Diseases

Pulmonary arteriovenous malformation

Pulmonary arteriovenous malformations are caused by abnormal communications between pulmonary arteries and pulmonary veins, which are most commonly congenital in nature. Although these lesions are quite uncommon, they are an important part of the differential diagnosis of common pulmonary problems such as hypoxemia and pulmonary nodules. Since their first description at autopsy in 1897 (1), these abnormal communications have been given various names including pulmonary arteriovenous fistulae, pulmonary arteriovenous aneurysms, hemangiomas of the lung, cavernous angiomas of the lung, pulmonary telangiectases, and pulmonary arteriovenous malformations (2). The term "pulmonary arteriovenous malformations" (PAVM) will be used in this review to describe these lesions.

Pulmonary artery agenesis

A rare defect where the pulmonary artery or a portion of it fails to develop. The pulmonary artery has two branches, each of which goes to a different lung. The affected lung tends to be smaller and the bronchial arteries that supply it tend to be enlarged.

Pulmonary artery- isolated unilateral absence of (Isolated UAPA)

A very rare malformation where either the right or left pulmonary artery is missing and there are no other heart abnormalities. The severity of the condition is variable with some infants being severely affected while others are asymptomatic for decades.

Pulmonary atresia with ventricular septal defect

A congenital heart defect where the pulmonary artery is closed off so that blood is unable to flow out to the lungs via the pulmonary artery. An abnormal opening between the two sides of the heart also allow blood to flow between the two heart chambers. The severity of symptoms depends on the degree of abnormality. Symptoms may be evident at birth or later.

Pulmonary branches stenosis

A very rare disorder where the pulmonary branches are narrower than normal. The severity of symptoms depends on the degree of narrowing.

Pulmonary cystic lymphangiectasis

A rare disorder characterized by the presence of numerous small lung cysts at birthinfant born with lung cysts which severely affects breathing and blood pressure and generally results in infant death.

Pulmonary fibrosis

Pulmonary fibrosis (literally "scarring of the lungs") is a respiratory disease in which scars are formed in the lung tissues, leading to serious breathing problems. Scar formation, the accumulation of excess fibrous connective tissue (the process called fibrosis), leads to thickening of the walls, and causes reduced oxygen supply in the blood. As a consequence patients suffer from perpetual shortness of breath.

In some patients the specific cause of the disease can be diagnosed, but in others the probable cause cannot be determined, a condition called idiopathic pulmonary fibrosis. There is no known cure for the scars and damage in the lung due to pulmonary fibrosis.

Pulmonary Lymphangiomatosis

Diffuse pulmonary lymphangiomatosis is a disease in which the overgrowth (proliferation) of lymphatic vessels (lymphangiomatosis) occurs in the lungs, pleura and typically the surrounding soft tissue of the chest (mediastinum). Lymphatic vessels are part of the lymphatic system, which includes lymph nodes, the small nodules where certain white blood cells (lymphocytes) and other cells participate in the immune regulatory system of the body. When fluid leaves arteries and enters the soft tissue and organs of the body, it does so without red or white blood cells. This thin watery fluid is known as lymph. The lymphatic system consists of a network of tubular channels (lymph vessels) that transport lymph back into the bloodstream. Lymph accumulates between tissue cells and contains proteins, fats, and lymphocytes. As lymph moves through the lymphatic system, it passes through the network of lymph nodes that help the body to deactivate sources of infection (e.g., viruses, bacteria, etc.) and other potentially injurious substances and toxins. Groups of lymph nodes are located throughout the body, including in the neck, under the arms (axillae), at the elbows, and in the chest, abdomen, and groin. The lymphatic system also includes the spleen, which filters worn-out red blood cells and produces lymphocytes; and bone marrow, which is the spongy tissue inside the cavities of bones that manufactures blood cells. 

Lymphangiomatosis can potentially affect any part of the body except the brain. The disorder can be widespread, affecting multiple areas simultaneously, as in the case of diffuse pulmonary lymphangiomatosis, or be isolated to one area (i.e. the lungs and chest). The specific symptoms and severity vary, depending in part upon the size and the specific location of the abnormalities. Diffuse pulmonary lymphangiomatosis causes functional impairment of the lungs and when the chest wall is involved, may be associated with disfigurement. The exact cause of diffuse pulmonary lymphangiomatosis is unknown.

Pulmonary lymphangiomatosis has two histologic patterns, conventional and Kaposiform, and in both is a bilateral process. 

