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Ikhaya Ezempilo Ukwelashwa kokuphazamiseka Ukuphazamiseka Ukwelapha oi-Amritha K By U-Amritha K. ngo-Okthoba 15, 2019

Ibhayisikobho entsha ye-Bollywood ethi 'Sky Is Pink' isematheni ngazo zonke izizathu ezifanele. Ngokuya ngendaba engokoqobo ka-Aisha Chaudhary, i-movie igxile esimweni sezempilo esingajwayelekile, ukulimala kwamaphaphu okwenziwe yiBusulfan noma i-pulmonary fibrosis edale ukufa okungazelelwe kuka-Aisha esemncane eneminyaka engu-18.

Izalwe inokukhubazeka okukhulu komzimba okuhlangene (i-SCID), le ntombazanyana kwakudingeka ifakelwe umongo wethambo inezinyanga eziyisithupha ubudala, ngenkathi ifakwa kuBusulfan (umuthi womdlavuza). Lesi sidakamizwa sabe sesiqhubeka sidala i-pulmonary fibrosis okuwumphumela wecala ongajwayelekile womuthi.

Ngaphambi kokuqonda ukuthi yikuphi ukulimala kwamaphaphu okubangelwa yiBusulfan nemininingwane yayo, ake sibheke i-SCID.

Kuyini Ukuqina Okuqinile Okuhlanganisiwe (i-SCID)?

Isifo esingajwayelekile sezakhi zofuzo, i-SCID ibonakala ngokuthuthuka okuphazamisayo kwamaseli we-T asebenzayo namaseli we-B, njengomphumela wezinguquko eziningi zofuzo. Lesi simo sidala ukuthi ingane ivikeleke kakhulu noma ingabi nokuvikela nhlobo, okwenza umzimba wengane uthambekele ezinhlotsheni ezahlukahlukene zezifo ^[1] ^[okubili] .

Izingane ezinalesi sifo esingajwayelekile, esizuzwe njengefa zigula kakhulu ngezifo ezinjenge-pneumonia, meningitis, nenkukhu futhi zingafa kungakapheli unyaka wokuqala wokuphila kwazo. Kodwa-ke, izindlela zokwelapha zanamuhla nezindlela zokwelashwa zisize ukuthuthuka.

Ukufakelwa komnkantsha wethambo kuyindlela ephumelela kunazo zonke yokwelapha i-SCID ^[3] .

Ngakho-ke, Kuyini Ukufakelwa Komongo Wethambo?

Ukufakelwa komnkantsha wethambo kuwumuthi obuyisela umongo ongenampilo ngomuthi ophilile. Umnkantsha wethambo izicubu ezithambile, ezinesiponji ngaphakathi kwamathambo akho ezenza amangqamuzana akha igazi (amangqamuzana egazi egazi) aphenduka amaseli egazi ukulwa nezifo, athwale umoya-mpilo emzimbeni wonke futhi alawule ukopha ^[4] .

Ukufakelwa kwenziwa ukufakela amangqamuzana omnkantsha anempilo kumuntu ngemuva kokuba umnkantsha wakhe ongenampilo ususiwe, futhi kuyasiza ekuthuthukiseni amasosha omzimba engane ^[5] .

Kanye nenqwaba yezinzuzo ukufakelwa okunakho ekuthuthukiseni impilo yomuntu, kubuye kube nezinkinga eziningi ezinjengokuxhunyelelwa kwesifo sokuxhunyelelwa, ukuhluleka kwamaseli we-stem, ukulimala komzimba, ukutheleleka, i-cataract, ukungazali, umdlavuza omusha ngisho nokufa ^[6] ^[1] .

I-Busulfan iyisidakamizwa sokulwa nomdlavuza sokulwa nomdlavuza esisetshenziswa kwinqubo yokufakelwa komnkantsha wethambo. Kodwa-ke, kuye kwaba nemibiko yokuthi lesi sidakamizwa sinomthelela omubi kubantu abebefakelwe ukufakelwa, i-pulmonary fibrosis iyona ebucayi kakhulu.

Manje njengoba sesizibekele izizathu, ake sibheke ingqikithi emaphakathi, 'Ukulimala kwamaphaphu okubangelwa yiBusulfan' futhi ngalokho, kudingeka sazi ukuthi yini uBusulfan nokuthi isebenza kanjani ^[7] .

Kuyini iBusulfan?

Kungumuthi kadokotela osetshenziselwa ukwelapha umdlavuza nokubhubhisa umnkantsha ongafuneki ngaphambi kokufakelwa komnkantsha wethambo. Umuthi wamakhemikhali ungoweqembu lezidakamizwa ezibizwa ngokuthi ama-alkylating agents futhi usebenza ngokwehlisa noma ukumisa ukukhula kwamangqamuzana omdlavuza emzimbeni wakho ^[8] . Ibuye isetshenziselwe ukwelapha ukuphazamiseka okuthile kwegazi njenge-polycythemia vera kanye ne-myeloid metaplasia kanye nokwelapha i-leukemia engapheli emithambo yegazi (CML) ^[9] .

