Kuwonongeka kwa Mapapu Opangidwa ndi Busulfan: Chilichonse Chimene Muyenera Kudziwa Zokhudza Matenda Osawerengeka Omwe Ali Kumwamba Ndi Pinki

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Kanema watsopano wa Bollywood 'Sky Is Pink' ali munyuzi pazifukwa zonse zoyenera. Kutengera ndi mbiri yeniyeni ya Aisha Chaudhary, kanemayo adazungulira matenda osowa kwambiri, kuwonongeka kwamapapo kapena Busulfan komwe kumayambitsa kufa kwa Aisha ali ndi zaka 18.

Wobadwa ndi matenda ophatikizika amthupi (SCID), msungwanayo amayenera kukapatsidwa fupa lamfupa ali ndi miyezi isanu ndi umodzi, pomwe adayikidwa Busulfan (mankhwala a khansa). Mankhwalawa anapitilizabe kuyambitsa pulmonary fibrosis yomwe ndi zotsatira zoyipa za mankhwalawo.

Tisanamvetsetse kuwonongeka kwamapapu komwe kumayambitsa Busulfan ndi tsatanetsatane wake, tiyeni tiwone SCID.

Kodi Kuphatikizidwa Kwambiri Kwambiri (SCID) N'kutani?

Matenda achilendo, SCID amadziwika ndi kukula kwakusokonekera kwa ma T maselo ndi ma B, chifukwa cha kusintha kwamitundu yambiri. Vutoli limapangitsa kuti mwana akhale wotsika kwambiri kapena asatetezedwe konse, ndikupangitsa kuti thupi la mwanayo lizitha kudwala matenda osiyanasiyana ^{[1] [ziwiri]} .

Ana omwe ali ndi matenda obadwa nawowa, amadwala kwambiri matenda monga chibayo, meningitis, ndi katsabola ndipo amatha kufa mchaka choyamba cha moyo wawo. Komabe, mankhwala amakono ndi njira zamankhwala zathandizira kusintha.

Kuika mafuta m'mafupa ndi mankhwala othandiza kwambiri ku SCID ^[3] .

Kotero, Kodi Kujambula Mafupa A Bone Ndi Chiyani?

Kuthira mafuta m'mafupa ndi mankhwala omwe amalowetsa m'malo mwake moyenera. Mafupa a mafupa ndi minofu yofewa, yamatope mkati mwa mafupa anu yomwe imapanga maselo opangira magazi (maselo amwazi) omwe amasandulika maselo amwazi olimbana ndi matenda, kunyamula mpweya mthupi lonse ndikuthana ndi magazi ^[4] .

Kuika kumachitika kuti alowetse m'mafupa amthupi mwa munthu munthu atachotsa m'mafupa, ndipo ndiwothandiza pakukweza chitetezo cha mwana ^[5] .

Pamodzi ndi kuchuluka kwa mapindu omwe kubalikirako kumakulitsa thanzi la munthu, kumakhalanso ndi zovuta zingapo monga matenda olumikizidwa motsutsana ndi omwe amakhala nawo, kulephera kwa ma cell, kuwonongeka kwa ziwalo, matenda, nthenda, kusabereka, khansa yatsopano komanso imfa ^{[6] [1]} .

Busulfan ndi mankhwala a anti-cancer chemotherapy omwe amagwiritsidwa ntchito pokonza m'mafupa. Komabe, pakhala pali malipoti onena za mankhwalawa omwe adasokoneza anthu omwe adamuika, ndi pulmonary fibrosis yomwe ili yovuta kwambiri.

Tsopano popeza tapeza malowa, tiyeni tiwone mutu wapakati, 'Kuwonongeka kwamapapu komwe kumayambitsa Busulfan' ndipo chifukwa cha izi, tiyenera kudziwa zomwe Busulfan ndi momwe zimagwirira ntchito ^[7] .

Kodi Busulfan ndi chiyani?

Ndi mankhwala akuchipatala omwe amagwiritsidwa ntchito kuthana ndi khansa ndikuwononga mafupa osafunikira musanalowe mafupa. Mankhwala a chemotherapy ndi a gulu la mankhwala omwe amatchedwa alkylating agents ndipo amagwira ntchito pochepetsa kapena kuletsa kukula kwa maselo a khansa mthupi lanu ^[8] . Amagwiritsidwanso ntchito pochiza matenda ena amwazi monga polycythemia vera ndi myeloid metaplasia ndikuchiza khansa ya m'magazi (CML) ^[9] .