Pulmonary Metastatic Osteosarcoma

Pulmonary metastatic osteosarcoma means osteosarcoma, a bone cancer, has spread to the lungs, the most common site, occurring in ~20% of patients at diagnosis and more later, causing symptoms like cough/chest pain, appearing as lung nodules on CT scans, and managed with chemotherapy & often surgery (metastasectomy) for better survival, despite being a major cause of death in OS. 

pulmonary neuroendocrine tumors

Neuroendocrine tumors (NETs) are a rare type of pulmonary neoplasm (growth) that represents less than two percent of all lung tumors. Among the types of neuroendocrine tumors, though, 20 percent to 30 percent start in lung or airway tissue.

Pulmonary sequestration

A pulmonary sequestration, also known as a bronchopulmonary sequestration or a cystic lung lesion, is a medical condition where a piece of tissue that develops into lung tissue is not attached to the pulmonary blood supply and does not communicate with the other lung tissue. Often it gets its blood supply from the thoracic aorta. Communication is a medical phrase indicating that it is not connected to the standard bronchial airways and that it performs no function in respiration.

Pulmonary surfactant protein B- deficiency of

A rare lung disease where protein and fat molecules accumulate in the distant parts of the lungs and affect breathing. The disease is called by a deficiency of a the lung surfactant protein B.

Pulmonary valve stenosis

Pulmonary valve stenosis is a valvular heart disease in which outflow of blood from the right ventricle of the heart is obstructed at the level of the pulmonic valve. This results in the reduction of flow of blood to the lungs. Valvular pulmonic stenosis accounts for 80% of right ventricular outflow tract obstruction.[1] While the most common cause of pulmonary valve stenosis is congenital heart disease, it may also be due to rheumatic heart disease or a malignant carcinoid tumor.[1]

Pulmonary valves agenesis

The total or partial absence of the pulmonary valve at birth. It is often associated with other malformations. Severity is variable.

Pulmonary veins stenosis

Pulmonary vein stenosis is a condition in which there is an obstruction (blockage) in the blood vessels that bring oxygen-rich blood from the lungs back to the heart. Stenosis occurs due to an abnormal process that thickens and narrows the walls in the veins. Pulmonary vein stenosis frequently progresses. As a result, total obstruction or partial loss of flow to a vessel or vessels may occur. This condition may occur as a complicating feature of complex congenital heart disease, but it may also occur in infants with otherwise normal hearts. When pulmonary vein stenosis occurs in children without congenital heart defects, it occurs in early infancy and usually progresses very rapidly. Infants with this disease may seem well for weeks before they develop difficulty breathing and low oxygen levels. They may become quite ill quickly. The effects of the disease vary in children with co-existing congenital heart defects.

Pulmonary veno-occlusive disease

Pulmonary veno-occlusive disease is an extremely rare form of high blood pressure in the lung area.

(aka Pulmonary venoocclusive disease, Venoocclusive disease of lung, Pulmonary vaso-occlusive disease)

 

Source: MedlinePlus

Pulmonary venoocclusive disease

Pulmonary venoocclusive disease is an extremely rare form of high blood pressure in the lung area.

(aka Pulmonary veno-occlusive disease, Venoocclusive disease of lung, Pulmonary vaso-occlusive disease)

 

Source: Medline Plus

Pulmonary venous return anomaly

A rare disorder where one or more of the four veins that carry oxygenated blood from the lungs drain to the right atrium of the heart instead of the left atrium. Symptom severity is determined by the number of veins involved and the exact location of the heart that the veins drain into.

Pulmonic stenosis

Pulmonic stenosis (or PS) is a general term indicating that there is obstruction to normal blood flow in the pulmonary arterial system, which conducts less oxygenated or "blue" blood to the lungs so it can become oxygenated or "pink". This can be at the level of the pulmonic valve, located between the right ventricle and the main pulmonary artery, or in the pulmonary arterial system itself . For the purposes of this discussion, we will discuss the most common type of PS, which is at the level of the valve. The pulmonic valve is normally quite thin (no more than a few millmeters in an adult), and composed of three portions arranged in a circle like a three-slice pie. Normally, the leaflets open in the direction of blood flow with each contraction of the right ventricle, then close to keep blood from traveling backward from the pulmonary arteries.

Pure red cell aplasia

Pure red cell aplasia (or PRCA) refers to a type of anemia affecting the precursors to red blood cells but not to white blood cells. In PRCA, the bone marrow ceases to produce red blood cells.