Obstructive Lung Disease CIS- Kinder

Question 1

Alpha-1 Antitrypsin Deficiency

Answer 1

Genetic risk factor for COPD
Etiology of 1-2% of COPD cases
Serine protease inhibitor secreted by the liver that protects the lung tissue against the action of neutrophil elastase and serine proteases.
Patients are very susceptible to damage from cigarette smoking.
Should be considered in young patients with COPD
Can lead to LFT abnormalities and cirrhosis

Question 2

Cystic Fibrosis

Answer 2

Autosomal recessive disorder caused by mutation of the cystic fibrosis transmembrane conductance regulator protein.
Organs affected include lungs, pancreas, intestines, liver, sweat gland, sinuses, and the vas deferens.
Lung disease leads to death in 90% of patients.
Median survival 37 years

Question 3

Lung Manifestations in CF

Answer 3

Cough, dyspnea, decreased exercise tolerance, fatigue, and increased sputum production
Steep decline in lung function at adolescence
Daily productive cough
Airway infection with Pseudomonas aeruginosa is the primary pathogen. Staphylococcus aureus, and methicillin resistant Staphylococcus aureus are also common.
Other infections include bronchopulmonary mycoses, and nontuberculous mycobacterial infections

Question 4

Pancreatic Manifestations of CF

Answer 4

Exocrine pancreatic insufficiency can lead to impaired growth
Signs of malabsorption include bulky, foul smelling stools and flatulence.
Malabsorption of fat soluble vitamins occur.

Question 5

Liver Manifestations of CF

Answer 5

Hepatomegaly
Splenomegaly
Hematemesis secondary to esophageal or gastric varices from portal hypertension

Question 6

Sweat Gland in CF

Answer 6

Failure of chloride absorption from the lumen into the ductal lining cell.
Marked elevation of chloride and sodium in sweat

Question 7

Vas deferens in CF

Answer 7

Almost all males are sterile

Question 8

Other clinical manifestations in CF

Answer 8

Endocrine Pancreas: 1/3 of patients have diabetes by age 30
Electrolyte abnormalities can lead to nausea/vomiting, decreased appetite, circulatory collapse, and seizures
Musculoskeletal: decreased bone density secondary to decreased absorption of vitamin D, glucocorticoid treatment, and decreased exercise.
Kidney: nephrolithiasis

Question 9

Cystic Fibrosis Diagnosis

Answer 9

Screening with immunoreactive trypsinogen which is a marker of pancreatic injury
Genetic mutation analysis
Diagnosis confirmed with sweat testing
The sweat test measures chloride concentration in sweat that is stimulated by pilocarpine iontophoresis.
5% of diagnosis are made after the age of 18

Question 10

Pulmonary function testing in CF

Answer 10

Obstruction

Question 11

Chest x-ray in CF

Answer 11

Hyperinflation

Bronchiectasis

Question 12

Oral Antibiotics in CF

Answer 12

Azithromycin [25-40 kg] 250 mg PO every Mon, Wed, Fri; [> 40 kg] 500 mg PO every Mon, Wed, Fri
Patients ≥ 6 years of age with Pseudomonas aeruginosa meet criteria for use
Trimethoprim/sulfamethoxazole 20 mg/kg/day PO divided every 6-8 hours
Ciprofloxacin 40 mg/kg/day PO divided every 12 hours

Question 13

IV antibiotics in CF

Answer 13

Aztreonam 200 mg/kg/day IV divided every 6-8 hours

Cefepime 150 mg/kg/day IV divided every 8 hours

Ceftazidime 200 mg/kg/day IV divided every 8 hours

Ciprofloxacin 30 mg/kg/day IV divided every 8-12 hours

Meropenem 120 mg/kg/day IV divided every 8 hours

Piperacillin/tazobactam 400 mg/kg/day divided every 4-6 hours

Tobramycin 7.5-15 mg/kg/day IV divided every 8-24 hours
[Higher dose, extended interval dosing may be more effective and less nephrotoxic]

Vancomycin 60 mg/kg/day divided IV every 6-8 hours

Question 14

Inhaled antibiotics in CF

Answer 14

Tobramycin 300 mg inhaled twice daily in 28 day cycle

Aztreonam 75 mg inhaled three times daily in 28 day cycle

Question 15

Other CF Treatments

Answer 15

Pancreatic enzymes, vitamin supplementation, bronchodilators, hypertonic saline inhalation, dornase alfa(rhDNase), ibuprofen, oxygen, bronchodilators, lung transplant

