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.
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…..
Gross anatomy of a lung with Gross anatomy of a healthy lung emphysemaNote the clusters of dilated air spaceswhich are conspicuous in the middleand 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.
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. The thickness of the space Increased retrosternalbetween the ascending aortaand 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
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……….
TreatmentLobectomy is the most commonform of treatment and has an 85%success rate with compete cure.Depending on thesymptoms, conservative measuresare sometimes taken, but thesemay 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 withprogressive 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 patients respiratory status improved immediately. The right upper lobe was then resected.
A gross photograph showing theemphysematous right upper lobe
A gross photograph showing the emphysematous right upper lobeExamination 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.
Number one cause of COPD/EmphysemaSO 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?
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 ofemphysema," said Wang. (Science Daily 2007)
Quitting is always the BEST option for yourself and others around you
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
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 madeEmphysema and Alpha 1 Antitrypsin DeficiencyPatients can present with symptoms of emphysema, such as shortness of breath, chronicliver disease, or cholestatic / obstructive jaundiceCarriers may go through their lives without ever developing symptoms but there is anincreased risk for carriers who smoke
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
The incidence of antitrypsin S and Z types are due to adeficiency is 1/2000 to 1/7000. Genealogy single amino acid substitution at positionsAutosomal recessive on 264 and 342 which lead tochromosome 14 and has a carrier decreased production offrequency of 1:10. antitrypsin. The genetic classifiedAs antitrypsin deficiency is variants are:autosomal recessive, if oneparent is a carrier, each child Medium (M)has a 1/4 chance of being a Slow (S)carrier themselves. Very Slow (Z).
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.
Normal white blood cells In a Alpha 1 Deficiency, in the lungs produce an The Alpha 1 Antitrypsin the enzyme elastaseenzyme 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 willprotect the lungs actually elasticity and as a result, loose their elasticitybegins 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
Treatment Options for Alpha-1 Bronchodilators, c orticoid steroids and oxygen therapy Augmentation Lung Transplant in the most sever therapy cases of lung destruction
Health TipsThere is no cure for Emphysema or Alpha 1 Antitrypsin and damage tothe 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