Burburka Bus-bus-ku-dhaca ee Sambabka: Wax kasta oo aad uga Baahan Tahay Inaad Ka Ogaato Cudurka Naadirka ah ee Cirka ku Yaala Waa Casaan

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Filimka cusub ee Bollywood-ka 'Sky Is Pink' ayaa ku jira warar sababo jira oo dhan. Iyada oo ku saleysan sheekada nolosha dhabta ah ee Aisha Chaudhary, filimku wuxuu ku wareegayaa xaalad caafimaad oo naadir ah, Busulfan oo ay sababtay dhaawac sambabaha ama sambabada oo keenta geerida lama filaanka ah ee Aisha da'da yar ee 18.

Gabadhan yar oo ku dhalatay iyada oo leh isku dheelitir la'aan daran (SCID), ayaa looga baahday in lagu sameeyo qalliin dhuuxa lafta markii ay 6 bilood jirtay, markii la saaray Busulfan (daawo kansar ah). Daroogadu waxay markaa sii waday inay keento cudurka sambabka sambabka oo saameyn dhif ah ku yeesha daawada.

Kahor inta aynaan fahmin waxa Busulfan sababay sambabaha iyo faahfaahintiisa, aan eegno SCID.

Waa Maxay Isku-Darka Isku-Dhafka Daran (SCID)?

Cudur naadir ah oo hidde ah, SCID waxaa lagu gartaa horumarka khalkhalsan ee unugyada T ee shaqeynaya iyo unugyada B, taasoo ka dhalatay is-beddelka hidde-wadaha badan. Xaaladdu waxay ilmaha ku keentaa inuu aad u hooseeyo ama aan lahayn wax difaac ah haba yaraatee, taasoo keenaysa in ilmaha jidhkiisu u nugul yahay noocyo cudurro kala duwan ah ^{[1] [laba]} .

Carruurta qaba cudurkan dhifka ah ee la iska dhaxlo ayaa si aad ah ugu xanuunsada cudurada sida oof wareenka, qoorgooyaha, iyo busbuska waxayna u dhiman karaan sanadka ugu horeeya noloshooda. Si kastaba ha noqotee, daawooyinka casriga ah iyo hababka daaweynta ayaa gacan ka geystay hagaajinta.

Beerista dhuuxa lafta ayaa ah daaweynta ugu waxtarka badan ee SCID ^[3] .

Marka, Waa Maxay Lafta Lafta?

Beerista dhuuxa lafta ayaa ah daaweyn lagu badalayo dhuuxa aan caafimaadka qabin loona badalo mid caafimaad qaba. Dhuuxa lafta waa nudaha jilicsan ee jilicsan ee ku dhex jira lafahaaga kaasoo sameeya unugyada dhiiga sameeya (unugyada asliga ah ee dhiiga) oo isu badala unugyada dhiiga si ay ula dagaalamaan infekshanada, waxay qaadaan oksijiinka jirka oo dhan waxayna xakameeyaan dhiigbaxa ^[4] .

Beerista xubinta jirka waxaa loo sameeyaa si loogu shubo unugyada dhuuxa lafta caafimaad qabta qofka ka dib marka la tirtiro dhuuxooda aan caafimaadka qabin, waxayna waxtar u leedahay hagaajinta habka difaaca jirka ee cunugga ^[5] .

Marka lagu daro faa iidooyinka badan ee laga helayo xubinta taranka ee lagu hagaajinayo caafimaadka qofka, waxay sidoo kale leedahay dhibaatooyin badan sida cudurada kala-soo horjeedka-martida, unugyada unugyada unugyada unugyada jirka, caabuqyada indhaha, indha la'aanta, kansarrada cusub iyo xitaa dhimashada. ^{[6] [1]} .

Busulfan waa dawo kahortaga kansarka dawada loo isticmaalo habka tallaalka dhuuxa lafta. Si kastaba ha noqotee, waxaa jiray warbixino sheegaya in daroogadu ay saameyn xun ku yeelatay shaqsiyaadkii lagu tallaalay, iyadoo cudurka sambabka ee sambabka uu yahay midka ugu daran.

Hadda oo aan soo koobnay sababaha, aan eegno dulucda udub dhexaad u ah, 'Burburka uu sababo sambabka Busulfan' taasna, waxaan u baahan nahay inaan ka warqabno waxa uu yahay Busulfan iyo sida ay u shaqeyso ^[7] .

Waa maxay Busulfan?

Waa daawo rijeeto ah oo loo qoray in lagu daaweeyo kansarrada laguna baabi'iyo dhuuxa lafta aan loo baahnayn kahor tallaalka dhuuxa lafta. Daawada kiimoteraabiga waxaa iska leh koox daroogo ah oo loo yaqaan wakiilada alkylating waxayna ku shaqaysaa adoo yareeya ama joojiya koritaanka unugyada kansarka ee jirkaaga. ^[8] . Waxaa sidoo kale loo isticmaalaa daaweynta cudurada dhiigga qaarkood sida polycythemia vera iyo myeloid metaplasia iyo in lagu daaweeyo myelogenous leukemia (CML) ^[9] .

