Fa: Respiratory - Pathology - 2

Front 1

What causes Neonatal Respiratory Distress Syndrome?

Back 1

Surfactant deficiency → ↑ surface tension → alveolar collapse

Front 2

What can predict whether a neonate will have neonatal respiratory distress syndrome?

Back 2

Lecithin:Sphingomyelin ratio <1.5 in amniotic fluid

Front 3

What is a potential complication of neonatal respiratory distress syndrome?

Back 3

- Persistently low O2 tension → risk of PDA
- Therapeutic supplementation of O2 can result in RETINOPATHY of prematurity and BROCHOPULMONARY DYSPLASIA

Front 4

What are the risk factors for neonatal respiratory distress syndrome?

Back 4

- Prematurity
- Maternal diabetes (due to ↑ fetal insulin)
- C-section delivery (↓ release of fetal glucocorticoids)

Front 5

How do you treat a neonate at risk for neonatal respiratory distress syndrome?

Back 5

Give mother steroids before birth to stimulate surfactant production

Front 6

How do you treat an infant with neonatal respiratory distress syndrome?

Back 6

Artificial Surfactant

Front 7

What can cause acute respiratory distress syndrome?

Back 7

- Trauma
- Sepsis
- Shock
- Gastric aspiration
- Uremia
- Acute pancreatitis
- Amniotic fluid embolism

Front 8

What changes occur in acute respiratory distress syndrome?

Back 8

- Diffuse alveolar damage → ↑ alveolar capillary permeability → protein-rich leakage into alveoli and non-cardiogenic pulmonary edema (normal PCWP)
- Formation of intra-alveolar hyaline membrane

Front 9

What does this x-ray show?

Back 9

Acute Respiratory Distress Syndrome
- Near complete opacification of the lungs
- Obscured cardiomediastinal silhouette

Front 10

What does this histology show?

Back 10

Acute Respiratory Distress Syndrome
- Alveolar fluid (clear and frothy)
- Thickened hyaline membranes (pink)

Front 11

What causes the initial damage in acute respiratory distress syndrome?

Back 11

- Release of neutrophilic substances toxic to alveolar wall
- Activation of coagulation cascade
- Oxygen-derived free radicals

Front 12

What is the normal FEV1/FVC ratio?

Back 12

80%

Front 13

What is the FEV1/FVC ratio in obstructive lung disease?

Back 13

< 80%

Front 14

What is the FEV1/FVC ratio in restrictive lung disease?

Back 14

≥ 80%

Front 15

Which lung disease has increased lung volumes (↑ TLC, ↑ FRC, and ↑ RV)?

Back 15

Obstructive lung disease

Front 16

Which lung disease has decreased lung volumes (↓ TLC, ↓ FRC, and ↓ RV)?

Back 16

Restrictive lung disease

Front 17

How does the change in FEV1 and FVC compare in obstructive vs restrictive lung disease?

Back 17

In both obstructive and restrictive disease, FEV1 and FVC are reduced
- In obstructive, however, FEV1 is more dramatically reduced compared to FVC, resulting in ↓ FEV1/FVC ratio

Front 18

What is the normal pulmonary artery pressure?

Back 18

10-14 mmHg

Front 19

What is the definition of pulmonary hypertension?

Back 19

≥ 25 mmHg at rest

Front 20

What are the consequences of pulmonary hypertension?

Back 20

- Arteriosclerosis
- Medial hypertrophy
- Intimal fibrosis of pulmonary arteries

Front 21

What causes Primary Pulmonary Hypertension? Prognosis?

Back 21

- Inactivating mutation in BMPR2 gene (normally functions to inhibit vascular smooth muscle proliferation)
- Poor prognosis

Front 22

What causes Secondary Pulmonary Hypertension? Prognosis?

Back 22

- COPD
- Mitral stenosis
- Recurrent thromboemboli
- Auto-immune disease
- L → R shunt
- Sleep apnea
- Living at high altitude

Front 23

How does COPD affect the pressure in the pulmonary circulation?

Back 23

Destroys lung parenchyma → 2° pulmonary hypertension

Front 24

Which valvular problem can affect the pressure in the pulmonary circulation? How?

Back 24

Mitral Stenosis → ↑ Resistance → ↑ Pressure → 2° pulmonary hypertension

Front 25

How do thromboemboli affect the pressure in the pulmonary circulation?

Back 25

Recurrent thromboemboli → ↓ cross-sectional area of pulmonary vascular bed → 2° pulmonary hypertension

Front 26

Which auto-immune diseases affect the pressure in the pulmonary circulation? Implications?

Back 26

Systemic Sclerosis
- Inflammation → Intimal Fibrosis → Medial Hypertrophy → 2° Pulmonary Hypertension

Front 27

What type of cardiac shunt can affect the pressure in the pulmonary circulation? Implications?

Back 27

Left-to-Right Shunt → ↑ shear stress → endothelial injury → 2° pulmonary hypertension

Front 28

How does sleep apnea affect the pressure in the pulmonary circulation?

