Pulmonary Pathology Part II: Bronchiectasis, Cystic Fibrosis, Pneumoconioses, Sarcoidosis, P. E., Pulm HTN

Question 1

T or F? Pulmonary Hypertension
A. Can lead to cor pulmonale
B. M > F 2:1
C. Mean age of onset is 35 yrs
D. Main finding is very thickened arteriole wall

Answer 1

A. True. Cor pulmonale = Rt ventricular failure (enlargement) due to high BP in lungs
B. False. Affects women > men in a 5:1 ratio.
C. True

Question 2

What is the effect of pulmonary HTN on arterial system and blood flow?

Answer 2

Arterial system in the lungs becomes more resistant to blood flow –> increasing pressure within the pulmonary circulation –> limited blood flow

Question 3

Pulmonary HTN: Primary or Secondary?
A. Due to arterial wall thickening and obliteration of the lumen of medium and small pulmonary arteries
B. Wall thickening may be through entire arterial system, from pulmonary arteries to arterioles
C. Usually due to other cardiac or pulmonary conditions
D. Majority of Pulm HTN

Answer 3

A. Primary
B. Secondary
C. Secondary
D. Secondary

Question 4

How do thromboemboli cause secondary Pulm HTN?

Answer 4

Recurrent thromboemboli can cause P.H. due to alveolar damage and increased pulmonary vascular resistance.

Question 5

Describe the pathophysiology of primary and secondary pulm. HTN.
A. Damaged endothel cells release ____ and ____
B. Migration of _____ cells to ______
C. Endothelial dysfunction –> Diminished _______
D. Diminished _______ can cause further _______

Answer 5

A. cytokines and growth factors
B. smooth muscle; vessel walls
C. nitric oxide
D. oxygenation; vasoconstriction

Question 6

Risks associated with pulmonary HTN?

Answer 6

Alveolar hypoxemia can also result in endothelial damage and subsequent elevated pulmonary pressures.
A.R.D.S.
Sleep apnea
High altitudes--> pulmonary edema
Emphysema
Fenfluramine-phentermine (fen-phen)
Crotalariaor bush tea

Question 7

How difficult is Pulmonary HTN to treat? What are some of the treatments?

Answer 7

P.H. is extremely refractory to treatment. Death may occur within 1 to 5 years from the time of diagnosis.

Supplemental oxygen, vasodilator drugs, Iloprost (Viagra-like), Lung transplantation.

Question 8

What treatment for pulmonary HTN has largely proven unsuccessful?

Answer 8

Steroids

Question 9

What is the viscious cycle regarding pulmonary HTN?

Answer 9

Narrowing causes HTN, and HTN causes narrowing!

Question 10

Name the condition
A. Caused by inhalation of dust, chemicals or organic materials (coal, asbestos)
B. Restrictive lung dz
C. Dilatation of bronchus assoc w necrotizing inflamm

Answer 10

A. Pneumoconiosis
B. Pneumoconiosis,
C. Bronchiectasis

Question 11

The development of a pneumoconiosis is dependent on at least four factors:

Answer 11

1. Amount of dust retained in the lung and airways
2. Size and shape of the particulate matter
3. Particle solubility and reactivity
4. other irritants, in particular, tobacco use

Question 12

Name the condition
A. A condition in which LARGE bronchi are damaged and DILATED due to a variety of causes.
B. Autosomal recessive

Answer 12

A. Bronchiectasis

B. Cystic Fibrosis

Question 13

Name the condition
A.
B. mucus hyper-secretion & destruction of the muscle & elastic tissue 
C. Disorder of epith. cell ion transport b/c of CTFR gene
D. Etio: congenital, TB, bacteria/virus, autoimmune dz

Answer 13

A.
B. Bronchiectasis
C. Cystic fibrosis
D. Bronchiectasis

Question 14

Name the condition
A. Thick mucous & repeated infx –> chronic bronchitis and bronchiectasis
•
B.
C. Sx: cough, fever, copious foul purulent sputum.
D. Marked hyperplasia and hypertrophy of mucous glands.

Answer 14

A. Cystic fibrosis
B.
C. Bronchiectasis
D. Cystic fibrosis