Iipulmonary alveolar proteinosis

IPulmonary alveolar proteinosis (PAP) sisifo esinqabileyo apho uhlobo lweprotheni lwakhiwa khona kwiingxowa zomoya (alveoli) yemiphunga, nto leyo eyenza ukuphefumla kube nzima. Iipulmonary zithetha ngemiphunga.

Ngamanye amaxesha, oyena nobangela wePAP akaziwa. Kwabanye, kwenzeka ngosulelo lwemiphunga okanye ingxaki yomzimba yokuzikhusela. Inokuthi yenzeke kunye nomhlaza wenkqubo yegazi, kwaye emva kokuvezwa kwinqanaba eliphezulu lezinto zokusingqongileyo, ezinje ngesilica okanye uthuli lwealuminium.

Abantu abaphakathi kweminyaka engama-30 nengama-50 ubudala bahlala bechaphazeleka. I-PAP ibonwa emadodeni rhoqo kunabafazi. Uhlobo lokuphazamiseka lukhona ekuzalweni (kokuzalwa).

Iimpawu zePAP zinokubandakanya oku kulandelayo:

• Ukuqhawukelwa ngumphefumlo
• Khohlela
• Ukudinwa
• Umkhuhlane, ukuba ngaba kukho usulelo lwemiphunga
• Ulusu oluhlaza okwesibhakabhaka (cyanosis) kwiimeko ezinzima
• Ukuhla ukusinda

Ngamanye amaxesha, akukho zimpawu.

Umboneleli wezempilo uya kumamela imiphunga nge-stethoscope kwaye unokuva ukuqhekeka (imithambo) emiphungeni. Rhoqo, ukuvavanywa komzimba kuyinto eqhelekileyo.

Olu vavanyo lulandelayo lunokwenziwa:

• I-Bronchoscopy nge-saline yokuhlamba imiphunga (ukuhlamba)
• I-x-ray yesifuba
• Ukuxilongwa kwe-CT kwesifuba
• Uvavanyo lomsebenzi wemiphunga
• I-biopsy evulekileyo yemiphunga (utyando lwe-biopsy)

Unyango lubandakanya ukuhlamba into yeprotein emiphungeni (lavage-lung lavage) amaxesha ngamaxesha. Abanye abantu banokufuna ukufakelwa kwemiphunga. Ukuphepha uthuli olunokubangela imeko kuyacetyiswa.

Olunye unyango olunokulingwa sisiyobisi esikhuthaza igazi esibizwa ngokuba yi-granulocyte-macrophage colony stimulating factor (GM-CSF), esilela kwabanye abantu abane-alveolar proteinosis.

Ezi zibonelelo zinokunika ulwazi oluthe kratya kwi-PAP:

• Umbutho weSizwe woNxunguphalo oluNqabileyo-ararediseases.org/rare-diseases/pulmonary-alveolar-proteinosis
• Isiseko sePAP-papfoundation.org

Abanye abantu abanePAP baya kuxolelo. Abanye banokwehla kosulelo lwemiphunga (ukusilela kokuphefumla) okuya kusiba mandundu, kwaye banokufuna ukufakelwa kwemiphunga.

Fowunela umnikezeli wakho ukuba uphuhlisa iimpawu zokuphefumla. Ukuphefumla okufutshane okuya kusiba mandundu ngokuhamba kwexesha kunokubonisa ukuba imeko yakho ikhula ibe yingxakeko yonyango.

IPAP; Alveolar proteinosis; I-alveolar alveolar ye-pulmonary phospholipoproteinosis; I-alveolar lipoproteinosis phospholipidosis

• Isifo semiphunga esiphakathi kwabantu abadala- ukukhutshwa

• Inkqubo yokuphefumla

U-Levine SM. Ukuphazamiseka kokugcwalisa kweAlveolar. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl. 85.

I-Trapnell BC, i-Luisetti M. Pulmonary alveolar proteinosis syndrome. Ku: Broaddus VC, Mason RJ, Ernst JD, et al, ii-eds. Incwadi kaMurray kunye neNadel yeyeza lokuphefumla. Umhla wesi-6. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 70.