Si kastaba ha noqotee, daweynta kemotherabi, sida aan horay u soo sheegnay waxay leedahay saameyn xun oo qabad leh oo sababa fibrosis sambabada oo loo yaqaan 'Busulfan-sababtoo ah dhaawaca sanbabada ^{[9] [10]} .

Waa Maxay Dhaawaca Sambabka Busulfan?

Sambabka Busulfan, oo caafimaad ahaan loogu yeero fibrosis sambabka waxaa sababa daawada halka unugyada sambabaha ay ku waxyeeloobaan kuna yeeshaan nabar. In kasta oo ay dhif tahay, xaaladdu waa dilaa waxayna ku dhacdaa ugu yaraan boqolkiiba 5 bukaannada lagu daweeyay Busulfan ^{kow iyo toban} . Warbixintii ugu horreysay ee waxyeellada sambabka Busulfan waxaa la soo sheegay 1961.

Xaaladdu waxay keentaa fiilooyin adag oo adag oo sambabaha laga sameeyo, taas oo iyaduna qofka ku adkeyneysa inuu neefsado.

Laakiin, waa in la ogaadaa in daroogadu leedahay waxyeelooyin kale oo daran, sida kuwa soo socda ^[12] :

• Dhiigbax
• Awood daro
• Cataract
• Atrophy xiniinyaha
• Amenorrhoea
• Cilad ku timaada uurjiifka
• Madhalaysnimada
• Fibroowga sambabada
• Ku shubista uric acid ee tuubbooyinka kelyaha
• Dhiig yaraan
• Alopecia
• Gynaecomastia

Qaar ka mid ah waxyeelooyinka fudud ee daawadu waa sida soo socota ^{kow iyo toban} :

• Miisaanka oo yaraada
• Lallabbo
• Dhiirrigelinta maqaarka
• Matagid
• Shuban
• Rashes
• Daal
• Af qalalan

Sidee Lagu Ogaadaa Burburka Sambabka Burburka?

Iyadoo la tixgelinayo in xaaladda ay sababtay daawo, way adag tahay in la ogaado xaaladda. Xaaladaha badankoodna, xaaladdu waxay ku jiri doontaa heerkeedii hore.

Xallinta sare ee sawir-qaadista xisaabinta (CT Scan) iyo histopathology waa hababka ugu dhow ee ka caawin kara baaritaanka xaaladda ^[13] . Xaaladaha qaarkood ee waxyeellada sanbabada ee Busulfan, baadhitaanada la sameeyay ayaa muujiyay waxyaabaha soo socda:

• Kaarboon monoksaydh (CO) oo la dhimay awoodeeda sii deynta
• Qaabka neefsashada xaddidan ee baaritaanka shaqada sambabada
• Raajada feeraha ayaa laga yaabaa inay muujiso dhibco iyo balastarro labada dhinac ee sanbabada ah
• Joogitaanka raajada
• Midabka buluuga ah ee bushimaha, maqaarka, ama ciddiyaha cidiyaha sababo la xiriira heerarka oksijiinta dhiigga oo hooseeya
• Kordhinta aan caadiga ahayn ee saldhiga ciddiyaha

Xusuusin Dhameystiran ...

Haddii xaaladdu ku dhacdo marxaladaha hore, way isbarbardhigi kartaa si fudud maareynta daroogada keenta cudurka fibrosis - maadaama daaweynta daroogada ee hadda jirta la joojin karo oo daawo cusub la bilaabi karo ^{[14] [shan iyo toban]} .

Si kastaba ha noqotee, daraasado dheeri ah waa in lagu qabtaa fahamka habka dawada dhimashada keentay iyo in la horumariyo daaweyn wax ku ool ah oo isku mid ah.

