Emphysema and Alfa 1 Antitrypsin
Nov 11, 2014
Emphysema and Alfa 1 Antitrypsin
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.
Emphysema Causes:
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)
Bronchospasm (“smooth muscle constriction of the bronchial airways”)
Hyperinflation of alveoli (“air trapping”)
General Overview
Notice how the alveoli are plentiful and well formed in the in the healthy lung , but in the lung with emphysema, the amount of alveoli are reduced and enlarged thus reducing the surface area of the lung…..
Gross anatomy of a lung with 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 lungs are markedly enlarged.
Gross anatomy of a healthy lung
A healthy, functioning lung with no apparent disease.
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.
An emphysematous lung shows increased anteroposterior (AP) diameter, increased retrosternal airspace, and flattened diaphragms on lateral chest
radiograph.
Source: Emphysematous Chest X-ray II
The thickness of the space between the
ascending aorta and the sternum is
normally no more than 2.5cm. Increased
retrosternal airspace is an indicator of
hyperinflation of the lungs and is usually due to emphysema.
Increased retrosternal airspace
is an indicator of hyperinflation of the lungs and is usually due to emphysema.
Congential Emphysema
Half of the cases of congenital
lobar emphysema occur in the
first four weeks of life, and three-
quarters occur in infants less
than six months old.
Congenital lobar emphysema is
more common in boys than in
girls.
Etiology is unknown
50% of the cases of CLE there is decreased bronchial cartilage tissue. This defect produces a ball valve effect with consequent overinflation.
It is diagnosed by respiratory symptoms and a chest x ray, which will show the over-inflation of the affected lobe and may show a blocked air passage.
“Also called infantile lobar emphysema, is a respiratory disease that occurs in infants when air enters the lungs but cannot leave easily. “
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……….
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
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.
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.
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.
A gross photograph showing the emphysematous right upper lobe
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.
Smoking and Emphysema
Number one cause of COPD/Emphysema
SO YOU WANT TO SMOKE???
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.
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?
Second Hand Smoke
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)
Quitting is always the BEST option for yourself and others around you
Alpha 1 Antitrypsin
What is alpha 1 Antitrypsin?
Cross section of native lung with alpha-1 anti-trypsin
deficiency
Alpha 1 antitrypsin deficiency is a 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
Alpha 1 Antitrypsin Deficiency: A Less Common
Cause of Emphysema
What is the purpose of Alpha 1 Antitrypsin?
The sole purpose of AAT is to protect the lungs from inflammation caused by infection and inhaled irritants such as cigarette smoke
It is estimated that AAT effects 1 out of every 2,500 people in the U.S.
It takes on the average, three doctors and seven years from the time the lung symptoms first appear before a confirmed diagnosis can be made
The most common side effects of AAT only related to
the lungs: Shortness of breath
Wheezing chronic cough
Sputum production
Reoccurring chest colds
These symptoms can be easily confused with other non-hereditary COPD or asthma
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
The genetic emphysema
In the Alpha-1 patient, the lower regions of the lungs
are affected
Usually causes symptoms in people in their 30’s and 40’s
Two types of AAT deficiencies:
The acquired emphysema
Upper portion of the lungs are affected
Mainly smoking caused and patient’s tend to be
diagnosed in their 60’s and 70’s. Both cases share the hyper-inflated lungs due to the
destruction of lung tissue as well as
flatten diaphragms also due to the
hyper-inflation of the lungs
Hyperlucency Low set flat
diaphragm Vertical heart Pre and infra
cardiac lungs Barrel shape
The incidence of antitrypsin deficiency is 1/2000 to 1/7000.
Autosomal recessive on chromosome 14 and has a carrier frequency of 1:10.
Genealogy
The genetic classified variants are:
Medium (M)Slow (S)Very Slow (Z).
S and Z types are due to a single amino acid substitution at positions 264 and 342 which lead to decreased production of antitrypsin.
As antitrypsin deficiency is autosomal recessive, if one parent is a carrier, each child has a 1/4 chance of being a carrier themselves.
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.
Alpha-1 Antitrypsin, How it Destroys Lung Tissue
In a Alpha 1 Deficiency, the
enzyme elastase keeps working by
attacking and destroying normal
lung tissue
Normal white blood cells in the lungs
produce an enzyme called neutrophil
elastase that destroys invading germs and digest damage or aging
cells.
These white blood cells which are
meant to protect the lungs actually begins to destroy
healthy lung tissue with very little to
stop it
The Alpha 1 Antitrypsin protein
is suppose to neutralize this enzyme after a short time, if
working normally
Lungs over time will begin to lose their elasticity and as a result, COPD can
and most likely will develop
In the healthy individual, lungs will loose their elasticity naturally over time, but with an Alpha 1
Antitrypsin Deficiency it may speed this process
Treatment Options for Alpha-1
Bronchodilators, corticoid
steroids and oxygen therapy
Augmentation therapy
Lung Transplant in the most sever cases of lung
destruction
Health TipsThere 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