Pulmonary alveolar proteinosis

Pulmonary alveolar proteinosis (PAP) chirwere chisina kujairika umo mhando yeprotein inovaka mumhepo masaga (alveoli) emapapu, ichiita kuti kufema kuve kwakaoma. Pulmonary zvinoreva zvine chekuita nemapapu.

Mune zvimwe zviitiko, chikonzero chePAP hachizivikanwe. Mune vamwe, zvinoitika nehutachiona hwemapapu kana dambudziko remumuviri. Izvo zvakare zvinogona kuitika nekenza yehurongwa hweropa, uye mushure mekuratidzwa kune yakakwira nhanho yezvinhu zvezvakatipoteredza, senge silika kana guruva realuminium.

Vanhu vari pakati pemakore makumi matatu nemakumi mashanu ekuberekwa vanowanzo kubatwa. PAP inoonekwa muvarume kazhinji kupfuura vakadzi. Chimiro chechirwere chiripo pakuzvarwa (kuberekwa).

Zviratidzo zvePAP zvinogona kusanganisira chero cheanotevera:

• Kufema kwekufema
• Kukosora
• Kuneta
• Fivha, kana paine hutachiona hwemapapu
• Bluish ganda (cyanosis) mumatambudziko akakomba
• Kuonda

Dzimwe nguva, hapana zviratidzo.

Anopa hutano anoteerera kumapapu ane stethoscope uye anogona kunzwa kutsemuka mumapapu. Kazhinji, kuongororwa kwemuviri kunowanzoitika.

Iyi bvunzo inotevera inogona kuitwa:

• Bronchoscopy ine saline kusuka mapapu (lavage)
• Chest x-ray
• CT scan yechifuva
• Lung basa bvunzo
• Vhura mapapu biopsy (yekuvhiya biopsy)

Kurapa kunosanganisira kusuka chinhu cheprotein kubva mumapapu (yakazara-mapapu lavage) nguva nenguva. Vamwe vanhu vangangoda kuiswa kwemapapu. Kudzivirira guruva iro rinogona kunge rakakonzera mamiriro acho kunokurudzirwa zvakare.

Kumwe kurapwa kunogona kuyedzwa mushonga unokurudzira ropa unonzi granulocyte-macrophage koloni inosimudzira chinhu (GM-CSF), iri kushomeka mune vamwe vanhu vane alveolar proteinosis.

Izvi zviwanikwa zvinogona kupa rumwe ruzivo paPAP:

• National Sangano reRare Dambudziko - rarediseases.org/rare-diseases/pulmonary-alveolar-proteinosis
• PAP Nheyo - www.papfoundation.org

Vamwe vanhu vane PAP vanoenda mukuregererwa. Vamwe vane kuderera kwehutachiona hwemapapu (kutadza kufema) kunowedzera, uye vangangoda kuiswa kwemapapu.

Fonera mupiki wako kana iwe ukaita zvakakomba zviratidzo zvekufema. Kupfupika kwemweya wekufema unowedzera zvakanyanya nekufamba kwenguva kunogona kuratidza kuti mamiriro ako ari kukura kuita emergency emergency.

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

• Chirwere chemukati chemapapu - vanhu vakuru - kubuda

• Kufuridzira system

Levine SM. Alveolar yekuzadza kusagadzikana. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chits. 85.

Trapnell BC, Luisetti M. Pulmonary alveolar proteinosis chirwere. Mune: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray naNadel Chinyorwa cheKufema Kwemishonga. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016: chits. 70.