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52 Cards in this Set

  • Front
  • Back
How do you make diagnosis of cystic fibrosis
- Recurrent lung infections and sometimes chronic malabsorption leading to failure to thrive (85% of patients suffer from pancreatic insufficiency)
- Abnormally high levels of chloride in sweat = positive sweat test
What is the most serious problem in cystic fibrosis
Obstruction of airways by viscous mucus in the bronchioles and colonization by bacteria - Staph Aureus and Pseudomonas - leads to lung destruction (bronchiectasis is common) and also cause R heart failure - most patients die from respiratory infections
Which GI problem is present in patients with CF
Pancreatic ducts are obstructed by mucus resulting in deficient secretion of pancreatic enzymes and indigestion of proteins and fats - failure to thrive and production of copious maloderous stools
Describe management of CF
- Vigorous regular chest PT with antibiotic treatment for infections
- Dietary supplementation of pancreatic enzymes and fat soluble vitamins (ADEK)
- Surgical intervention for small intestinal obstruction and both liver and heart-lung transplants = extreme cases
Name gene defective in CF
CFTR = cystic fibrosis transmembrane regulator, functions as chloride channel
Which amino acid is deleted in CF and where - this mutation responsible for 70% of cases
Phenylalanine at position 508 in the CFTR
Which method is used for screening of carriers of CF
What type of protein is alpha 1 antitrypsin
Serine protease inhibitor - major component of alpha 1 globulin of human plasma and is synthesized by hepatocytes and also macrophages
What is the major function of alpha 1 antitrypsin
Protect lungs from digestion by inhibition of the activity of elastase - a proteolytic enzyme produced principally by neutrophils
Alpha 1 antitrypsin defficiency results in _
Chronic pulmonary disease (emphysema) and liver disease
Which mutant alleles are responsible for alpha 1 antitrypsin defficiency
S and Z
Pittsburgh variant antitrypsin defficiency
Missense point mutationsubstituting an arginine for the methionine - destroys affinity for elastase but now targets thrombin causing lethal bleeding disorder
2 causes of increased pulmonary disabilities in Americans
Smoking and air pollutants
Common diagnoses of patients who would benefit from pulmonary rehab
Obstructive - asthma, COPD (bronchitis + emphysema)
Cystic fibrosis - bronchiectasis
Restrictive diseases - interstitial fibrosis, pneumoconiosis, kyphoscoliosis
Lung reductin and transplant candidates before and after the surgery
Conditions that would prevent patient from participating in a rehab
Terminal disease states

Psychiatric problems- dementia, substance abuse

Serious medical conditions - unstable CHF, post MI, CVA
Parameters that should be monitored during simple 6 or 12 minute walk



Respiratory rate

Symptoms- orthopedic as well as respiratory
3 possible reasons for loss of appetite in patinets with COPD
Air swallowing, dyspnea

Using O2, loss of sense of smell and taste

Taking meds and water (stomach full)
What is collaborative self management in pulmonary rehab
Assesment of learning abilities and needs - involves patient, family and caregivers, hypoxemia can impair ability to leart and retain information

Educate on topics of anatomy and physiology, pathology, meds, diet/nutrition, relaxation techniques, self care, establishing realistic objectives/goals, patient interests, travel and sexuality, and end of life decision making (advanced directives)

Testing on those can formal or informal, need to repeat demonstrations if needed
Common psychosocial issues that commonly effect pulmonary rehab candidates

Role reversal

Dependence (loss of independence)

Low self esteem



Learning abilities

Coping difficulties/strategies
2 contraindications for Trendelenburg position if performing chest physical therapy
Vomiting and risk of aspiration

Recent CVA or cranial surgeries
Describe technique for pursed lip breathing
Inspire during diaphragmatic breathing

Exhale normally through pursed lips

Emphasize slow exhalation not forced

Practice is required
Benefits of pursed lip breathing
Slows respiratory rate

Prevents or delays airway collapse by pressure splinting airway

Decreases air trapping

Decreases dyspnea
Describe 3 oxygen supply methods available to home care patients
Concentrators for continuous users who mostly stay at home

