pulmonary alveolar proteinosis

pulmonary alveolar proteinosis

      respiratory disorder caused by the filling of large groups of alveoli with excessive amounts of surfactant, a complex mixture of protein and lipid (fat) molecules. The alveoli are air sacs, minute structures in the lungs in which the exchange of respiratory gases occurs. The gas molecules must pass through a cellular wall, the surface of which is generally covered by a thin film of surfactant material secreted from the alveolar cells. When too much surfactant is released from the alveolar cells, or when the lung fails to remove the surfactant, gas exchange is greatly hindered and the symptoms of alveolar proteinosis occur.

      The disease manifests itself in laboured breathing at rest or shortness of breath with exertion, and it is often accompanied by chest pain and a dry cough. There may also be general fatigue and weight loss. The skin becomes tinged with blue in the most serious cases, an indication that blood is not being adequately oxygenated or rid of carbon dioxide. X-rays most frequently show evidence of excess fluids in the lungs.

      The precipitating cause of the disease is unknown. Persons affected are usually between 20 and 50 years of age. The disease can exist without causing symptoms for considerable periods, and spontaneous improvement has been known to occur; it is sometimes fatal, but rarely so, if treated. Treatment involves removal of the material by a rinsing out of the lungs (lavage). One lung at a time is rinsed with a saltwater solution introduced through the windpipe. The fluids drawn back out of the lungs have been found to have a high content of fat. Sometimes the lesions totally clear up after one procedure, but subsequent treatments are often necessary.

* * *


Universalium. 2010.

Игры ⚽ Нужно решить контрольную?

Look at other dictionaries:

  • Pulmonary alveolar proteinosis — (PAP) is a rare lung disease in which abnormal accumulation of surfactant occurs within the alveoli, interfering with gas exchange. PAP can occur in a primary form or secondarily in the settings of malignancy (especially in myeloid leukemia),… …   Wikipedia

  • pulmonary alveolar proteinosis — n a chronic disease of the lungs characterized by the filling of the alveoli with proteinaceous material and by the progressive loss of lung function * * * a chronic lung disease characterized by dyspnea, productive cough, chest pain, weakness,… …   Medical dictionary

  • alveolar proteinosis — pulmonary alveolar p …   Medical dictionary

  • Alveolar lung disease — Alveolar lung disease, also known as airspace disease, is a general term that described edema and exudates in the airspaces of the lung (the acini and alveoli).CausesAlveolar lung disease may be divided into acute or chronic. Causes of acute… …   Wikipedia

  • Pulmonary edema — Classification and external resources Pulmonary edema with small pleural effusions on both sides. ICD 10 J …   Wikipedia

  • Pulmonary hypertension — Pulmonary arterial hypertension Classification and external resources The pulmonary artery receives blood (blue arrow) from the right ventricle of the heart; increased pressure on the artery can impair the function of the right ventricle ICD …   Wikipedia

  • Pulmonary consolidation — Classification and external resources Pneumonia as seen on chest x ray. A: Normal chest x ray. B: Abnormal chest x ray with consolidation from pneumonia in the right lung, middle or inferior lobe (white area, left side of image) …   Wikipedia

  • Pulmonary embolism — Classification and external resources Chest spiral CT scan with radiocontrast agent showing multiple filling defects both at the bifurcation and in the pulmonary arteries. ICD 10 …   Wikipedia

  • Pulmonary surfactant — Diagram of the alveoli with both cross section and external view Pulmonary surfactant is a surface active lipoprotein complex (phospholipoprotein) formed by type II alveolar cells. The proteins and lipids that surfactant comprises have both a… …   Wikipedia

  • proteinosis — A state characterized by disordered protein formation and distribution, particularly as manifested by the deposition of abnormal proteins in tissues. [protein + G. osis, condition] lipoid p. [MIM*247100] a disturbance of lipid metabolism …   Medical dictionary

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”