- Emphysema can be caused by congenital factors, smoking, or alpha 1-antitrypsin deficiency. It results in destruction of lung tissue and inability to exhale fully. - In emphysema, the air spaces in the lungs enlarge and the walls between them are destroyed. This reduces the surface area for gas exchange. - Chest x-rays show enlarged, hyperinflated lungs in emphysema patients. Lobectomy is often used to treat congenital lobar emphysema in infants.

2. Emphysema Causes:
 Congential (present at birth)
 Smoking and second hand smoke
 The results of Alpha 1-Antitrypsin deficiency
Emphysema is an obstructive disorder which air can enter the lungs
but the patient is unable to breathe out easily and as a result, over a
long period of time, air trapping begins and the chest wall will begin to
expand.

3. General Overview
What happens inside the lungs of an
Emphysema patient?
 Permanent enlargement & destruction
of the airspaces distal to terminal
bronchioles
 Destruction of the pulmonary
capillaries
 Weakening of the distal airways
(primarily the respiratory bronchioles)
Notice how the alveoli are plentiful and
 Bronchospasm (“smooth muscle well formed in the in the healthy lung
constriction of the bronchial airways”) , but in the lung with emphysema, the
 Hyperinflation of alveoli (“air trapping”) amount of alveoli are reduced and
enlarged thus reducing the surface area
of the lung…..

4. Gross anatomy of a lung with Gross anatomy of a healthy lung
emphysema
Note the clusters of dilated air spaces
which are conspicuous in the middle
and lower lobes of the right lung and
the lower lobe of the left lung. Both A healthy, functioning lung with no
lungs are markedly enlarged. apparent disease.

5. What are the x-ray findings of
emphysema?
 Lungs are large and hyperinflated.
 Signs of hyperinflation are low set
diaphragm, increased AP
diameter, vertical heart and
increased retrosternal air.
 Signs of hyperinflation can be seen
in emphysema, chronic bronchitis
and asthma. We can call it
emphysema only when
hyperinflation is associated with
blebs and paucity of vascular
markings in the outer third of the
film.

6. An emphysematous lung shows increased anteroposterior (AP)
diameter, increased retrosternal airspace, and flattened diaphragms
on lateral chest radiograph.
The thickness of the space
Increased retrosternal
between the ascending aorta
and the sternum is normally
airspace is an indicator of
no more than 2.5cm. hyperinflation of the lungs
Increased retrosternal and is usually due to
airspace is an indicator of emphysema.
hyperinflation of the lungs
and is usually due to
emphysema.
Source: Emphysematous Chest X-ray II

7. Congential Emphysema
“Also called infantile lobar emphysema, is a respiratory disease that occurs in infants
when air enters the lungs but cannot leave easily. “
 Half of the cases of congenital lobar  Etiology is unknown
emphysema occur in the first four  50% of the cases of CLE there is decreased
weeks of life, and three-quarters occur bronchial cartilage tissue. This defect produces a
ball valve effect with consequent overinflation.
in infants less than six months old.
 It is diagnosed by respiratory symptoms and a
 Congenital lobar emphysema is more chest x ray, which will show the over-inflation of
the affected lobe and may show a blocked air
common in boys than in girls. passage.
 Congential Emphysema is caused by an unknown Etiology……….
 Blocked airway passages may contribute to the disease………
 Prognosis is good in most patients if caught in time……….

8. Treatment
Lobectomy is the most common
form of treatment and has an 85%
success rate with compete cure.
Depending on the
symptoms, conservative measures
are sometimes taken, but these
may fail in the presence of inter-
current infections Child with Congenital Lobar Emphysema being
prep for a lobectomy

9. Congenital Lobar Emphysema or
CLE
Thirty month old male with
progressive respiratory distress
AP and lateral chest films from the
day of admission demonstrate
hyperinflation of the right upper
lobe.

10. Congenital Lobar Emphysema or
CLE
 The patient was taken emergently to
the operating room after his
respiratory decompensation.
 A rigid bronchoscopy was performed to
rule out the presence of a foreign body
before a thoracotomy was performed
for congenital lobar emphysema. No
airway foreign body was seen on
bronchoscopy. Mucosal edema and
thin white secretions were seen
throughout the airway.

11. Congenital Lobar Emphysema or
CLE
 A thoracotomy was then performed
through a standard right posterolateral
thoracotomy incision.
 Upon opening the chest, a very large
right upper lobe was encountered and
was allowed to herniate out through the
incision, thus decompressing the other
intrathoracic structures.
 The patient's respiratory status
improved immediately. The right upper
lobe was then resected.

12. A gross photograph showing the
emphysematous right upper lobe

13. A gross photograph showing the
emphysematous right upper lobe
Examination of the surgical specimen
revealed a lobe of lung with focal
hemorrhage and subpleural bullae.
Emphysematous change, bronchiolitis
with proliferation of bronchiolar
epithelium, and patchy interstitial
pneumonitis was seen.

14. Smoking and Emphysema

15. Number one cause of
COPD/Emphysema
SO YOU WANT TO
SMOKE???

16. So you want to
smoke?
 Over 4,000 various chemical compounds
 Inhaling smoke into the lungs ignites massive amounts of elastase into the lungs
rendering the available Alpha-1 Antitrypsin utterly useless.
 When the lungs are exposed to cigarette smoke, the body goes into a defense mode
resulting in macrophages (defense cells) to
release the elastin and collagen proteins thus
speeding the destruction of the patient’s lungs.
 Damage done by cigarette smoke also damages
the cilia, inhibiting the body’s ability to sweep
away dangerous particles out of the respiratory
tract.

