Pulmonary alveolar proteinosis Murray p1789 - 1801
1. °³¿ä
alveoli & bronchiole¿¡ amorphous, insoluble, proteinaceous materialÀÌ ÃàÀûµÇ´Â º´ÀÌ´Ù.
proteinaceous material¿¡´Â phospholipid & surfactant proteinÀÌ Ç³ºÎÇÏ´Ù.
ÀÌ ¹°ÁúÀÌ ¿°Áõ¹ÝÀÀÀ» ÀÚ±ØÇÏÁö´Â ¾ÊÀ¸¹Ç·Î lung fibrosis´Â °ÅÀÇ ¾ø´Ù.
1Â÷Àû Àå¾Ö´Â »ê¼Ò¿î¹Ý´ÉÀÇ Àå¾ÖÀ̸ç ȯÀÚ´Â È޽ĻóÅ¿¡¼ Àú»ê¼ÒÁõÀ» º¸ÀδÙ.
±âȸ°¨¿°¿¡ ÀÇÇØ »ç¸ÁÇϱ⵵ Çϳª °á±¹Àº hypoxemia·Î »ç¸ÁÇÑ´Ù.
2. Á¶Á÷º´¸®: PAS(+) cytoplasm
alveolar septa´Â no significant fibrosis
ÀϺο¡¼ recurrent pneumonia history°¡ ÀÖ´Ù.
Norcardia asteroides¿¡ ´ëÇÑ ¹Ýº¹Àû Æó°¨¿°ÀÌ ÀϾ ¼ö ÀÖ´Ù.
ÀϺο¡¼ repeat infectionÀÌ fibrosis & inflammatory responseÀÇ ¿øÀÎÀÌ µÈ´Ù.
3. ÀÚ¿¬°æ°ú
1965³â whole-lung lavage°¡ ¼Ò°³µÈÈÄ ¿¹Èİ¡ Çâ»óµÇ¾ú´Ù. ´Ù¸¥ º´°ú´Â º¸Åë °ü°è°¡ ¾øÀ¸³ª
hematologic malignancy, inorganic dust exposure, infection in immunocompromised
person¿¡¼ º¸°íµÇ¾ú´Ù.
young or middle aged adult¿¡ ÁÖ·Î »ý±â°í ³²³àºñ´Â 2:1ÀÌ´Ù.
Nocardia pneumonia ºóµµ°¡ ³ôÀ¸¸ç, ƯÈ÷ ¸é¿ª¾ïÁ¦È¯ÀÚ¿¡¼ ºóµµ°¡ ³ô´Ù.
* secondary alveolar proteinosis
HIV(+), malignancy(+), silicoproteinosis
4. ¿øÀÎ ¹× º´ÀÎ
¸¹Àº °¡¼³ÀÌ ÀÖ´Ù.
genetic defect, defective alveolar macrophage, surfactant overproduction or decreased
clearance, immunodeficiency
i) primary
ii) secondary: coexisting abnormality°¡ µ¿¹ÝµÉ ¶§
infection, hematologic malignancy, silicosis
* silicoproteinosis : airspace¿¡ »ó´ç·®ÀÇ silica or silicates°¡ ¹ß°ßµÉ ¶§ silicoproteinosis
¶ó´Â ¿ë¾î¸¦ ¾´´Ù.
5. ÀÓ»ó¹ßÇö
ÁÖÁõ»óÀº ¿îµ¿½Ã È£Èí°ï¶õÀÌ´Ù.
±× ¿Ü, wt loss, malaise, fatigue
fever°¡ ÀÖ´Ù¸é, pulmonary alveolar proteinosis°¡ ¾Æ´Ï°Å³ª superinfectionµÈ °æ¿ìÀÌ´Ù.
6. ¹æ»ç¼±ÇÐÀû ¼Ò°ß
CXR : uniformly infiltration
perihilar¿¡ ´õ ½ÉÇÏ¿© pulmonary edema¶§ º¼ ¼ö ÀÖ´Â "bat wing" appearance¼Ò°ßÀ»
º¸ÀδÙ.
interstitial marking¡è, acinar filling¡è
CT : dense consolidation or ground glass appearance
thickened lobular septa => diffuse "interstitial" pattern
7. Lab: LDH¡è, SGOT: normal
pulmonary surfactant protein A¡è
8. PFT: DLco¡é
9. Áø´Ü: Áõ»ó + CXR => ¸¹Àº ÆóÁúȯ¿¡¼ ÈçÈ÷ º¼¼ö ÀÖ´Â ¼Ò°ßÀÌ´Ù.
BAL or TBLB(open lung biopsy´Â ÇÇÇϵµ·Ï ÇÑ´Ù)
BALÀÇ LM¼Ò°ß: PAS(+) deep pink
macrophage¸¦ ÈçÈ÷ º¼¼ö ÀÖ°í ´Ù¸¥ cellÀº ¾ø´Ù.
Áø´ÜÀº lung biopsy or BAL fluid·Î Çϵµ·Ï ÇÑ´Ù.
10. Ä¡·á: whole-lung lavage
º¸Åë 6-12°³¿ù °£°ÝÀ¸·Î ½ÃÇàÇÑ´Ù.
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