Chest percussion and postural drainage

Question 16

Which of the following Pulmonary Function Test results are most likely in a patient with bullous emphysema?
A) Decreased FEV1/FVC
B) Decreased functional residual capacity
C) Decreased total lung capacity
D) Increased FEV1
E) Increased FEV1/FVC

Answer 16

A, decreased FEV1/FVC

Question 17

Chronic Obstructive Pulmonary Disease

Answer 17

Progressive, mostly irreversible airflow obstruction
Onset: Middle age or elderly 20-30 years after exposure
4th leading cause of mortality in the United States
Cigarette smoking is the leading cause of COPD
Smokers have a 40ml/year reduction in FEV1 after age 30
Lung growth can be impaired by maternal smoking in pregnancy and second hand smoke in childhood
Other exposures: workplace dusts from mining, cotton mills, and grain handling facilities

Question 18

Lung Mechanics in COPD

Answer 18

Elastic recoil is the lungs innate ability to deflate following inflation.
Elastic fibers in the lung parenchyma, along with surface tension at the alveolar air-liquid interface are responsible for elastic recoil
Elastic recoil maintains the patency of small airways
Elastic recoil is markedly decreased in COPD
Airway resistance is increased in COPD
The sites of airflow obstruction are distal airways less than 2mm diameter

Question 19

COPD History

Answer 19

Current or past cigarette use
Dyspnea with slow progression
History of acute bronchitis
History of a chronic cough
Sputum production
Wheezing

Question 20

Physical Findings COPD

Answer 20

Barrel Chest
Prolonged expiratory phase
Accessory muscle use
Low Diaphragm
Distant Heart Sounds
Diminished Breath Sounds
Rhonchi
Wheezing
Cyanosis
Pedal Edema
Distended Jugular Veins
Hepatic congestion
Cachexia
“Blue Bloaters” -cyanotic, respiratory acidosis
“Pink Puffers”- usually emphysematous, don't respond to bronchodilators, cachectic

Question 21

pumonary function testing stages and severity

Answer 21

Mild- FEV1 > 80% predicted
moderate- > 50%
severe: > 30%
Very severe: under 30%

For all categories FEV1/FVC is under 70%

Question 22

Chest x-ray in COPD

Answer 22

Hyperinflation
Flattened Diaphragms
Increased restrosternal space
Bullae
Can be normal in mild to moderate COPD

Question 23

Emphysema

Answer 23

Enlargement of the air spaces distal to the terminal bronchiole with destruction of the alveolar walls
Centriacinar: affects respiratory bronchioles distal to the terminal bronchiole, remainder of the acinus spared. Occurs with smoking
Panacinar: alveolar ducts, adjacent alveoli, coalescence and bullae formation. Common in alpha 1 antitrypsin deficiency. Occurs in smoking
Most severe COPD patients have a combination of centriacinar and panacinar emphysema

Question 24

Chronic Bronchitis

Answer 24

Enlargement of bronchial mucous glands and increased epithelial goblet cell production leads to cough and increased mucous production.

increased Reid index

Question 25

Asthma defn

Answer 25

Clinical syndrome of unknown etiology with 3 distinct components 1) Recurrent airway obstruction that resolves spontaneously or with treatment. 2) Airway hyperresponsiveness: exaggerated bronchoconstrictor response to stimuli that have little or no effect on nonasthmatic patients. 3) Airway Inflammation

Question 26

Population Affected by Asthma

Answer 26

8% of Adults Boys more common than girls before puberty Women more common than men Most cases start before age 25, but can occur at any age

Question 27

Asthma Pathology

Answer 27

Mild Asthma: edema and hyperemia of the mucosa and infiltration of the mucosa with mast cells, eosinophils, and lymphocytes. Moderate Asthma: chemokines eotaxin, RANTES, macrophage inflammatory protein 1 alpha, and interleukin 8 lead to inflammation and smooth muscle constriction Severe: hypertrophy and hyperplasia of airway glands and smooth muscle lead to severe airway thickening

