Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) ke lefu le sa tloaelehang leo ho lona mofuta oa protheine o hahang ka har'a mekotla ea moea (alveoli) ea matšoafo, e leng ho etsang hore ho heme ho be thata. Mokhoa oa pulmonary o amanang le matšoafo.

Maemong a mang, sesosa sa PAP ha se tsejoe. Ho tse ling, e hlaha ka tšoaetso ea matšoafo kapa bothata ba 'mele ea ho itšireletsa mafung. E ka hlaha hape ka mofets'e oa sistimi ea mali, 'me kamora ho pepesetsoa maemo a phahameng a lintho tsa tikoloho joalo ka silica kapa lerole la aluminium.

Hangata batho ba lilemo tse pakeng tsa 30 le 50 ba ameha haholo. PAP e bonoa ho banna khafetsa ho feta basali. Mofuta oa bokuli o teng nakong ea tsoalo (congenital).

Matšoao a PAP a ka kenyelletsa tse latelang:

• Phefumoloho e khutšoanyane
• Khohlela
• Mokhathala
• Feberu, haeba ho na le tšoaetso ea matšoafo
• Letlalo la Bluish (cyanosis) maemong a boima
• Ho fokotsa boima ba 'mele

Ka linako tse ling, ha ho na matšoao.

Mofani oa tlhokomelo ea bophelo o tla mamela matšoafo ka stethoscope mme a ka utloa mapatso (rales) matšoafong. Hangata, tlhahlobo ea 'mele e tloaelehile.

Liteko tse latelang li ka etsoa:

• Bronchoscopy e nang le letsoai le hlatsoang matšoafo (ho hlatsoa)
• X-ray sefubeng
• Tlhatlhobo ea sefuba ea CT
• Liteko tsa mosebetsi oa matšoafo
• Open biopsy lung (biopsy ea ho buoa)

Kalafo e kenyelletsa ho hlatsoa ntho ea protheine ho tsoa matšoafong (ho hlatsuoa ka hohle-hohle) nako le nako. Batho ba bang ba kanna ba hloka ho kenyetsoa matšoafo. Ho qoba lerōle le kanna la baka boemo bona le hona hoa khothaletsoa.

Kalafo e 'ngoe e ka' nang ea lekoa ke sethethefatsi se susumetsang mali se bitsoang granulocyte-macrophage colony stimulating factor (GM-CSF), se haelloang ho batho ba bang ba nang le alveolar proteinosis.

Lisebelisoa tsena li ka fana ka tlhaiso-leseling e batsi ka PAP:

• Mokhatlo oa Naha oa Mathata a Rare - rarediseases.org/rare-diseases/pulmonary-alveolar-proteinosis
• Motheo oa PAP - www.papfoundation.org

Batho ba bang ba nang le PAP ba ea tšoarelong. Ba bang ba na le ts'oaetso ea tšoaetso ea matšoafo (ho hloleha ho hema) e mpefalang, mme ba ka hloka ho kenyetsoa matšoafo.

Bitsa mofani oa hau haeba u ba le matšoao a tebileng a ho hema. Phefumoloho e fokolang e ntseng e mpefala ha nako e ntse e feta e ka bontša hore boemo ba hau bo fetoha boemo ba tšohanyetso ba bongaka.

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

• Boloetse ba sefuba - batho ba baholo - ho tsoa

• Tsamaiso ea phefumoloho

Levine SM. Mathata a ho tlatsa alveolar. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 85.

Trapnell BC, Luisetti M. Pulmonary alveolar proteinosis lefu. Ka: Broaddus VC, Mason RJ, Ernst JD, et al, li-eds. Buka ea Bongaka ea Phefumoloho ea Murray le Nadel. La 6th. Philadelphia, PA: Elsevier Saunders; 2016: khaolo ea 70.