1. [1]De Ravin, S. S., Wu, X., Moir, S., Kardava, L., Anaya-O'Brien, S., Kwatemaa, N., ... & Marquesen, M. (2016). Daaweynta hidda-wadaha unugyada dhiiga ee Lentiviral hematopoietic ee loogu talagalay X-la xiriirnaanta yaraanta daran ee isku-dhafan. Daawada tarjumaada sayniska, 8 (335), 335ra57-335ra57.
2. [laba]Palacios, T. V., Vergales, B., Wisniewski, J., Borish, L., & Lawrence, M. G. (2016). Jaamacadda Virginia waxay khibrad u leedahay hirgelinta baaritaanka dhallaanka cusub ee isku-filnaanshaha yaraanta jirka (SCID). Wargeyska Xasaasiyadda iyo Immunology Clinical, 137 (2), AB216.
3. [3]Sundin, M., Marits, P., Ramme, K., Kolios, A. G., & Nilsson, J. (2019). Isugeyn la'aanta jirka ee daran (SCID) oo soo bandhigeysa caruurnimada, oo leh agammaglobulinemia, oo laxiriira isbadal heterozygous kujira DCLRE1C. Immunology Clinical, 200, 16-18.
4. [4]Klein, O. R., Buddenbaum, J., Tucker, N., Chen, A. R., Gamper, C. J., Loeb, D., ... & Holuba, M. J. (2017). Ku-tallaalidda laf-dhabarka laf-dhabarka ee haweenta ah ee loo yaqaan 'nonmyeloablative haploidentical dhrow' oo lagu beddelayo tallaalidda 'cyclophosphamide' kadib ee loogu talagalay bukaanka carruurta iyo dhallinyarada qaangaarka ah ee leh halista sare ee dhiig-baxa dhiig-baxa. Bayoolaji Dhiigga iyo Dhuuxa Goynta, 23 (2), 325-332.
5. [5]Robinson, T. M., O'Donnell, P. V., Fuchs, E. J., & Luznik, L. (2016, Abriil). Dhuuxa lafta Haploidentical iyo xubinta unugyada unugyada unugyada unugyada: khibrad leh ku-tallaalidda ku-tallaalidda 'cyclophosphamide' kadib. Seminaaro ku saabsan cilmiga dhiigga (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). Muhiimadda bayoolojiga ee isku dheelitirka goobta HLA ee ku-tallaalida dhuuxa dhuuxa laf-dhabarta. Dhiig, 125 (7), 1189-1197.
7. [7]Ikeda, J., Scipione, C., Hyduk, S., Althagafi, M. G., Gao, X., Jongstra-Bilen, J., & Cybulsky, M. I. (2019). Beerista Lafta Dhuuxa waxay saameyn ku yeelaneysaa Marxaladda ugu horreysa ee Samaynta Qalitaanka Atherosclerotic. Arteriosclerosis, Thrombosis, iyo Bayoolaji Vascular, 39 (Suppl_1), A534-A534.
8. [8]Bezinelli, L. M., Eduardo, F. P., De Carvalho, D.LC, dos Santos Ferreira, C. E., De Almeida, E. V., Sanches, L. R., ... & Corrêa, L. (2017). Kormeerka salivary-ka daaweynta ee IV busulfan ee bukaanada maraya bedelka unugyada unugyada dhiiga ee hematopoietic: daraasad tijaabo ah. Beerista dhuuxa lafta, 52 (10), 1384.
9. [9]Tutschka, P. J., Copelan, E. A., & Klein, J. P. (1987). Beerista lafta ee leukemia kadib nidaamka cusub ee busulfan iyo cyclophosphamide. Dhiig, 70 (5), 1382-1388.
10. [10]Jain, R., Gupta, K., Bhatia, A., Bansal, A., & Bansal, D. (2017). Cudurka cagaarshowga-xannibaadda cagaarshowga iyo dhaawaca sanbabada ee busulfan-ku dhaca ee qaata qaybta unugyada unugyada unugyada dambe ee gadaal-autologous. Dhakhaatiirta caafimaadka Hindiya, 54 (9), 765-770.
11. kow iyo tobanMatijasic, N., Bonevski, A., Tokic Pivac, V., & Pavic, I. (2019). Dhaawaca Nabarrada Busulfan-ee Bukaan-jiifka Bukaanka Oncology-Dib-u-eegista suugaanta oo leh kiis sawir leh. Xasaasiyadda carruurta, Immunology, iyo Pulmonology, 32 (3), 86-91.
12. [12]Mourad, M., & Crosser, M. S. (2016). A48 CUDURKA SHAQADA SHAQADA LAMBARKA LA XIRIIRA: Sambabka oo Faafiya Ka Dib Qalitaanka Qalitaanka Stem Peripheral Stem, Maaha Had iyo jeer Oof-wareenka Infekshinka Leh. Xaaladda Dhaawaca Sambabada ee Busulfan Joornaalka Mareykanka ee Neefsashada iyo Daryeelka Khatarta ah, 193, 1.
13. [13]Rix, A., Drude, N. I., Mrugalla, A., Baskaya, F., Pak, K. Y., Gray, B., ... & Mottaghy, F. M. (2019). Qiimeynta Burburka Noolaha ee Kiimoteraabiga leh Ga-68 oo sumaddiisu tahay Duramycin. Sawirka Molecular iyo Biology, 1-11.
14. [14]Themanns, M., Koban, F., Bergmayr, C., Chrzan, A., Strohmaier, W., Haybaeck, J., ... & Zebedin-Brandl, E. (2019). Treprostinil wuxuu yareeyaa dhaawaca endothelial ee murine sinusoidal obstruction syndrome. Joornaalka Daawada Molecular, 97 (2), 201-213.
15. [shan iyo toban]Averyanov, A., Kogan, E., & Lesnyak, V. (2020). Cudurada sambabada ee dawadu keento. Way Ku Adag Tahay Inaad Baadho Cudurka Sambabka ee Faafida Loo Yaqaano (pp. 393-408). Saxaafadda Tacliinta.

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