Respiratory

Question 1

Right sided heart failure; an enlargement of the right ventricle due to high blood pressure in the arteries of the lungs usually caused by chronic lung disease

Answer 1

Cor Pulmonale

Question 2

Asthma symptoms present less than once/week, otherwise asymptomatic. Nighttime symptoms present less than twice/month

Answer 2

Mild Intermittent

Question 3

Mainly airway obstruction with air trapping; X-ray shows high lung volume. Examples include asthma, CF, and BPD

Answer 3

Obstructive Lung Disease

Question 4

Montelukast and zafirlukast; Long term medication used in combination with ICS and LABA

Answer 4

Leukotriene modifier

Question 5

Asthma symptoms present more than once/week but less than once/day. Nighttime symptoms present more than twice/month.

Answer 5

Mild Persistent

Question 6

Albuterol, levalbuterol Taken as needed to provide prompt reversal of acute airflow obstruction; Step 1. Onset of action is within 5 minutes; if using more than 2x/week, additional therapy required

Answer 6

SABA

Question 7

Chronic inflammatory disorder characterized by chronic airway inflammation due to airway hyperresponsiveness to a variety of stimuli and variable airway obstruction. Immunohistopathologic features include inflammtory cell infiltration with neutrophils, eosinophils, lymphocytes; and mast cell activation and epithelial cell injury. Persistent airway inflammation can lead to airway wall remodeling, irreversible changes, and loss of pulmonary function

Answer 7

Asthma

Question 8

Most common fatal genetic disorder affecting Caucasian population; median life expectancy is 41. Respiratory failure is leading cause of death

Answer 8

Cystic Fibrosis

Question 9

Signs and symptoms include: 1. Extremely viscid meconium (newborns), with delayed passage 2. Poor growth despite normal to increased appetite; FTT 3. Recurrent and chronic respiratory infections; dyspnea on exertion that progresses to dyspnea at rest as disease progresses 4. Large, bulky, foul-smelling, greasy stools 5. Frequent flatulence or abdominal pain 6. Chronic cough that is usually productive, blood-streaked mucus is not uncommon 7. Recurrent or persistent wheezing 8. Salty-tasting skin 9. Distal small bowel obstruction 10. Recurrent pancreatitis 11. Related DM 12. Salt loss syndromes 13. Heat prostration with hypernatremia dehydration 14. Male infertility and female reduced fertility

Answer 9

Cystic Fibrosis

Question 10

Physical findings include: 1. Wheezing and air trapping with increased AP diameter of the chest 2. Crackles 3. Increased work of breathing with accessory muscle use 4. Nasal polyps, chronic sinusitis 5. FTT from maldigestion and fat malabsorption 6. Abdominal pain and distention, unexplained pancreatitis or cirrhosis, hepatosplenomegaly, cholelithiasis 7. Rectal prolapse 8. Digital clubbing 9. Delayed puberty 10. Meconium ileus

Answer 10

Cystic Fibrosis

Question 11

Used in short courses for control of asthma symptoms; also used as controller with step 6 severity (if severe, chronic asthma)

Answer 11

Oral Corticosteroids

Question 12

Omalizumab Long term control and prevention of symptoms in children >12 years of age who have moderate to severe persistent asthma inadequately controlled by ICS. Prevents the binding of IgE to mast cells, used for steps 5-6 in controlled clinical settings because of side effects

Answer 12

Immunomodulator

Question 13

Autosomal recessive disorder that causes defective epithelial ion transport, which results in dehydrated, viscous secretions that obstruct the exocrine ducts in the respiratory, hepatobiliary, gastrointestinal, and reproductive tracts.