Question 28

Airway Obstruction

Answer 28

Caused by a combination of 1) constriction of airway smooth muscle 2) thickening of airway epithelium 3) liquids in the airway

Question 29

Asthma Triggers

Answer 29

``` Atopy Occupational Allergy Food Cold Air Smoking or Smoke in the environment Pollution Climate Changes Emotion Medications ```

Question 30

Asthma Mediators

Answer 30

Acetylcholine: released from intrapulmonary motor nerves stimulate M3 muscarinic receptors causing airway smooth muscle constriction Histamine: released from mast cells – minor role Leukotrienes and Lipoxins: derived by the lipoxygenation of arachidonic acid released from the target cell membrane phospholipids during cellular activation Nitric Oxide: produced by airway epithelial cells and by inflammatory cells found the asthmatic lung. High levels found during asthma attacks

Question 31

Asthma History

Answer 31

Dyspnea, cough, wheezing, and anxiety Exercise induced, aspirin ingestion, extrinsic(allergen induced), or intrinsic(unknown) Cough, hoarseness, or inability to sleep through the night Rapid changes in temperature or humidity may lead to an attack Consider occupation exposures

Question 32

Asthma Categories (in terms of severity)

Answer 32

Mild intermittent: symptoms present for 2 days/week or less, or 2 nights/month or less Mild persistent: symptoms present for > 2 days/week but less than once daily, or > 2 nights/ month Moderate persistent: symptoms present daily or greater than once/night Severe persistent asthma: symptoms are continual during the day and frequent at night

Question 33

Asthma Physical Examination

Answer 33

Often normal between attacks Vital Signs tachypnea with respiratory rate often between 25-40 breaths per minute, tachycardia, and pulsus paradoxus. Accessory muscle use, hyperinflation, prolonged expiratory phase Wheezing loudest during expiration, but can also be heard during inspiration. Wheezing is polyphonic. Decreased breath sounds in asthma patients is an indication of severe obstruction. Ominous signs are inability to speak or drink, fatigue, drowsiness, confusion, and cyanosis.

Question 34

Asthma Testing

Answer 34

ABG Often mild hypocapnea. If PaCO2 normalizes during a severe attack, this may indicate impending respiratory failure. PFT: obstruction CBC: Eosinophilia(Hypersensitivity Only), Elevated IgE Chest X-ray is often normal, hyperinflation, in severe asthma may have pneumothorax or pneumomediastinum EKG: sinus tachycardia is usual, May see right axis, RBBB, P pulmonale, and even ST-T changes in a severe attack that will resolve after treatment. Sputum often contains eosinophils

Question 35

Outpatient Treatment of Asthma

Answer 35

Step 1 Intermittent Asthma - No daily medication - Short-acting beta-2 agonist as needed Step 2 Mild Persistent Asthma - Short acting beta-2 agonist as needed - Inhaled corticosteroid - Alternate treatments mast-cell stabilizer, leukotriene- receptor antagonist, or theophylline Step 3 Moderate Persistent Asthma - Short-acting beta-2 agonist as needed - Low to medium dose inhaled corticosteroid - Long-acting beta-2 agonist Alternate treatments: increase in inhaled corticosteroids within medium dose range; or low to medium dosed inhaled corticosteroids and either a leukotriene-receptor antagonist or theophylline Step 4 Severe Persistent Asthma - Short-acting beta-2 agonist as needed - High-dose inhaled corticosteroid and long-acting beta-2 agonist - If symptoms persist, 2mg/kg/day of prednisone may be required, generally not to exceed 60mg/day Other Treatments Omalizumab: monoclonal antibody in patients with moderate to severe persistent asthma who have shown reactivity to an allergen and whose symptoms are inadequately controlled by an inhaled corticosteroid.

Question 36

Bronchiectasis

Answer 36

Abnormal permanent dilatation of the bronchi and bronchioles caused by repeated cycles of airway infection and inflammation. Abnormalities of cilia, mucous clearance, mucus rheology, airway drainage, and host defenses can lead to bronchiectasis. Patients develop chronic infections that lead to lung destruction.

Question 37

Bronchiectasis Etiologies

Answer 37

One half of patients have cystic fibrosis One third have an infectious etiology often years before the onset of disease. Infectious etiologies include pertussis, TB, Mycobacterium avium-intracellulare. MAI typically involve the right middle lobe and lingula Genetic etiologies include cystic fibrosis, primary ciliary dyskinesia, and alpha 1 antitrypsin deficiency. Anatomic etiologies include esophageal dysfunction with aspiration, COPD, allergic bronchopulmonary aspergillosis, endobronchial tumors, extrinsic compression by lymph nodes and foreign bodies. Immune and autoimmune etiologies include primary hypogammaglobulinemia, immunoglobulin G deficiencies, HIV, Sjogren’s syndrome, and rheumatoid arthritis

Question 38

Bronchiectasis Symptoms

Answer 38

