I-pulmonary alveolar proteinosis

I-Pulmonary alveolar proteinosis (PAP) isifo esingajwayelekile lapho uhlobo lwamaprotheni lakhiwa khona ezikhwameni zomoya (alveoli) zamaphaphu, okwenza ukuphefumula kube nzima. Izindlela zamaphaphu zihlobene namaphaphu.

Kwezinye izimo, imbangela ye-PAP ayaziwa. Kwabanye, kwenzeka ngokutheleleka kwamaphaphu noma inkinga yokuzivikela komzimba. Kungenzeka futhi ngomdlavuza wesistimu yegazi, nangemva kokuchayeka emazingeni aphezulu wezinto zemvelo, njengothuli lwe-silica noma i-aluminium.

Abantu abaphakathi kweminyaka engama-30 nengama-50 ubudala bavame ukuthinteka. I-PAP ibonakala emadodeni kaningi kunakubesifazane. Uhlobo lokuphazamiseka lukhona lapho kuzalwa (lokuzalwa).

Izimpawu ze-PAP zingafaka noma yikuphi okulandelayo:

• Ukuphelelwa umoya
• Khwehlela
• Ukukhathala
• Imfiva, uma kukhona ukutheleleka kwamaphaphu
• Isikhumba se-Bluish (cyanosis) ezimweni ezinzima
• Ukwehla kwesisindo

Kwesinye isikhathi, azikho izimpawu.

Umhlinzeki wezokunakekelwa kwezempilo uzolalela amaphaphu nge-stethoscope futhi angezwa ama-crackles (rales) emaphashini. Imvamisa, ukuhlolwa komzimba kuyinto ejwayelekile.

Ukuhlolwa okulandelayo kungenziwa:

• I-bronchoscopy enosawoti wokugeza amaphaphu (ukuhlanza)
• I-x-ray yesifuba
• I-CT scan yesifuba
• Ukuhlolwa kwemisebenzi yamaphaphu
• Vula i-lung biopsy (i-biopsy yokuhlinzwa)

Ukwelashwa kuhilela ukuwashwa kwephrotheni kusuka emaphashini (ukuqhuma okuphelele kwamaphaphu) ngezikhathi ezithile. Abanye abantu bangadinga ukufakelwa kwamaphaphu. Ukugwema uthuli okungenzeka ukuthi kubangele isimo nakho kuyanconywa.

Okunye ukwelashwa okungazanywa umuthi ovuselela igazi obizwa ngegranulocyte-macrophage colony stimulating factor (GM-CSF), entula kwabanye abantu abane-alveolar proteinosis.

Lezi zinsizakusebenza zinganikeza imininingwane eminingi nge-PAP:

• Inhlangano Kazwelonke Yezinkinga Ezingajwayelekile - rarediseases.org/rare-diseases/pulmonary-alveolar-proteinosis
• Isisekelo se-PAP - www.papfoundation.org

Abanye abantu abane-PAP bangena ekuxolelweni. Abanye banokwehla kokutheleleka kwamaphaphu (ukwehluleka ukuphefumula) okuya kuba kubi kakhulu, futhi bangadinga ukufakelwa kwamaphaphu.

Shayela umhlinzeki wakho uma uba nezimpawu zokuphefumula ezinzima. Ukuphefumula okuya kuba kubi ngokuhamba kwesikhathi kungakhombisa ukuthi isimo sakho sikhula sibe yisimo esiphuthumayo sezokwelapha.

I-PAP; I-Alveolar proteinosis; I-alveolar alveolar phospholipoproteinosis; I-alveolar lipoproteinosis phospholipidosis

• Isifo samaphaphu esiphakathi - abantu abadala - ukukhipha

• Uhlelo lokuphefumula

U-Levine SM. Izinkinga zokugcwalisa i-Alveolar. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 85.

UTrapnell BC, uLuisetti M. Pulmonary alveolar proteinosis syndrome. Ku: Broaddus VC, Mason RJ, Ernst JD, et al, abakwa-eds. Incwadi kaMurray neNadel Yemithi Yokuphefumula. Umhlaka 6. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 70.