Answer 13

Cystic Fibrosis

Question 14

Findings include: 1. Pilocarpine iontophoresis sweat test - 2 tests with sweat chloride in excess of 60 2. Genotyping can show 2 disease-causing mutations 3. Elevated immunoreactive trypsin in newborn; must be confirmed with sweat test 4. Chest x-ray (hyperinflation, increased peribronchial markings, atelectasis, bronchiectasis 5. CT scan - early bronchiectasis 6. PFT 7. Sputum culture 8. Oximetry 9. Hyponatremia, hypoproteinemia, hypochloremic alkalosis 10. Elevated liver enzymes 11. Vitamin A, E, K deficiencies

Answer 14

Cystic Fibrosis

Question 15

Daily asthma symptoms and beta agonist use, activity interference. Nighttime symptoms are present more than once/week

Answer 15

Moderate Persistent

Question 16

5-Lipoxygenase inhibitor used for long term control and prevention of symptoms in mild persistent asthma for children >12 years of age; may be used in combination with ICS for moderate persistent asthma Microsomal P450 enzyme inhibitor that can inhibit the metabolism of warfarin and theophylline

Answer 16

Zileuton

Question 17

Asthma symptoms are continuous and physical activity is limited. Frequent nighttime symptoms present.

Answer 17

Severe Persistent

Question 18

Caused by mutations in a single gene on the long arm of chromosome 7, which directs the production of CFTR, the principle chloride channel of epithelial cells and controller of other ion transport. Defective CFTR results in increased sodium reabsorption, decreased chloride secretion, and dehydrated, highly vicous secretions in all exocrine ducts, which causes disease

Answer 18

Cystic Fibrosis

Question 19

Budesonide, fluticasone Long-term control medication taken daily; preferred medication for all children with mild, moderate, or severe persistent asthma; Step 2

Answer 19

Inhaled Corticosteroids

Question 20

Management includes: replacement of pancreatic enzymes, nutritional support, airway clearance techniques, prevention and aggressive treatment of infection 1. Inhaled mucolytic agent 2. Recombinant human DNase (pulmozyme) 3. Inhaled tobramycin and oral azithromycin 4. Bronchodilators and anti-inflammatory therapies

Answer 20

Cystic Fibrosis

Question 21

Ipratroprium bromide, inhaled Quick relief medication used for the relief of acute airflow obstruction; may be used as alternative to SABAs

Answer 21

Anticholinergic

Question 22

Salmeterol and formoterol, inhaled Long term medication used in combination with ICSs when ICSs are not effective alone; not to be used as monotherapy. Black box warning due to association with severe asthma attacks and death; close monitoring advised; Step 3A

Answer 22

LABA

Question 23

Symptoms include: 1. Recurrent episodes of cough, wheezing, mucus production, chest tightness, breathlessness, and decreased endurance 2. Symptoms often worse at night, early morning, and during or after exercise

Answer 23

Asthma

Question 24

Causes airway narrowing and increased airway secretions; contributes to airway hyperresponsiveness

Answer 24

Airway inflammation

Question 25

Virulent pathogen that has innate antibiotic resistance, causes rapid decline in lung function, and is associated with mortality in CF patients

Answer 25

Burkholderia Cepacia

Question 26

Pulmonary disease that causes diminished lung capacity due to poor chest wall compliance, decreased muscle strength, and poor cough reflex leading to decreased airway clearance. Causes include NM disease, scoliosis, chest wall deformities, CP, and myelomeningocele.

Answer 26

Restrictive Lung Disease

Question 27

Findings include: 1. Splinting of upper limb girdle 2. Barrel chest 3. Air trapping on x-ray Causes: asthma, bronchiolitis, HIV-associated lung disease

Answer 27

Chronic Wheezing

Question 28

Physical findings include: 1. Cough and shortness of breath 2. Diffuse wheezes with decreased airflow and prolonged expiratory phase 3. Respiratory distress, retractions, increased hypoxia, and decreased breath sounds as severity increases 4. Allergic appearance - allergic shiners, nasal crease, nasal congestion and mouth breathing, boggy turbinates and nasal edema 5. Skin - atopic dermatitis 6. Concurrent respiratory infection - viral respiratory illness, sinusitis

Answer 28

Asthma