Pulmonary alveolar proteinosis

ʻO ka pulmonary alveolar proteinosis (PAP) kahi maʻi laha ʻole kahi e kūkulu ʻia ai kahi ʻano protein i loko o nā ʻeke ea (alveoli) o nā akemāmā, e paʻakikī ana ka hanu ʻana. ʻO ke ʻano o ka pulmonary e pili ana i nā māmā.

I kekahi mau hihia, ʻike ʻole ʻia ke kumu o ka PAP. I nā poʻe ʻē aʻe, loaʻa ia me ka maʻi maʻi a i ʻole kahi pilikia pale. Hiki iā ia ke hana me nā maʻi ʻino o ka ʻōnaehana koko, a ma hope o ka hōʻike ʻana i nā kiʻekiʻe o nā mea kaiapuni, e like me ka silica a i ʻole ka lepo aluminika.

Hoʻopilikia pinepine ʻia ka poʻe ma waena o 30 a 50 mau makahiki. ʻIke pinepine ʻia ʻo PAP i nā kāne ma mua o ka wahine. Aia kekahi ʻano o ka maʻi i ka wā hānau (congenital).

Pākuʻi ʻia paha nā ʻōuli o PAP i kekahi o kēia mau mea:

• Pokole o ka hanu
• Cough
• Ka luhi
• Fever, inā loaʻa ka maʻi ʻaʻai
• ʻO kaʻili Bluish (cyanosis) i nā hihia koʻikoʻi
• Kaumaha emi

I kekahi manawa, ʻaʻohe hōʻailona.

E hoʻolohe ka mea mālama ola i nā akemāmā me kahi stethoscope a lohe paha i nā māwae (rales) i nā ake māmā. ʻO ka manawa pinepine, he maʻamau ka ʻike kino ʻana.

E hana ʻia paha kēia mau hoʻāʻo:

• ʻO Bronchoscopy me ka holoi saline o nā akemāmā (lavage)
• Pākuʻi x-ray
• CT scan o ka umauma
• Nā hoʻāʻo hana lung
• Open biopsy lung (ʻoki lāʻau lapaʻau biopsy)

Hoʻopilikia ka mālama ʻana i ka holoi ʻana i ka mea protein mai ka pulupulu (lavage lung holoʻokoʻa) mai kēlā me kēia manawa. Pono paha kekahi poʻe i kahi transplant lung. ʻO ka pale ʻana i nā huna lepo i kumu ke ʻano o kēia ʻano paipai pū kekahi.

ʻO kekahi ʻano lapaʻau e hoʻāʻo ʻia paha he lāʻau hoʻonāukiuki i ke koko i kapa ʻia ʻo kolone granulositte-macrophage stimulate factor (GM-CSF), i nele i kekahi poʻe me ka alveolar proteinosis

Hiki i kēia mau kumuwaiwai ke hāʻawi i ka ʻike hou aku ma PAP:

• National Organization for Rare Disorder - rarediseases.org/rare-diseases/pulmonary-alveolar-proteinosis
• PAP Foundation - www.papfoundation.org

Hele kekahi poʻe me PAP i ke kala. Loaʻa ka hāʻule o nā poʻe ʻē aʻe i ka maʻi o ke akemāhā (ʻole hanu hanu) iʻoi aku ka maikaʻi, a pono lākou i kahi transplant lung.

Kāhea i kāu mea hāʻawi inā hoʻomōhala ʻoe i nā hōʻailona hanu koʻikoʻi. Hiki i ka hanu ke hele a ʻoi aku ka maikaʻi ma mua o ka manawa ke hōʻailona e ulu ana kāu maʻi i kahi pilikia olakino.

PAP; Alveolar proteinosis; Pulmonary alveolar phospholipoproteinosis; Alveolar lipoproteinosis phospholipidosis

• Maʻi maʻi Interstitial lung - makua - hoʻokuʻu

• ʻŌnaehana hanu

ʻO Levine SM. Nā maʻi hoʻopihapiha Alveolar. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 85.

Trapnell BC, Luisetti M. Pulmonary alveolar proteinosis maʻi. I: Broaddus VC, Mason RJ, Ernst JD, et al, eds. ʻO Murray a me Nadel's Textbook of Respiratory Medicine. ʻElua huaʻōlelo Piladelapia, PA: Elsevier Saunders; 2016: chap 70.