Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) o se faʻamaʻi seasea tupu ai le ituaiga o polotini e faʻaputuputu i luga o le ea (alveoli) o mama, ma faʻafaigata ai ona manava. Pulmonary o lona uiga e fesoʻotaʻi ma māmā.

I nisi tulaga, o le mafuaʻaga o le PAP e leʻo iloaina. I isi, e tupu faʻamaʻi pipisi poʻo se faʻafitauli tau puipuiga. E mafai foi ona tupu i kanesa o le toto faiga, ma a maeʻa aafia i le maualuga tulaga o siʻosiʻomaga mea, pei o le silica poʻo le alumini pefu.

O tagata i le va o le 30 ma le 50 tausaga le matutua e masani ona aʻafia. E masani ona vaaia le PAP i tamaloloa nai lo fafine. O se ituaiga o le le atoatoa o loʻo i ai i le fanau mai (fanau mai).

Faʻailoga o le PAP ono aofia ai se tasi o mea nei:

• Manava le mānava
• Mama
• Le lavava
• Fiva, pe afai e i ai siama mama
• Paʻu lanumoana (cyanosis) i matuia tulaga
• Paʻu pauna

O isi taimi, e leai ni faʻailoga.

O le tausi soifua maloloina o le a faʻalogo i mama i se stethoscope ma ono faʻalogo i paʻu (rales) i mama. E masani lava, o le faʻataʻitaʻiga o le tino e masani.

O suʻega ia e ono faia:

• Bronchoscopy ma fufulu masima o le mama (lavage)
• Fatafata x-ray
• CT faʻataʻitaʻi le fatafata
• Luga gaioiga suʻega
• Tatala biopsy mama (taotoga biopsy)

Togafitiga aofia ai le fufulu ese o le polotini vailaʻau mai le mama (atoa-mama lavage) mai lea taimi i lea taimi. Nisi tagata ono manaʻomia se tui mama. O le aloese mai pefu e ono mafua ai le tulaga ua fautuaina foi.

O le isi togafiti e ono faʻataʻitaʻia o se vailaʻau e faʻaosofia ai le toto e faʻaigoaina o le granulocyte-macrophage colony stimulate factor (GM-CSF), lea e le o maua i nisi tagata e iai le alveolar proteinosis.

O nei fesoasoani e mafai ona maua ai nisi faʻamatalaga i luga o le PAP:

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

Nisi tagata ma PAP alu i le faʻamagaloga. O isi ua i ai le paʻu o mama papala (manava toilalo) e atili ai ona leaga, ma latou ono manaʻomia se fesuiaʻiga mama.

Valaʻau lau tautua pe a fai e te maua ni faʻamaʻi manava manava. O le pupuu o le manava e atili ai ona leaga i le taimi e ono ono faʻaalia ai o lou tulaga ua amata ona avea ma se faʻalavelave faʻafuaseʻi.

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

• Faʻamaʻi pipisi i totonu ole vaʻa - tagata matutua - faʻateʻaina

• Respiratory system

Levine SM. Alveolar faʻatumu faʻaletonu. I totonu: Goldman L, Schafer AI, eds. Faʻafomaʻi Goldman-Cecil. 26th lomiga. Filatelefia, PA: Elsevier; 2020: chap 85.

Trapnell BC, Luisetti M. Pulmonary alveolar proteinosis syndrome. I: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray ma Nadel's Textbook of Respiratory Medicine. 6th ed. Filatelefaia, PA: Elsevier Saunders; 2016: mata 70.