In normal breathing, air is drawn in through the bronchi and into the alveoli, which are tiny sacs

surrounded by capillaries. Alveoli absorb oxygen and then transfer it into the blood. When toxicants, such as cigarette smoke, are breathed into the lungs, the harmful particles become trapped in the alveoli, causing a localized inflammatory response. Chemicals released during the inflammatory response (e.g., elastase) can eventually cause the alveolar septum to disintegrate. This condition,

the side responsible for pumping deoxygenated blood to the lungs. and the body is not able to maintain high enough oxygen levels in the blood. A1AT inhibits inflammatory enzymes (such as elastase) from destroying the alveolar tissue. The large cavities left by the septal rupture are known as bullae (sin. Due to decreased ventilation. as the heart continues to fail. as well as decreased ventilation of the surrounding healthy parenchyma. it becomes larger and blood backs up in the liver. as immediate and spontaneous consequence of septal rupture. but smoking and severely decreased A1AT levels (10-15%) can cause emphysema at a young age. the elastic lung recoil resets healthy parenchyma expansion at a lower level. Patients with alpha 1-antitrypsin deficiency (A1AD) are more likely to suffer from emphysema. Most A1AD patients do not develop clinically significant emphysema.known as septal rupture. which physiologically depends completely on lung elastic recoil. The type of emphysema caused by A1AD is . increasingly depends on the thoracic cage and abdominal muscle action. This results in a decreased Transfer Factor of the Lung for Carbon Monoxide (TLCO). In red is the extra space of the new cavity after septal rupture due to the lung elastic recoil rearrangement. In other words. To accommodate the decreased surface area. This leads to pulmonary hypertension. particularly in the end expiratory phase. necessarily at the expenses of the surrounding healthy parenchyma. the ability to exude carbon dioxide is significantly impaired. hyperventilation is unable to compensate for the progressively shrinking surface area. Expiration. The body's last resort is vasoconstricting appropriate vessels. Eventually. in proportion to the amount of septal disruption. An elastic net distended on finite surface may offer a bidimensional model useful for understanding the pure mechanical consequences of septal rupture. These deformations result in a large decrease of alveoli surface area used for gas exchange. thoracic cage expansion (barrel chest) and diaphragm contraction (flattening) take place. necessarily at the expenses of the space of the surrounding healthy meshes (alveoli)(VIDEO)[8] in fact because of the lacking mechanical support of the broken septa the lung elastic recoil further enlarges this new space. This condition is often accompanied by the appearance of jugular venous distension. As the alveoli continue to break down. The key mechanical event consequent to septal rupture is that the resulting cavity is larger than the sum of the two alveolar spaces (see side figure). oxygen uptake is also impaired. which places increased strain on the right side of the heart. In the more serious cases. The heart muscle thickens in order to pump more blood. leads to significant deformations of the lung architecture[6][7] (video) that have important functional consequences. = bulla).

known as panacinar emphysema (involving the entire acinus) as opposed to centrilobular emphysema. Mild emphysema can often develop into a severe case over a short period of time (1–2 weeks). as a primary contributor to the connective tissue damage seen in the disease. just because these substances can destroy connective tissue in the lung. often 0–3 years at most. Patients who think they may have contracted the disease are recommended to seek medical attention as soon as possible. which is caused by smoking. Studies for the better part of the past century have focused mainly upon the putative role of leukocyte elastase (also neutrophil elastase). Panacinar emphysema typically affects the lower lungs. Smokers with A1AD are at the greatest risk for emphysema. However. as anyone would be able to predict. more recent studies have brought into light the possibility that one of the many other numerous proteases. These animals developed connective tissue damage. More recent experiments have focused on more technologically advanced approaches. hereditary A1AT deficiency only accounts for a small proportion of the disease. a serine protease found in neutrophils. and A1AT is the primary inhibitor of neutrophil elastase. This hypothesis. However. Pulmonary rehabilitation can be very helpful to optimize the patient's quality of life and teach the patient how to actively manage his or her care. and the assessment of whether they would be less susceptible to the development of the disease. The most important measure to slow its progression is for the patient to stop smoking and avoid all exposure to cigarette smoke and lung irritants. A1AD causes about 2% of all emphysema. while centrilobular emphysema affects the upper lungs. a result of the observation that neutrophil elastase is the primary substrate for A1AT. such as ones involving genetic manipulation. together have been known as the "protease-antiprotease" theory. While A1AD provides some insight into the pathogenesis of the disease. especially matrix metalloproteases might be equally or more relevant than neutrophil elastase in the development of non-hereditary emphysema. Management Emphysema is an irreversible degenerative condition. which was taken as support for the protease-antiprotease theory. The better part of the past few decades of research into the pathogenesis of emphysema involved animal experiments where various proteases were instilled into the trachea of various species of animals. which are genetically deficient in one or more proteases. . Often individuals who are unfortunate enough to contract this disease have a very short life expectancy. implicating neutrophils as an important mediator of the disease. doesn't establish causality. One particular development with respect to our understanding of the disease involves the production of protease "knock-out" animals.

a new treatment option may soon become available. There are lightweight portable oxygen systems which allow patients increased mobility. The stem cells formed human bronchioles. oxygen deprivation and the side-effects of the medications used to treat emphysema cause damage to the kidneys.Emphysema is also treated by supporting the breathing with anticholinergics. but 7% of patients suffered partial lung collapse. effective body positioning (High Fowlers). The only known "cure" for emphysema is lung transplant. In July 2006 a new treatment. Treating the patient's other conditions including gastric reflux and allergies may improve lung function. heart and other organs. steroid medication (inhaled or oral). alveoli. and pulmonary vessels integrated structurally and functionally with the damaged mouse organ. Lung volume reduction surgery (LVRS) can improve the quality of life for certain carefully selected patients. cruise. Supplemental oxygen used as prescribed (usually more than 20 hours per day) is the only non-surgical treatment which has been shown to prolong life in emphysema patients. Surgical transplantation also requires the patient to take an anti-rejection drug regimen which suppresses the immune system. and work while using supplemental oxygen. but few patients are strong enough physically to survive the surgery. and supplemental oxygen as required. . With the discovery of multipotent lung stem cells in 2011. some of which are minimally invasive. bronchodilators. Patients can fly. Other medications are being researched. placing tiny valves in passages leading to diseased lung areas. The combination of a patient's age. It can be done by different methods. The May 2011 report in the New England Journal of Medicine [9] concluded that human lung stem cells "have the undemonstrated potential to promote tissue restoration in patients with lung disease". was announced to have good results. and can lead to microbial infection of the patient. Scientists injected human lung stem cells into mice with damaged lungs.

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