used for?
Targets G551D mutations Improves pulmonary function Decreases exacerbations Increases weight gain
57
What is the dosing for ivacaftor?
150 mg PO BID with fatty foods | Hepatic and 3A4 dose reductions required
58
What is lumacaftor used for?
``` > 12 years old at this time Targets deltaF508 Improves pulmonary function Decreases exacerbations Increases weight gain ```
59
What is the dosing for lumacaftor?
In combination with ivacaftor 400mg/250mg PO BID with fatty foods Hepatic 3A4 dose reductions required
60
What is the presentation of acute pulmonary exacerbations?
``` Increased cough with sputum production Sputum changes Increased SOB with exertion Decreased intake Fever Increased fatigue ```
61
What is the duration of treatment for acute pulmonary exacerbations?
10-14 days
62
What is the abx regimen based on in acute pulmonary exacerbations?
Historical cultures | Age-correlated pathogens
63
What abx are used for the treatment of acute pulmonary exacerbations?
``` Clear faster (except for Vanc) Beta-lactams AGs FQs Vanc (no changes in PK) Sulfonamides ```
64
How is TMP cleared?
Renal
65
How is SMX cleared?
SMX

Pediatrics (9 decks)

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