Liquid oxygen for continuous users who are active

Mechanical ventilation at home - conventional positive pressure ventilation, noninvasive positive pressure ventilation, bilevel positive pressure ventilation BiPAP
Home oxygen supply method most suited for active ambulatory patient
Liquid oxygen for continuous users who are active
Identify disadvantage or hazard of each home oxygen supply method
Liquid O2 - potential hazard of thermal injury if not proporly handled

Cylinders - high pressure systems, finite amount, needs to be monitored to avoid runing out

Concentrators for stay at home users - increase electric cost, preventative maintenance, need back up power supply
3 benefits of exercise training to a dyspnea limited COPD patient
Desensitization to dyspnea

More mechanically efficient

Psychologically - improved ego strength

Carries over to ADL
Benefits of pulmonary rehab
Quality of life

Reduction in symptoms

Increased exercise tolerance

Increase in functional activities of daily living

Increased independence

Improved psych function
Describe appropriate use of nicotine replacement along with behavior modification in smoking cessation
Nicotine addicted smokers (> 1 pack a day) benefit from nicotine replacement

Nicotine replacement must be accompanied by behavior modification

Recent studies indicate better success with nicotine replacement than "cold turkey"

Increased success with bupropion (Zyban) that may also help prevent undesirable weight gain
Currently recommended immunizations for someone with pulmonary disability
Pneumonia every 6 years

Influenza yearly
Virchows triad


Endothelial injury
Shortness of breath + clear lungs - what should you suspect
Describe pathophysiology of PE
Impaired gas exchange --> increased dead space leading to hypoxia

Increased pulmonary vascular resistance --> caused by obstruction and neurohormonal influence (serotonin)

Increased airway resistance --> bronchoconstriction

Decreased pulmonary compliance --> lung edema, hemorrhage, loss of surfactant
Name risk factors for PE
Bed bound
What is the most common coagulopathy
Factor V Leiden mutation
What are the common symptoms of PE

Pleuritic chest pain

Cough - possible hemoptysis

Leg pain and swelling - DVT

Palpitations, diaphoresis, wheezing

Non specific symptoms

Massive cardiovascular collapse, coma, death
What are the signs of PE


Rales (crackles) or wheezes


Pleural friction rub

Which work up is ordered in patient with PE



CT angiogram - gold standard

Lung scan - not used as much
Westermarks sign - decreased vascularity or focal oligemia is the sign of _
Pleural effusion, atelectasis and decreased vascularity on x ray are signs of _
Pleuritic chest pain + hemoptysis =
Pulmonary infarction
Chest x ray is suggestive of pneumonia - shows parenchymal density which extends to the pleural surface - what else should you think about?
PE with infarction
NSR - 30%

Sinus tachycardia

Right heart strain pattern - RBBB, S1, Q3

A fib, A flutter (PAC, PVC)

P pulmonale (peaked in II,III)
Very sensitive but not specific test for PE - gives many false positives, but very few false negatives
D dimer
What is the differential diagnosis of PE





Tests for DVT



Radionuclide scanning
Treatment of PE
First thing is to prevent further PE - heparin and warfarin (anticoagulants, not thrombolytics), dont dissolve blood clots


IVC filter - bleeding, recurrences

Thrombolytic therapy - massive PE, hemodynamic instability

Intubation and/or vasopressors

Give examples of non thrombotic PE
Fat embolism - long bone fractures, petechiae, confusion

Amniotic fluid embolism - postpartum pulmonary edema

IVDA -talc
Bronchial breath sounds heard peripherally (where they are not supposed to be heard) - diagnosis?
Pneumonia (consolidation)
Egophony is present in what disease
Wheezing in people without asthma is the sign of _
Crackles and rales are lung sounds heard in _
Silent chest + signs of respiratory distress are indicative of _
Asthma - severe - emergency