17. Would you like some……….
 Hypertension
 Diabetes
 Dyslipidaemia (High blood cholesterol levels)
 Studies performed in dogs demonstrated a smoking-related reduction in arterial
flow and venous restriction
 Reduced blood flow has been documented in men who smoke leading to
possible………………………………..
ERECTILE DYSFUNCTION
With that cigarette?

18. Second Hand Smoke

19. Research is beginning to prove even more definite that
exposure to these second hand chemicals can be just as
harmful or even more harmful to an individual, even if the
person has never smoked a cigarette in their life.
The researchers found that almost one-third of the non-
smokers with high exposure to second hand smoke had
structural changes in their lungs similar to those found
in smokers.
“We interpreted those changes as early signs of
lung damage, representing very mild forms of
emphysema," said Wang. (Science Daily 2007)

20. Quitting is always the BEST option for yourself and
others around you

21. Alpha 1 Antitrypsin

22. What is alpha 1 Antitrypsin? Alpha 1 Antitrypsin Deficiency: A
 Alpha 1 antitrypsin deficiency is a Less Common Cause of Emphysema
hereditary condition that is passed
on from parents to their children
 This condition may result in serious
lung disease and or liver disease in
infants, children and adults
 Alpha 1 occurs when there is a
severe lack of protein in the blood
called Alpha-1 Antitrypsin (AAT)
that is mainly produced by the liver
Cross section of native lung with
alpha-1 anti-trypsin deficiency

23. What is the purpose of Alpha 1 The most common side effects of
Antitrypsin? AAT only related to the lungs:
 The sole purpose of AAT is to protect  Shortness of breath
the lungs from inflammation caused
 Wheezing chronic cough
by infection and inhaled irritants such
as cigarette smoke  Sputum production
 It is estimated that AAT effects 1 out  Reoccurring chest colds
of every 2,500 people in the U.S.
 These symptoms can be easily
 It takes on the average, three doctors confused with other non-hereditary
and seven years from the time the COPD or asthma
lung symptoms first appear before a
confirmed diagnosis can be made
Emphysema and Alpha 1 Antitrypsin Deficiency
Patients can present with symptoms of emphysema, such as shortness of breath, chronic
liver disease, or cholestatic / obstructive jaundice
Carriers may go through their lives without ever developing symptoms but there is an
increased risk for carriers who smoke

24. Two types of AAT deficiencies:
The genetic emphysema The acquired emphysema
In the Alpha-1 patient, the lower Upper portion of the lungs are
regions of the lungs are affected affected
Usually causes symptoms in
people in their 30’s and 40’s Mainly smoking caused and
patient’s tend to be diagnosed in
their 60’s and 70’s.
 Hyperlucency Both cases share the
 Low set flat hyper-inflated lungs due
diaphragm to the destruction of lung
 Vertical heart tissue as well as flatten
 Pre and infra diaphragms also due to
cardiac lungs the hyper-inflation of the
 Barrel shape lungs

25. The incidence of antitrypsin S and Z types are due to a
deficiency is 1/2000 to 1/7000. Genealogy single amino acid
substitution at positions
Autosomal recessive on 264 and 342 which lead to
chromosome 14 and has a carrier decreased production of
frequency of 1:10. antitrypsin.
The genetic classified
As antitrypsin deficiency is variants are:
autosomal recessive, if one
parent is a carrier, each child Medium (M)
has a 1/4 chance of being a Slow (S)
carrier themselves. Very Slow (Z).

26. If one parent has the disease (homozygous), then all their
children will be carriers. If both parents are carriers, then
there is a 1/4 chance of their child having the disease
while, 1/2 chance their child will be a carrier.

27. Normal white blood cells In a Alpha 1 Deficiency,
in the lungs produce an The Alpha 1 Antitrypsin the enzyme elastase
enzyme called neutrophil protein is suppose to keeps working by
elastase that destroys neutralize this enzyme attacking and
invading germs and after a short time, if destroying normal lung
digest damage or aging working normally tissue
cells.
Alpha-1 Antitrypsin, How it Destroys
Lung Tissue
These white blood cells Lungs over time will In the healthy
which are meant to begin to lose their individual, lungs will
protect the lungs actually elasticity and as a result, loose their elasticity
begins to destroy healthy COPD can and most naturally over time, but
lung tissue with very likely will develop with an Alpha 1
little to stop it Antitrypsin Deficiency it
may speed this process

28. Treatment Options for Alpha-1
Bronchodilators, c
orticoid steroids
and oxygen
therapy
Augmentation Lung Transplant in the most sever
therapy cases of lung destruction

29. Health Tips
There is no cure for Emphysema or Alpha 1 Antitrypsin and damage to
the lungs done by these diseases are irreversible….
 Quit smoking
 Avoid irritants from chemicals or air pollution
 Protect yourself from lung infections
 Received pneumonia and annual flu vaccines to decrease the chances of respiratory
problems.
 Healthy diet (eat right, loose weight and staying fit)
 Take the medications that your doctor prescribes
 Exercise is a great option and even though it will not improve lung function, it may
decrease the patient’s frequency of hospital stays as well as improve overall health.
**Gains made by exercising will be lost once the person decides to quit exercising.
Even though the damage done cannot be reversed, sticking to a good health
care plan may improve overall quality of life and may in some cases slow the
progression of these diseases