Back 28

Hypoxic vasoconstriction → 2° pulmonary hypertension

Front 29

How does living at a high altitude affect the pressure in the pulmonary circulation?

Back 29

Hypoxic vasoconstriction → 2° pulmonary hypertension

Front 30

What is the course of having pulmonary hypertension?

Back 30

Pulmonary hypertension → severe respiratory distress → cyanosis and RVH → death from decompensated cor pulmonale

Front 31

What happens in sleep apnea? Consequences?

Back 31

Repeated cessation of breathing >10 seconds during sleep → disrupts sleep → daytime somnolence

Front 32

What happens to the PaO2 during the day and night in a patient with sleep apnea?

Back 32

- Normal PaO2 during day
- Nocturnal hypoxia

Front 33

What are the complications of sleep apnea?

Back 33

Nocturnal hypoxia → systemic / pulmonary HTN, arrhythmias (atrial fibrillation / flutter), and sudden death

Front 34

What are the types of sleep apnea? How do they differ?

Back 34

- Central sleep apnea: no respiratory effort
- Obstructive sleep apnea: respiratory effort against airway obstruction, associated with obesity and loud snoring

Front 35

How do you treat sleep apnea?

Back 35

- Weight loss
- CPAP
- Surgery

Front 36

How does sleep apnea affect erythropoiesis?

Back 36

Hypoxia → ↑ EPO release → ↑ erythropoiesis

Front 37

What variation of sleep apnea is seen in obese patients?

Back 37

Obesity Hypoventilation Syndrome:
- Obesity (BMI ≥ 30 kg/m2) → hypoventilation → ↓ PaO2 and ↑ PaCO2 during waking hours

Front 38

In what lung pathology are there the following findings:
- Breath sounds: ↓
- Percussion: dull
- Fremitus: ↓
- Tracheal deviation: -

Back 38

Pleural Effusion

Front 39

In what lung pathology are there the following findings:
- Breath sounds: ↓
- Percussion: Dull
- Fremitus: ↓
- Tracheal deviation: Toward side of lesion

Back 39

Atelectasis (bronchial obstruction)

Front 40

In what lung pathology are there the following findings:
- Breath sounds: ↓
- Percussion: hyperresonant
- Fremitus: ↓
- Tracheal deviation: -

Back 40

Spontaneous pneumothorax

Front 41

In what lung pathology are there the following findings:
- Breath sounds: ↓
- Percussion: hyperresonant
- Fremitus: ↓
- Tracheal deviation: away from side of lesion

Back 41

Tension pneumothorax

Front 42

In what lung pathology are there the following findings:
- Breath sounds: bronchial breath sounds; late inspiratory crackles
- Percussion: dull
- Fremitus: ↑
- Tracheal deviation: -

Back 42

Consolidation (lobar pneumonia, pulmonary edema)

Front 43

In what lung pathologies are there decreased breath sounds?

Back 43

- Pleural effusion
- Atelectasis (bronchial obstruction)
- Spontaneous pneumothorax
- Tension pneumothorax

Front 44

In what lung pathologies are there bronchial breath sounds and late inspiratory crackles?

Back 44

Consolidation (lobar pneumonia or pulmonary edema)

Front 45

In what lung pathologies are the lungs dull to percussion?

Back 45

- Pleural effusion
- Atelectasis (Bronchial obstruction)
- Consolidation (lobar pneumonia or pulmonary edema)

Front 46

In what lung pathologies are the lungs hyperresonant to percussion?

Back 46

- Spontaneous pneumothorax
- Tension pneumothorax

Front 47

In what lung pathologies do the lungs have decreased fremitus?

Back 47

- Pleural effusion
- Atelectasis (bronchial obstruction)
- Spontaneous pneumothorax
- Tension pneumothorax

Front 48

In what lung pathologies do the lungs have increased fremitus?

Back 48

Consolidation (lobar pneumonia or pulmonary edema)

Front 49

In what lung pathologies is there a tracheal deviation toward the side of the lesion?

Back 49

Atelectasis (bronchial obstruction)

Front 50

In what lung pathologies is there a tracheal deviation away the side of the lesion?

Back 50

Tension pneumothorax

Front 51

What is the leading cause of cancer death?

Back 51

Lung cancer

Front 52

What is the classic presentation of lung cancer?

Back 52

- Cough
- Hemoptysis
- Bronchial obstruction
- Wheezing
- Pneumonic "coin" lesion on x-ray film or non-calcified nodule on CT

Front 53

What finding on x-ray is characteristic of lung cancer?

Back 53

Pneumonic "coin" lesion

Front 54

What finding on CT is characteristic of lung cancer?

Back 54

Non-calcified nodule

Front 55

What is more common: primary neoplasms or metastases to the lungs?

Back 55

In the lung, metastases (usually multiple lesions) are more common than 1° lesions

Front 56

What are the most common sites that metastasize to the lungs?

