Respiratory 2

Question 1

What results in hypoxia secondary to a diffusional defect?

Answer 1

restrictive pulmonary disease

-develops in edema and fibrosis of alveolar walls

Question 2

Restrictive pulmonary disease is characterized by damage to alveolar walls with three main phases of reaction in the lung

Answer 2

1 hyaline membranes in alveolar sacs-exudation of fibrin-rich edema fluid mixed with the cytoplasmic and lipid remnants of necrotic epithelial cells

2 edema and inflammation of the interstitium

3 fibrosis in the interstitium

Question 3

What produces acute respiratory distress syndrome (ARDS)?

Answer 3

Diffuse Alveolar damage (DAD) to lung alveolar walls leading to widespread systemic metabolic derrangements

(increase in alveolar capillary permeability causing leakage of fibrin-rich edema fluid into alveoli)

Question 4

ARDS Phase 1: Acute Exude

Answer 4

Exudation of fibrin-rich edema fluid mixed with the cytoplasmic and lipid remnants of necrotic epithelial cells, forms hyaline membranes within alveolar spaces

Question 5

ARDS Phase 2: Late Organization

Answer 5

Cell proliferation and fibrosis

1 congested, impaired ventilation
2 Regeneration of type II alveolar lining cells and organization of hyaline membranes with pulmonary fibrosis
3 Interstitial fibrosis with thickening of alveolar walls or fibrous obliteration of alveolar spaces

Question 6

ARDS treatment

Answer 6

• mortality reduced to 40% with treatment

- continous positive airway pressure ventilation and intensive support of cardiac, circulatory, and renal function

Question 7

What causes chronic interstitial lung disease? (inhaled)

Answer 7

inhaled

1 extrinsic allergic alveolitis immune reaction to inhaled organic dusts
2 pneumoconiosis: inhaling industrial (mineral) dustsq

Question 8

What causes chronic interstitial lung disease? (intrinsic disease)

Answer 8

intrinsic disease

1 idiopathic interstitial pneumonitis

2 non-organ specific autoimmune CT diseases
(atypical pneumonias»Chlamydia, Mycoplasma

3 Sarcoidosis

Question 9

What causes extrinsic allergic alveolitis (hypersensitivity pneumonitis)

[Restrictive Pulmonary Disease]

Answer 9

immune reaction in the lung to inhaled antigens

1 animal : proteins in bird droppings

2 microbial: contaminated vegetable-derived material

or actinomyces and fungi that colonize rotting crops

(e.g.: hay, compost, sugar cane, maple bark)

Question 10

What causes Coal Worker’s Pneumoconiosis?

Answer 10

inhalation of coal dust which contains both carbon and silica

Question 11

What the two types of coal worker’s pneumonoconiosis?

Answer 11

1. simple coalworker’s pneumoconiosis

2. progressive massive fibrosis

Question 12

What is progressive massive fibrosis (PMF) surrounded by

Answer 12

irregular emphysema

Question 13

What is the histopathology of silicosis like?

Answer 13

silica particles with nodules under polarized light

Question 14

What complication is associated with silicosis?

Answer 14

development of TB due to impaired local defenses consequence of silica in macrophages (silicotuberculosis)

Question 15

What is Asbestosis?

Answer 15

Insidious feature is the long latent period often seen of up to 50 years between exposure and onset of clinical disease

Question 16

What occurs when Asbestosis progresses with a restrictive defect in the late stages?

Answer 16

pulmonary HTN and cor pulmonale

Question 17

Asbestos bodies within macrophages= Ferruginous Bodies

Answer 17

long, thin fibers coated with hemosiderin and protein to form yellow brown filaments with a beaded or drumstick pattern

Question 18

What can result in amrked predisposition to bronchogenic carcinoma and to malignant mesotheloma of the pleura or peritoneum?

Answer 18

Asbestosis

Question 19

Cigarette smoking and what…….. further increases the risk of bronchogenic carcinoma

Answer 19

Asbestosis

Question 20

Sarcoidosis

Answer 20

1 Progressive Granulomatous lung disease

2 Histiocytic and giant cell inflammation

Question 21

What are other granulomatous lung diseases besides sarcoidosis?

Answer 21

1 Tuberculosis
2 Granulomatous Vasculitis
3 Fungal infection s(e.g. histoplasmosis)
4 Inhaled foreign bodies (e.g. pneumoconioses)

Question 22

Clinical features of Sarcoidosis

Answer 22

1 shadowing on chest radiograph with enlargement of hilar lymph nodes
2 eye, associated glands, and salivary glands involved in up to 50%

Question 23

Prognosis of sarcoidosis

Answer 23

approximately 70% recover via steroid treatment

• 20% have permanent loss of some lung or eye movement
• 10-15 % fatality (pulmonary fibrosis and cor pulmonale)

Question 24

What affects interstitium of lungs, causing fibrosis?

Answer 24

restrictive pulmonary disease

Question 25

Extrinsic allergic alveolitis is due to hypersensitivity to

Answer 25

inhaled allergens (e.g. farmer's lung)

Question 26

What is the MOST COMMON CAUSE OF DEATH from neoplasia in industrialized nation?

Answer 26

Carcinoma of the lung (lung cancer)

Question 27

Hematogenous Lung cancer spread

Answer 27

Main sites are brain, bone liver, and adrenal glands | *Bone sites esp. are ribs, vertebrae, humeri, and femora with pain or pathological fracture

Question 28

Squamous cell carcinoma of the lung

Answer 28

1 most common in males 2 usually central and close to the carina with feature related to bronchial obstruction

Question 29

What is the most highly malignant of lung cancers?

