Pulmonary Pathology I

Question 1

Pulmonary Hypoplasia

Description, Causes (4) and Complication

Answer 1

Bilateral decreased development of lungs

Diaphragmatic hernia (decreased space)
Oligohydramnios (impaired inhalation)
Tracheal Stenosis (impaired inhalation)
Chest motion disorders (impaired inhalation)

High infant mortality rate

Question 2

Foregut Cysts

Description and Complications (3)

Answer 2

Outpouchings of foregut on mediastinum and hilum

Rupture
Airway compression
Infection

Question 3

Congenital Pulmonary Adenomatoid Malformation (CPAM)

Description and Complications (3)

Answer 3

Arrested development of tissue forming intrapulmonary cystic masses
(connected to airway and blood supply)

Fetal hydrops
Pulmonary hypoplasia
Infection

Question 4

Pulmonary Sequestrations

Description (3) and Intralobar vs Extralobar (4)

Answer 4

Nonfunctioning lung tissue forming accessory lung bud
Not attached to tracheal tree, has own blood supply

Intra: Lack of airway makes susceptible to infection

Extra: Has own vessels, pleura and airway
Present with other congenital malformations

Question 5

Atelectasis

Types with Descriptions (3)

Answer 5

Resorption
Resorption of air distal to obstruction

Compression
Material in pleural cavity compresses lungs (effusion)

Contraction
Fibrosis restricting pleura or lung

Question 6

Pulmonary Edema

Types (3) and Specific Causes

Answer 6

Hemodynamic
   Pushing Out (LHF)
   Leaking Out (Decreased oncotic pressure)

Alveolar Injury
Infection, Smoking

Unsure Mechanism
Neurogenic, High Altitude

Question 7

Acute Respiratory Distress Syndrome (ARDS)

Pathogenesis (4), Presentation (4)

Answer 7

Endothelium activation
Neutrophil Influx
Fluid leakage
Cell damage

Acute onset
Hypoxemia
Bilateral infiltrates
Non-cardiac in nature

Question 8

ARDS Stages (3)

Answer 8

Exudative
edema, hyaline membranes, neutrophils

Proliferative
fibroblasts proliferate

Fibrotic or Resolution
Extensive fibrosis/alveolar damage or restored cells

Question 9

Neonatal Respiratory Distress Syndrome

Pathogenesis and Presentation (2)

Answer 9

Underdeveloped lungs without type II pneumocytes have inadequate surfactant

Progressive atelectasis
Hypoxemia

Question 10

Acute Interstitial Pneumonia

Presentation (4) and Unique Feature

Answer 10

Acute onset
Hypoxemia
Bilateral infiltrates
Non-cardiac in nature

Cause unknown

Question 11

Chronic Bronchitis

Mechanism (3), Diagnosis, Presentation (4) and Complications (2)

Answer 11

Mucus gland hyperplasia leading to infection and inflammation

Persistent productive cough for 3+ months for 2 consecutive years

Cyanosis
Edema
Increased hemoglobin
Rhonchi and wheezing

Bronchiectasis
Squamous metaplasia

Question 12

Emphysema
Mechanism (2), Diagnosis (2), Presentation (3)

Answer 12

Dilated alveoli from constricted terminal ducts
Can be from chronic bronchitis spreading distally

Enlarged clear lungs on X-ray and barrel chest

Severe SOB
Quiet chest on auscultation
Old and thin patient

Question 13

Alpha-1 Antitrypsin Deficiency

Genetics, Pathophysiology and Complication

Answer 13

Encoded by Proteinase inhibitor (Pi) on chromosome 14

AAT gets trapped in liver leaving lungs vulnerable to neutrophil elastase damage

Panacinar basilar emphysema

Question 14

Asthma

Description and Presentation (4)

Answer 14

Recurrent reversible airway obstruction

Bronchoconstriction
Mucus secretion
Increased vascular permeability
Inflammatory cell recruitment

Question 15

Asthma

Types (3) Triggers, and Descriptions

Answer 15

Atopic: Hypersensitive Th2 cells triggered by allergens

Non-Atopic: Exercise, Cold and Infection triggers

Asipirin Sensitive: Cox-1 inhibitors trigger
Associated with nasal polyps and recurrent rhinitis

Question 16

Asthma

Complications (2)

Answer 16

Airway Remodeling: irreversible fibrosis causing decreased response to bronchodilators and corticosteroids

Status Asthmaticus: thick mucus that completely occludes bronchus
(see Charcot Leyden Crystals)

Question 17

Bronchiectasis

Pathogenesis (3) and Presentation (3)

Answer 17

Dilation of bronchi/bronchioles
From smooth muscle and elastin destruction
Caused by chronic necrotizing infections

Persistent cough with foul-smelling bloody sputum
Dyspnea and orthopnea
Hemoptysis

Question 18

Bronchiectasis

Causes (3) with Descriptions

Answer 18

Cystic Fibrosis: CFTR gene defect causing thick mucus that increases pyogenic infections

Primary Ciliary Dyskinesia: dynein arm of mT defective leading to recurrent infections

Allergic Bronchopulmonary Aspergillosis: exaggerated aspergillus response
Diagnosed with IgE/skin test

Question 19

Pneumoconiosis

Types (3), Exposure and Complications

Answer 19

Coal Worker’s: Anthracosis, Coal Macules and Progressisve fibrosis

Silicosis: Miners and concrete workers exposed
Insidious onset of fibrosis and cancer

Asbestosis: insulation, shipyard, paper and oil workers
Interstitial and pleural disease, mesothelioma
See candlewax drippings on pleura (hyaline)