Kodwa-ke, umuthi we-chemotherapy, njengoba kushiwo ngenhla unomphumela omubi kakhulu odala i-pulmonary fibrosis ebizwa ngokuthi ngumonakalo wamaphaphu owenziwe nguBusulfan ^[9] ^[10] .

Kuyini Ukulimala KweBusulfan Lung?

I-Busulfan lung, ebizwa ngokomtholampilo njenge-pulmonary fibrosis ibangelwa yimithi lapho izicubu zamaphaphu zilimala futhi zibe nesibazi. Yize kungavamile, lesi simo siyabulala futhi sivela ezingaphansi kwamaphesenti ama-5 eziguli ezelashwa ngeBusulfan ^{[ishumi nanye]} . Umbiko wokuqala wokulimala kwamaphaphu eBusulfan wabikwa ngo-1961.

Lesi simo sidala ukuthi kwakheke imicu eqinile futhi eqinile emaphashini, okwenza kube nzima ukuthi umuntu aphefumule.

Kepha, kufanele kuqashelwe ukuthi umuthi uneminye imiphumela emibi kakhulu, efana nale elandelayo ^[12] :

Ukopha kakhulu
Ukungabi namandla
I-cataract
I-atrophy yamasende
Okuhle
Ukulungiswa kombungu
Inzalo
I-pulmonary fibrosis
Ukufakwa kwe-uric acid kuma-tubules ezinso
Ukushoda kwegazi
I-Alopecia
Gynaecomastia

Eminye yemiphumela emibi yomuthi imi kanjena ^{[ishumi nanye]} :

Ukwehla kwesisindo
Isicanucanu
Hyperpigmentation kwesikhumba
Ukuhlanza
Uhudo
AmaRash
Ukukhathala
Umlomo owomile

Kutholakala Kanjani Ukulimala KweBusulfan Lung?

Uma ucabanga ukuthi lesi simo sidalwa umuthi, kunzima ukuthola isimo. Futhi ezimweni eziningi, isimo sizoba sesigabeni saso esithuthukile.

I-high-resolution computed tomography (CT Scan) ne-histopathology yizindlela eziseduze kakhulu ezingasiza ukuxilonga lesi simo ^[13] . Kwezinye izimo zomonakalo wamaphaphu weBusulfan, ukuxilongwa kuveze okulandelayo:

Umthamo okhipha i-carbon monoxide (CO)
Iphethini yokuphefumula evinjelwe ekuhlolweni komsebenzi wamaphaphu
I-X-ray yesifuba ingakhombisa izindawo ezingahleliwe namachashazi ezinhlangothini zombili zamaphaphu
Ukuba khona kwama-rales
Umbala oluhlaza okwesibhakabhaka wezindebe, isikhumba, noma izinzipho ngenxa yamazinga aphansi e-oxygen egazini
Ukwandiswa okungavamile kwesisekelo sezinzipho

Encwadini Yokugcina ...

Uma isimo sibanjwa ezigabeni zokuqala, kuba lula ngokuqhathaniswa ukuphatha i-pulmonary fibrosis eyenziwe yizidakamizwa - njengoba ukwelashwa kwezidakamizwa kwamanje kungamiswa futhi kungaqalwa umuthi omusha ^[14] ^{[ishumi nanhlanu]} .

Kodwa-ke, kufanele kwenziwe izifundo eziningi ekuqondeni inkambo yesimo esibulalayo esenziwa yimithi nokuthuthukisa ukwelashwa okuphumelelayo okufanayo.

Buka Izinkomba ze-Article

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[4]UKlein, O. R., Bainlessbaum, J., Tucker, N., Chen, A. R., Gamper, C. J., Loeb, D., ... & Holuba, M. J. (2017). Ukufakelwa komnkantsha we-nonmyeloablative haploidentical bone nge-post-transplantation cyclophosphamide yeziguli zezingane kanye nabantu abadala abanobungozi be-hematologic malignancies. I-Biology Yokufakelwa Kwegazi Nomongo, 23 (2), 325-332.
[5]URobinson, T. M., O'Donnell, P. V., Fuchs, E. J., & Luznik, L. (2016, Ephreli). Umnkantsha we-haploidentical bone kanye ne-stem cell transplantation: isipiliyoni nge-post-transplantation cyclophosphamide. Ezingqungqutheleni ze-hematology (Umq. 53, No. 2, amakhasi 90-97). Ama-WB Saunders.
[6]UMorishima, Y., Kashiwase, K., Matsuo, K., Azuma, F., Morishima, S., Onizuka, M., ... & Mori, T. (2015). Ukubaluleka kwezinto eziphilayo ngokufana kwe-HLA locus ekufakelweni komongo wethambo okungahlobene. Igazi, 125 (7), 1189-1197.
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