Updated: 9/14/2017

Emphysema

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Introduction
  • A condition characterized by dilation of air spaces (with ↓ elasticity and ↑ compliance)
    • due to alveolar wall destruction
      • normally, elastin in the wall functions to keep alveoli open
    • capillaries are destroyed along with the alveoli
  • Causes
    • smoking
      • most common cause
    • α1-antitrypsin (AAT) deficiency
      • AD disorder (codominant)
        • MM phenotype is normal
        • ZZ phenotype results in disease
      • AAT normally inhibits the action of elastase
      • without AAT elastase is unchecked and destroys the elastic tissue of the alveoli  
      • as a result emphysema develops at early age
        • may also develop cirrhosis due to the inability to release an abnormal form of AAT from the liver resulting in hepatotoxicity 
Classification
  • Centriacinar 
    • dilated respiratory bronchiole
    • most common presentation of emphysema due to smoking
      • result of inhaled tobacco toxins arriving first in the respiratory bronchioles before traveling to the alveolus
    • most commonly in the upper lobes
      • result of upper lobes receiving exposure to smoke
  • Panacinar
    • dilated alveoli
    • most common presentation of AAT deficiency
      • also due to a functional AAT deficiency as a result of smoking
        • oxidants and inflammatory reaction of smoke can destroy AAT
        • smoking exacerbates effects of genetic AAT deficiency 
    • most commonly in the lower lobes
      • result of lower lobes recieving ↑ perfusion allowing more immune cells to traffic into the alveoli
  • Paraseptal
    • most commonly involves young, otherwise healthy males
    • does not obstruct the airway
      • associated with bullae
      • found near the pleura
      • increased risk for spontaneous pneumothorax
Presentation
  • Symptoms
    • dyspnea
    • classic pursed-lip breathing
      • results in increased airway pressure and prevents airway collapse during exhalation
  • Physical exam
    • decreased breath sounds on auscultation
    • increased anterior-posterior diameter (barrel chest)
    • hyperresonant to percussion
    • "pink puffer" (end-stage)
Imaging
  • Chest radiograph
    • increased AP diameter with flattened diaphragms 
    • hyperinflated lungs
    • loss of lung markings
    • elongated heart
Evaluation
  • Labs
    • ABG during exacerbation shows hypoxemia and acute respiratory acidosis
    • AAT shows no α-globin peak on electrophoresis
  • Pulmonary function tests
    • decreased FEV1 sec / FVC
    • increased TLC and RV
    • decreased diffusion capacity from destruction of capillaries
Treatment
  • Conservative 
    • smoking cessation
    • ambulatory O2 
  • Pharmacologic
    • bronchodilators 
      • for symptom improvement
    • inhaled steroids
      • reduce exacerbations
    • oral/IV steroids and antibiotics
      • for acute exacerbations
 

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Questions (7)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
Calculator

(M1.PL.85) A 28-year-old patient presents to the hospital complaining of progressively worsening dyspnea and a dry cough. Radiographic imaging is shown below. Pulmonary function testing (PFT's) reveals a decreased FEV1 and FEV1/FVC, but an increase TLC. The patient states that he does not smoke. Which of the following conditions is most consistent with the patients symptoms? Review Topic

QID: 100888
FIGURES:
1

Chronic bronchitis

0%

(0/13)

2

Alpha1-antitrypsin deficiency

85%

(11/13)

3

Pneumothorax

8%

(1/13)

4

Asthma

0%

(0/13)

5

Hypersensitivity pneumonitis

8%

(1/13)

M1

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PREFERRED RESPONSE 2
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(M1.PL.81) A patient with a1-antitrypsin deficiency is warned by his physician that his increasing dyspnea may be worsened by his continued cigarette smoking. Which of the following factors, released by both neutrophils and alveolar macrophages, is responsible for the patient's condition? Review Topic

QID: 100884
1

Major Basic Protein

4%

(1/24)

2

Antibodies against alpha-3 segment of collagen IV (COL4A3)

8%

(2/24)

3

Mucus

12%

(3/24)

4

Surfactant

0%

(0/24)

5

Elastase

58%

(14/24)

M1

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PREFERRED RESPONSE 5

(M1.PL.137) A 65-year-old male presented to his primary care physician with exertional dyspnea. The patient had a 30-year history of smoking one pack of cigarettes per day. Physical examination reveals a barrel-chested appearance, and it is noted that the patient breathes through pursed lips. Spirometry shows decreased FEV1, FVC, and FEV1/FVC. This patient’s upper lobes are most likely to demonstrate which of the following? Review Topic

QID: 100940
1

Panacinar emphysema

25%

(1/4)

2

Centriacinar emphysema

75%

(3/4)

3

Calcified nodule

0%

(0/4)

4

Hypersensitivity pneumonitis

0%

(0/4)

5

Uncalcified nodule

0%

(0/4)

M1

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PREFERRED RESPONSE 2

(M1.PL.45) A 50-year-old Caucasian male presents to the Emergency Department complaining of shortness of breath and unintentional weight loss over the past several months. On physical examination, the patient appears quite thin and breathes through pursed lips. Breath sounds are decreased in all lung fields. The patient's chest x-ray is provided in Figure A. Which of the following findings is expected on spirometry? Review Topic

QID: 100848
FIGURES:
1

Increased FEV1

10%

(1/10)

2

Decreased FEV1/FVC

90%

(9/10)

3

Decreased TLC

0%

(0/10)

4

Normal FEV1 but increased FVC

0%

(0/10)

5

Normal lung values

0%

(0/10)

M1

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PREFERRED RESPONSE 2

(M1.PL.87) A 60-year-old male presents to your office complaining of dyspnea on exertion. He reports smoking two packs of cigarettes per day for the past 25 years. A lung CT is shown in Figure A. Which of the following is LEAST likely to be involved in the pathogenesis of this patient's disease? Review Topic

QID: 100890
FIGURES:
1

Immunoglobulin A

60%

(3/5)

2

Proteinase 3

20%

(1/5)

3

Neutrophil elastase

20%

(1/5)

4

Proteases released by macrophages

0%

(0/5)

5

Matrix metalloproteinases

0%

(0/5)

M1

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