Komabe, mankhwala a chemotherapy, monga tafotokozera pamwambapa, ali ndi vuto lalikulu lomwe limayambitsa pulmonary fibrosis yomwe imadziwika kuti kuwonongeka kwamapapu komwe kumachitika ndi Busulfan ^{[9] [10]} .

Kodi Kuwonongeka kwa Mapulani a Busulfan N'kutani?

Mapapu a Busulfan, omwe amadziwika kuti pulmonary fibrosis amayamba chifukwa cha mankhwala omwe minofu ya m'mapapo imawonongeka komanso imakhala ndi zipsera. Ngakhale ndizosowa, vutoli limakhala lakupha ndipo limachitika mwa ochepera 5% ya odwala omwe adathandizidwa ndi Busulfan ^{[khumi ndi chimodzi]} . Ripoti loyamba la kuwonongeka kwamapapo a Busulfan lidanenedwa mu 1961.

Vutoli limapangitsa kuti ulusi wolimba ndi wolimba upangidwe m'mapapu, zomwe zimapangitsa kuti zikhale zovuta kuti munthu apume.

Koma, ziyenera kudziwika kuti mankhwalawa ali ndi zovuta zina zoyipa, monga izi ^[12] :

• Kutaya magazi
• Mphamvu
• Katemera
• Mayeso a atrophy
• Khalidwe
• Kusokonezeka kwa mwana wosabadwayo
• Wofooka
• Matenda a m'mapapo mwanga
• Kuyika kwa uric acid m'matope a impso
• Kuchepa kwa magazi m'thupi
• Alopecia
• Gynaecomastia

Zina mwa zovuta zoyipa za mankhwala ndi awa ^{[khumi ndi chimodzi]} :

• Kuchepetsa thupi
• Nseru
• Hyperpigmentation khungu
• Kusanza
• Kutsekula m'mimba
• Ziphuphu
• Kutopa
• Pakamwa pouma

Kodi Kuwonongeka kwa Mapafu a Busulfan Kumadziwika Bwanji?

Poganizira kuti vutoli limayambitsidwa ndi mankhwala, ndizovuta kuzindikira vutoli. Ndipo nthawi zambiri, vutoli limakhala likupita patsogolo.

High-resolution computed tomography (CT Scan) ndi histopathology ndi njira zoyandikira kwambiri zomwe zingathandize kuzindikira vutoli ^[13] . Nthawi zina kuwonongeka kwa m'mapapo a Busulfan, matendawa awulula izi:

• Kuchepetsa kutulutsa kwa carbon monoxide (CO)
• Njira yoletsa kupuma poyesa m'mapapo mwanga
• X-ray pachifuwa imatha kuwonetsa mawanga osakanikirana mbali zonse zamapapu
• Kukhalapo kwa rales
• Mtundu wabuluu wa milomo, khungu, kapena zikhadabo chifukwa chotsika magazi
• Kukulitsa kwachilendo pamunsi mwa zikhadabo

Pamapeto pake ...

Ngati vutoli layambika koyambirira, ndizosavuta kuyang'anira mankhwala osokoneza bongo am'mapapo - popeza mankhwala omwe alipo pakadali pano atha kuyimitsidwa ndipo mankhwala atsopano atha kuyambitsidwa ^{[14] [khumi ndi zisanu]} .

Komabe, maphunziro ochulukirapo amayenera kuchitidwa kuti amvetsetse zomwe zimayambitsa mankhwala omwe amapha ndikupanga chithandizo chamankhwala chimodzimodzi.

