Alveolus protein deposits pathologic _'s general knowledge of cause of disease of disease

Alveolus protein deposits pathologic _'s general knowledge of cause of disease of disease
Alveolus protein deposits disease (pulmonary alveolar proteinosis, is abbreviated as PAP) ,Also called Rosen-Castle-man-Liebow syndrome. Full of PAS and dye the masculine gender in alveolus and thin bronchus, rich phosphatide protein material from lung is its characteristic. Good hair, on blue and green middle age, man have illness coming on about 3 times as much as that of woman. Cause of disease bright, might with the intersection of immunological function and obstacle (until thymus wither and immune defect, lymphocyte reduce etc.) Have something to do. The dust can cause PAP in order to contact the animal of the silicious dust particularly, so think it may be to some non- peculiar responses of stimulating the thing, cause alveolus macrophages to decompose, produce PAS positive protein.
Cause of disease
The cause of disease has not been bright, infer that relates to several respect factors: For instance a large amount of dust is sucked (aluminium, oxidize silicons,etc. two times) ,Organism immunological function drops ' Especially infant and pre-school children) ,Inherent cause, get drunk, the microorganism is infected with etc.. And for infect, sometimes very difficult to confirm, send, cause cause of disease to be plain or continue, take place at the intersection of the alveolus and the intersection of albumen and calm disease originally. Such as the virus of big and small afterbirth, the lung sporozoite of card, organizes the afterbirth thick liquid fungus to infect etc. finding that there is calm high protein in alveoluses.
Though it is still indeterminate to start the factor, basically agree that the course is due to lipid supersession obstacle to have illness coming on. Namely because factor function causes metabolic disorder of the pulmonary surfactant in, outside the organism. Up till now, alveolus macrophage vigors to study more one, animal's experiment proves their vigor obviously drops after macrophages engulf the dust, and the patient can make normal cell vigor drop to irritate the macrophage inner particles in lotion, after the bronchus alveolus is irritated and washes and treats, its macrophage vigor of alveolus can rise. Study and has not found Model 2 cells turn into the albumen to increase, the lipoprotein supersession of whole body is not unusual. So generally think a disease drops and has something to do with with removing ability at present.
Pathology
The lung presents consolidation mostly, can be seen yellow or yellow ashy tubercle under the pleura, the tangent plane has yellow liquid that is oozed out. The mirror examines and shows and has a liking for sour PAS strong positive materials to fill in alveolus and detailed bronchus, other protein and combination of immunoglobulin (Ig) in surface active substance phosphatide and alveolus inner liquid that it is the Model 2 alveolus cells produce, the alveolus separates and the structure around is basically intact. The electric mirror can be seen alveolus macrophages and increase in a large amount, engulf the surface active substance of lung, thick liquid swelling of afterbirth, present one kind of appearances of vacuole or foam.
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