``` Chronic cough with purulent sputum production Dyspnea Intermittent hemoptysis Pleuritic chest pain Weight loss Fatigue ```

Question 39

Bronchiectasis Physical Exam

Answer 39

Wheezing, rales Slow decline in pulmonary function Decline more rapid if patient has Pseudomonas aeruginosa

Question 40

Bronchiectasis Imaging

Answer 40

High Resolution CT used to make diagnosis Findings: - Lack of bronchial tapering - Bronchi visible in the peripheral 1 cm of the lungs - Internal bronchial diameter greater than the diameter of the accompanying bronchial artery

Question 41

Bronchiectasis Location

Answer 41

Cystic Fibrosis: upper lobe predominance Aspiration: lower lobe predominance MAI: right middle lobe and lingular predominance Bronchopulmonary aspergillosis: central bronchiectasis

Question 42

Bronchiectasis Testing

Answer 42

PFT: demonstrates obstruction Bronchoscopy: Used to detect airway abnormalities including tumors, structural deformities, and foreign bodies. Most helpful if the bronchiectasis is localized Sputum cultures and bronchial alveolar lavage used to assess infectious etiologies

Question 43

Bronchiectasis Treatment

Answer 43

Treat underlying condition Specific antimicrobials Anti-inflammatory – inhaled steroids, macrolide antibiotics Surgery for localized or refractory disease Transplantation for end-stage disease

Question 44

Ventury mask

Answer 44

you can dial in the amount of oxygen you want to deliver. When patients receive too much oxygen they can lose their respiratory drive

Question 45

Acute Farmer’s Lung

Answer 45

Develops after large exposure to moldy hay or contaminated compost Symptoms often spontaneously resolve within 12 hours to days if antigen exposure is eliminated or avoided Acute farmer’s lung manifest as new onset of fever, chills, nonproductive cough, chest tightness, dyspnea, headache , and malaise. If the inhalational exposure is large, patients may develop acute respiratory failure

Question 46

Subacute Farmer’s Lung

Answer 46

Manifest as chronic cough, dyspnea, anorexia, and weight loss Subacute disease is insidious in onset and may occur over weeks to months.

Question 47

Chronic Farmer’s Lung

Answer 47

Results from prolonged and continuous exposure to antigen Patients may have irreversible lung damage Patients may experience severe dyspnea at rest or with exertion

Question 48

Samter’s Triad

Answer 48

Asthma Nasal Polyps ASA, NSAID sensitivity

Question 49

When do you get Reactive airways dysfunction syndrome (RADS)?

Answer 49

one-time exposure to a toxic substance

Question 50

beta-blocker induced asthma can come from, for example,

Answer 50

glaucoma treatments

Question 51

branched tubular radiodensities that the radiologist describes as “gloved finger” shadows.

Answer 51

bronchiectasis

Question 52

treatment for allergic bronchopulmonary aspergillosis

Answer 52

Prednisone in severe cases hydroconizone (an antifungal)

Question 53

Allergic bronchopulmonary aspergillosis

Answer 53

Occurs in asthmatics and CF patients from a hypersensitivity reaction to Aspergillus colonization of the tracheobronchial tree This syndrome may cause fever and pulmonary infiltrates that are unresponsive to antibacterial therapy. Patients often have a cough and produce mucous plugs, which may form bronchial casts. Possible hemoptysis. People with asthma who have ABPA are usually poorly controlled asthmatics with difficulty tapering off oral corticosteroids ABPA may occur in conjunction with allergic fungal sinusitis, with symptoms including chronic sinusitis with purulent sinus drainage.