Back 56

Cancer of:
- Breast
- Colon
- Prostate
- Bladder

Front 57

What are the most common sites of metastases from the lungs?

Back 57

- Adrenals
- Brain
- Bone (pathologic fracture)
- Liver (jaundice, hepatomegaly)

Front 58

What are the types of lung cancer?

Back 58

- Adenocarcinoma
- Squamous cell carcinoma
- Small cell (oat cell) carcinoma
- Large cell carcinoma
- Bronchial carcinoid tumor

Front 59

What types of lung cancer are located peripherally?

Back 59

- Adenocarcinoma
- Large cell carcinoma

- Bronchial carcinoid tumor (either peripheral or central)

Front 60

What types of lung cancer are located centrally?

Back 60

- Squamous cell carcinoma
- Small cell (oat cell) carcinoma

- Bronchial carcinoid tumor (either peripheral or central)

Front 61

What is the most common type of lung cancer (except for metastases)?

Back 61

Adenocarcinoma

Front 62

What is the most common type of lung cancer in non-smokers?

Back 62

Adenocarcinoma

Front 63

What genetic changes are associated with Lung Adenocarcinoma?

Back 63

Activating mutations:
- k-ras
- EGFR
- ALK

Front 64

What is a specific physical sign of Lung Adenocarcinoma?

Back 64

Hypertrophic osteoarthropathy (clubbing)

Front 65

Which subtype of lung adenocarcinoma is associated with hazy infiltrates on CXR (similar to pneumonia)? Why this appearance? Prognosis?

Back 65

Bronchioloalveolar Subtype of Adenocarcinoma:
- Tumor grows along alveolar septa giving an apparent "thickening" to the alveolar walls
- Excellent prognosis

Front 66

What type of cancer does this show? What are the characteristics of it that tell you that?

Back 66

Squamous Cell Carcinoma
- Keratin pearls and intercellular bridges
- Sheets of large dysplastic squamous cells (arrows) surrounding dark, pink keratin pearls (lower right)

Front 67

What is the location of lung Squamous Cell Carcinoma? Other characteristics indicative of it?

Back 67

- Central location
- Hilar mass arising from bronchus
- Cavitation, Cigarettes, and HyperCalcemia

Front 68

What type of lung cancer is associated with hypercalcemia? How?

Back 68

Squamous Cell Carcinoma
- Hypercalcemia because tumor produces PTHrP

Front 69

What type of cancer does this show? What are the characteristics of it that tell you that?

Back 69

Small Cell (Oat Cell) Carcinoma
- Sheets of dark purple tumor cells with nuclear molding, high mitotic rate, necrosis, and "salt and pepper" neuroendocrine-type chromatin
- Neoplasm of neuroendocrine Kulchitsky cells → small dark blue cells

Front 70

What type of lung cancer is known for producing certain substances that can cause seemingly unrelated symptoms? What substances?

Back 70

Small Cell (Oat Cell) Carcinoma:
- May produce ACTH or ADH
- May also produce Antibodies against pre-synaptic Ca2+ channels causing Lambert-Eaton Myasthenic Syndrome

Front 71

What genetic change is sometimes associated with Small Cell (Oat Cell) Carcinoma?

Back 71

Ampification of myc oncogenes common

Front 72

What is the prognosis of Small Cell (Oat Cell) Carcinoma? How do you treat it?

Back 72

- Undifferentiated → very aggressive
- Inoperable, treat with chemotherapy

Front 73

Which type of lung cancer is associated with pleomorphic giant cells?

Back 73

Large Cell Carcinoma

Front 74

What are the characteristics of a Large Cell Carcinoma of the lung? Location?

Back 74

- Peripheral locatoin
- Highly anaplastic undifferentiated tumor

Front 75

What is the prognosis for Large Cell Carcinoma? How do you treat?

Back 75

- Poor prognosis
- Less responsive to chemotherapy, removed surgically

Front 76

What type of lung cancer is composed of nests of neuroendocrine cells and is chromogranin A positive?

Back 76

Bronchial Carcinoid Tumor

Front 77

What is the prognosis for Bronchial Carcinoid Tumor? Symptoms?

Back 77

- Excellent prognosis, metastasis rare
- Symptoms usually due to mass effect
- Occasionally causes carcinoid syndrome (5-HT secretion → flushing, diarrhea, wheezing)

Front 78

What type of lung cancer is associated with Carcinoid Syndrome? What does that mean?

Back 78

Bronchial Carcinoid Tumor
- 5-HT secretion → flushing, diarrhea, wheezing

Front 79

Which types of lung cancer have a good prognosis?

Back 79

- Bronchioalveolar subtype of Adenocarcinoma
- Bronchial Carcinoid Tumor

Front 80

What is the name of the malignancy of the pleura? What is it associated with?

Back 80

Mesothelioma
- Associated with asbestosis

Front 81

What are the complications of a Mesothelioma?

Back 81

Results in hemorrhagic pleural effusion and pleural thickening

Front 82

What are the histologic signs of Mesothelioma?

Back 82

Psamomma bodies