1. [1]De Ravin, S. S., Wu, X., Moir, S., Kardava, L., Anaya-O'Brien, S., Kwatemaa, N., ... & Marquesen, M. (2016). Mankhwala a Lentiviral hematopoietic stem cell gene a X-yolumikizidwa kwambiri ophatikizika ndi immunodeficiency. Sayansi yotanthauzira yasayansi, 8 (335), 335ra57-335ra57.
2. [ziwiri]Palacios, T. V., Vergales, B., Wisniewski, J., Borish, L., & Lawrence, M. G. (2016). Chidziwitso cha University of Virginia pakukhazikitsa kuwunika kwa ana akhanda kuti awoneke ngati ali ndi chitetezo chamagulu (SCID). Zolemba pa Ziwengo ndi Clinical Immunology, 137 (2), AB216.
3. [3]Sundin, M., Marits, P., Ramme, K., Kolios, A. G., & Nilsson, J. (2019). Kuwonjezeka kophatikizana kwa immunodeficiency (SCID) komwe kumachitika muubwana, ndi agammaglobulinemia, yolumikizidwa ndi kusintha kwakapangidwe kake ka heterozygous ku DCLRE1C. Matenda a Immunology, 200, 16-18.
4. [4]Klein, O. R., Bainlessbaum, J., Tucker, N., Chen, A. R., Gamper, C. J., Loeb, D., ... & Holuba, M. J. (2017). Kusintha kwa mafupa osakanikirana ndi mafupa osakanikirana ndi post-kumuika cyclophosphamide kwa ana komanso achikulire omwe ali ndi ziwopsezo zowopsa za hematologic. Biology Yakuika Magazi ndi Marrow, 23 (2), 325-332.
5. [5]Robinson, T. M., O'Donnell, P. V., Fuchs, E. J., & Luznik, L. (2016, Epulo). Mafupa a mafupa osadziwika bwino komanso kupatsira maselo am'madzi: zomwe zimachitika atapitanso patsogolo pa cyclophosphamide. M'masemina mu hematology (Vol. 53, No. 2, pp. 90-97). WB Saunders.
6. [6]Morishima, Y., Kashiwase, K., Matsuo, K., Azuma, F., Morishima, S., Onizuka, M., ... & Mori, T. (2015). Kufunika kwachilengedwe kwa HLA locus yofananira ndi kupatsirana kwa mafupa opereka osagwirizana. Magazi, 125 (7), 1189-1197.
7. [7]Ikeda, J., Scipione, C., Hyduk, S., Althagafi, M. G., Gao, X., Jongstra-Bilen, J., & Cybulsky, M. I. (2019). Kusintha Kwa Bone Marrow Kumakhudza Gawo Lakale Kwambiri la Mapangidwe a Atherosclerotic Lesion. Arteriosclerosis, Thrombosis, ndi Vascular Biology, 39 (Suppl_1), A534-A534.
8. [8]Bezinelli, L. M., Eduardo, F. P., De Carvalho, D. L. C., dos Santos Ferreira, C. E., De Almeida, E. V., Sanches, L. R., ... & Corrêa, L. (2017). Kuwunika kwapadera kwa IV busulfan mwa odwala omwe ali ndi ma hematopoietic stem cell kumuika: kafukufuku woyendetsa ndege. Kuika mafuta m'mafupa, 52 (10), 1384.
9. [9]Tutschka, P. J., Copelan, E. A., & Klein, J. P. (1987). Kuika mafuta m'mafupa a khansa ya m'magazi kutsatira busulfan yatsopano ndi cyclophosphamide regimen. Magazi, 70 (5), 1382-1388.
10. [10]Jain, R., Gupta, K., Bhatia, A., Bansal, A., & Bansal, D. (2017). Matenda a hepatic sinusoidal-obstruction ndi busulfan-omwe amachititsa kuti mapapu avulazidwe pambuyo pake. Ana achimwenye, 54 (9), 765-770.
11. [khumi ndi chimodzi]Matijasic, N., Bonevski, A., Tokic Pivac, V., & Pavic, I. (2019). Kuvulala kwa Mapapu a Busulfan mu Matenda Oncology Odwala-Kubwereza Zolemba ndi Nkhani Yosonyeza. Matenda a Ana, Immunology, ndi Pulmonology, 32 (3), 86-91.
12. [12]Mourad, M., & Crosser, M. S. (2016). A48 MITU YA NKHANI YOPHUNZITSIDWA YOPHUNZITSIDWA NDI MATENDA A MPHAMVU: M'mapapo mwanga Amalowerera Kutsata Kuphatika kwa Maselo Ozungulira, Osati Nthawi Zonse Chibayo. Mlandu Wa Kuvulala kwa Pulmonary Yopangidwa ndi Busulfan. American Journal of Kupuma ndi Ovuta Kusamalira Mankhwala, 193, 1.
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14. [14]Themanns, M., Koban, F., Bergmayr, C., Chrzan, A., Strohmaier, W., Haybaeck, J., ... & Zebedin-Brandl, E. (2019). Treprostinil imachepetsa kuwonongeka kwa endothelial mu murine sinusoidal obstruction syndrome. Zolemba pa Molecular Medicine, 97 (2), 201-213.
15. [khumi ndi zisanu]Averyanov, A., Kogan, E., & Lesnyak, V. (2020). Matenda am'mapapo. Zovuta Kuzindikira Matenda Aakulu Opatsirana a M'mapapo (pp. 393-408). Nkhani Zaphunziro.

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