Obstructive Lung Diseases Full Presentation: Kinder Flashcards
Case 1
- 40 yo male presents with gradual onset of dyspnea, cough, and wheezing over the last 2-3 years.
- Family History is remarkable for a father deceased at age 53 with cirrhosis.
- Social History: 20 pack year history of tobacco use
- Physical Examination: decreased breath sounds to auscultation, lungs are hyperinflated by percussion, peripheral cyanosis and clubbing of the digits
- Pulmonary Function Testing
- FEV1 1.06 L 36% of predicted
- FEV1/FVC 38%
- No significant improvement with bronchodilator treatment
- Pick the letter that best describes this patient’s disorder
A) Sweat testing confirms diagnosis
B) Purified protein derivative skin testing will confirm diagnosis
C) This disorder is caused by an imbalance in neutrophil elastase
D) Sputum cytology will confirm diagnosis
E) Acid fast sputum stain will confirm diagnosis
Case 1
•Pick the letter that best describes this patient’s disorder
A) Sweat testing confirms diagnosis
B) Purified protein derivative skin testing will confirm diagnosis
C) This disorder is caused by an imbalance in neutrophil elastase
D) Sputum cytology will confirm diagnosis
E) Acid fast sputum stain will confirm diagnosis
Alpha-1 Antitrypsin Deficiency
- 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
Case 2
- 19 year old male presents to the emergency room in acute respiratory distress. He has had a productive cough for several days
- Past Medical History: Frequent bouts of sinusitis, hospital admission 2 years ago with pneumonia, and meconium ileus at birth
- Physical Examination: Oxygen Saturation 93% on 2L NC, nostril flaring, subcostal retractions, wheezes, rhonchi, and clubbing of the fingers
- Pulmonary Function Testing with moderate airway obstruction.
- Which statement is true regarding this disorder?
A) B Vitamin Malabsorption is common in this disorder.
B) 95% of males with this disorder are sterile.
C) This disease is characterized by non-caseating granulomas
D) This is an autosomal dominant disorder
E) All siblings will be gene carriers
Case 2
- Pulmonary Function Testing with moderate airway obstruction.
- Which statement is true regarding this disorder?
A) B Vitamin Malabsorption is common in this disorder.
B) 95% of males with this disorder are sterile.
C) This disease is characterized by non-caseating granulomas
D) This is an autosomal dominant disorder
E) All siblings will be gene carriers
Cystic Fibrosis
- 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
•
Lung Manifestations in CF
- 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
Pancreatic Manifestations of CF
- Exocrine pancreatic insufficiency can lead to impaired growth
- Signs of malabsorption include bulky, foul smelling stools and flatulence.
- Malabsorption of fat soluble vitamins occur.
Liver Manifestations of CF
- Hepatomegaly
- Splenomegaly
- Hematemesis secondary to esophageal or gastric varices from portal hypertension
Sweat Gland in CF
- Failure of chloride absorption from the lumen into the ductal lining cell.
- Marked elevation of chloride and sodium in sweat
Vas deferens in CF
•Almost all males are sterile
Other clinical manifestations in CF
- 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
Cystic Fibrosis Diagnosis
- 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
Pulmonary function testing in CF
- Obstruction
Chest x-ray in CF
- Hyperinflation
- Bronchiectasis
Oral Antibiotics in CF
- 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
•
IV antibiotics in CF
- 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
IV antibiotics in CF
Part II
- 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
•
Inhaled antibiotics in CF
- Tobramycin 300 mg inhaled twice daily in 28 day cycle
- Aztreonam 75 mg inhaled three times daily in 28 day cycle
Other CF Treatments
- Pancreatic enzymes, vitamin supplementation, bronchodilators, hypertonic saline inhalation, dornase alfa(rhDNase), ibuprofen, oxygen, bronchodilators, lung transplant
- Chest percussion and postural drainage
Case 3
- 55 year old male with a history of chronic obstructive pulmonary disease complains of increased shortness of breath, dyspnea with minimal exertion, and decreased exercise tolerance.
- Past Medical History : Multiple hospital admissions for COPD exacerbations, intubated last admit
- Social History: 40 pack year history of tobacco use
- Medications: maximal doses of inhaled steroid, salmeterol, and tiotropium. He frequently has been treated with oral antibiotics and oral steroids.
- Which of the following Pulmonary Function Test results are most likely in this patient?
A) Decreased FEV1/FVC
B) Decreased functional residual capacity
C) Decreased total lung capacity
D) Increased FEV1
E) Increased FEV1/FVC
Case 3
•Which of the following Pulmonary Function Test results are most likely in this patient?
A) Decreased FEV1/FVC
B) Decreased functional residual capacity
C) Decreased total lung capacity
D) Increased FEV1
E) Increased FEV1/FVC
Chronic Obstructive Pulmonary Disease
- 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
Lung Mechanics in COPD
- 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
COPD History
- Current or past cigarette use
- Dyspnea with slow progression
- History of acute bronchitis
- History of a chronic cough
- Sputum production
- Wheezing
Physical Findings COPD
- Barrel Chest
- Prolonged expiratory phase
- Accessory muscle use
- Low Diaphragm
- Distant Heart Sounds
- Diminished Breath Sounds
- Rhonchi
- Wheezing
Physical Findings COPD
part 2
- Cyanosis
- Pedal Edema
- Distended Jugular Veins
- Hepatic congestion
- Cachexia
- “Blue Bloaters”
- “Pink Puffers”
Pulmonary Function Testing in COPD
Stage and Severity
- I: Mild
- FEV1/FVC < 70%
- FEV1/FVC = 80% of predicted
- II: Moderate
- FEV1/FVC < 70%
- 50% <= FEV1 < 80% of predicted
- III: Severe
- FEV1/FVC < 70%
- 30% <= FEV1 < 50% of predicted
- IV: Very Severe
- FEV1/FVC <70%
- < 30% of predicted or FEV1 < 50% of predicted plus chronic respiratory failure
Chest x-ray in COPD
- Hyperinflation
- Flattened Diaphragms
- Increased restrosternal space
- Bullae
- Can be normal in mild to moderate COPD
Differential Diagnosis of COPD
- Asthma
- Bronchiectasis
- Bronchiolitis obliterans
Emphysema
- 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
Chronic Bronchitis
•Enlargement of bronchial mucous glands and increased epithelial goblet cell production leads to cough and increased mucous production.
Case 4
- 45 year old female with a history of asthma complains of a daily cough and increased dyspnea. She now wakes up 2 or 3 nights a week with symptoms. Her current medicine is a medium dose inhaled steroid and albuterol inhaler as needed.
- Physical Examination: Pulse 80, Respiratory Rate 16 Lung examination is remarkable for bilateral expiratory wheezing.
- How would you categorize this patient’s asthma?
A) Intermittent asthma
B) Mild persistent asthma
C) Moderate persistent asthma
D) Severe persistent asthma
Case 4
•How would you categorize this patient’s asthma?
A) Intermittent asthma
B) Mild persistent asthma
C) Moderate persistent asthma
D) Severe persistent asthma