Answer 29

Small cell anaplastic carcinoma of lung 1 bronchial epithelium but differentiates into neuroendocrine cells 2 ectopic hormone (e.g. ACTH) production leads to paraneoplastic syndrome

Question 30

What are local symptoms of lung cancer?

Answer 30

Cough (80% infection distal to airway blocked by tumor)

Question 31

Lung cancer metastasis

Answer 31

1 frequent presenting sign present in about 70% 2 hematogenous spread -pathological fracture (bone metastasis) -leuko-erythroblastic anemia (extensive bone marrow replacement) -CNS symptoms (brain mets) -Hepatomegaly and jaundice (liver metastasis)

Question 32

What are ways of local spread during lung cancer metastasis?

Answer 32

intra-thoracic nodes

Question 33

Intrathoracic nodes of lung cancer metastasis

Answer 33

pancoast tumor- superior sulcus tumor at apex of the lung; often with invasion of cervical sympathetic chain leading to Horner's syndrome

Question 34

Horner's syndrome

Answer 34

Ptosis, enopthalmos, small pupil (miosis), lack of sweating [anhydrosis] on ipsilateral face

Question 35

Lung cancer staging and histopathological type

Answer 35

1 determine outcome and likely response to treatment (e.g. squamous cell carcinoma detected at early stage (slow growth and operable) vs. small cell tumors (metastasis and poorest prognosis) 2 survival is better for early stage disease except for small cell carcinoma

Question 36

Lung cancer prognosis

Answer 36

very senstive to radiotherapy and chemotherapy but survival poor 1 complete local response in 30% of cases 2 median survival = 11 mos. 3 one year survival =45%

Question 37

coin lesions

Answer 37

1 rounded solitary radiopaque lesion on a chest radiograph | 2 1/3 cases (in persons over age 50) caused by carcinoma of the lung

Question 38

Common causes of coin lesions

Answer 38

``` 1 primary bronchial or lung carcinoma 2 metastatic tumor (esp. kidney) -bronchial hamartoma -carcinoid tumor 3 granulomatous inflammation (e.g. TB) 4 Lung Abscess ```

Question 39

Uncommon causes of coin lesions

Answer 39

1 pulmonary cysts 2 pulmonary cysts 3 fungal mycetoma 4 vascular malformations

Question 40

Lung cancer summary

Answer 40

Overall 5-year survival= 5-30%, dependent on type and stage, median approximately 15%

Question 41

Pleural fluid types

Answer 41

1 pus-empyema; due to infection 2 blood-hemothorax; due to trauma or surgery 3 chylo-thorax (lymphatic fluid) - leakage from the thoracic duct of chyle (lymph fluid) 4 transudate= low protein fluid due to movement of excess fluid through normal vessel walls (e.g. high hydrostatic pressure in cardiac failure)

Question 42

pleural fluid types

Answer 42

1 exudate: high protein fluid (fibrin) due to movement of fluid through damaged vessel walls (e.g. tumor, infection, infarction) 2 air-pneumothorax 3 fluid effusion= transudate or exudate; caused by several diseases

Question 43

What pathology of the pleura may have a latent period of 25 to 45 years?

Answer 43

malignant mesothelioma

Question 44

How do we diagnose a malignant mesothelioma?

Answer 44

pleural biopsy

Question 45

What causes hyaline membrane disease (neonatal respiratory distress syndrome)?

Answer 45

deficiency of surfactant in the lungs as a result of immaturity

Question 46

What is the histopathology of pediatric lung disease?

Answer 46

hyaline membranes

Question 47

Pediatric lund disease has 50% mortality if weight is...

Question 48

Cystic Fibrosis

Answer 48

Molecular Pathogenesis 1 defective function in a membrane chloride channel epithelial cells 2 decreased release of sodium and water to liquify mucus 3 bronchi and bronchioles become obstructed

Question 49

4 primary problems of cystic fibrosis

Answer 49

1 repeated infections 2 bronchiectasis and hemoptysis 3 hyperinflation of lungs due to air trapping and pneumothorax 4 hypoxia, scarring, and destruction of the pulmonary vascular bed leads to pulmonary hypertension and cor pulmonale

Question 50

What is the median survival for patients with cystic fibrosis?

Answer 50

30 years

Question 51

What is an acute form of diffuse alveolar damage casued by severe sepsis and shock

Answer 51

ARDS

Question 52

What is the prototype pattern of initial response in the lung

Answer 52

diffuse alveolar damage response in the lung

Question 53

What leads to honeycomb lung?

Answer 53

progressive pulmonary interstitial fibrosis

Question 54

What leads to idiopathic progressive fibrosis of lung interstitium ?

Answer 54

Idiopathic interstitial pneumonitis leads

Question 55

what exhibits fibrosis caused by reactions to inhaled mineral dusts?

Answer 55

pneumoconioses

Question 56

What has simple and progressive massive fibrosis forms?

Answer 56

coalworker's pneumoconiosis

Question 57

What predisposes to interstitial fibrosis, lung cancer, and mesothelioma?

Answer 57

asbestos

Question 58

What causes interstitial fibrosis

Answer 58

Sarcoidosis and autoimmune CT diseases