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