Hoʻopilikia ʻia ʻo Busulfan e ka ʻino puʻu: ʻo nā mea āpau āu e ʻike ai e pili ana i ka maʻi maʻamau i ka Sky he Poni

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ʻO ka kiʻi ʻoniʻoni ʻo Bollywood 'Sky Is Pink' i ka nūhou no nā kumu kūpono āpau. Ma muli o ka moʻolelo ola maoli o Aisha Chaudhary, puni ke kiʻi ʻoniʻoni i kahi ʻano olakino olakino, Busulfan i hoʻokomo ʻia i ka ʻeha a i ʻole fibrosis pulmonary ka mea i make ai ʻo Aisha i ka makahiki ʻōpio o 18.

Hānau ʻia me ka immunodeficiency hui nui (SCID), koi ʻia ke kaikamahine ʻōpio e hoʻoliʻiliʻi i ka iwi iwi i 6 mau makahiki, ke kau ʻia ʻo ia ma Busulfan (kahi lāʻau maʻi ʻaʻai. Ua hele ka lāʻau i kumu i ka fibrosis pulmonary kahi hopena ʻē aʻe o ka lāʻau.

Ma mua o ka hoʻomaopopo ʻana i nā mea i hōʻino ʻia e Busulfan i hōʻino ʻia a me kāna mau kikoʻī, e nānā iā SCID.

He aha ka Immunodeficiency (SCID) ʻoi loa.

ʻO kahi maʻi laha ʻole, ʻike ʻia ʻo SCID e ka hoʻomohala kūpilikiʻi o nā pūnaewele T a me nā hunaola B, no ka hopena o nā hoʻololi kūpuna. ʻO ke kumu e hoʻohaʻahaʻa loa ai ke keiki a i ʻole ʻaʻohe pale pilikino iki, e kumu ana i ke kino o ke keiki i nā ʻano maʻi like ʻole ^{[1] [elua]} .

ʻO nā keiki me kēia maʻi laha ʻole, hoʻoilina i lilo i maʻi loa me nā maʻi e like me ka niumonia, meningitis, a me ka moa a hiki ke make i loko o ka makahiki mua o ko lākou ola. Eia nō naʻe, kōkua nā lāʻau lapaʻau o kēia manawa a me nā ʻano hana i ka hoʻomaikaʻi.

ʻO ka hoʻoneʻe iwi iwi ka hana kūpono loa no SCID ^[3] .

pehea e hoola ai i ka lauoho keokeo

No laila, He aha ke ʻano o ka hoʻoneʻe iwi?

ʻO ka hoʻoneʻe ʻana o ka iwi iwi kahi lapaʻau e hoʻololi i ka momona maikaʻi ʻole me kahi mea olakino. ʻO ka iwi o ka iwi ka palupalu, nā ʻāpana spongy i loko o kou mau iwi e hana i nā hunaola koko (nā huna koko) e lilo i mau huna koko e hakakā i nā maʻi, lawe i ka oxygen ma loko o ke kino a kāohi i ke kahe. ^[4] .

Hana ʻia ke transplant e hoʻokomo i nā hunaola iwi momona i loko o ke kanaka ma hope o ka hoʻopau ʻia ʻana o ko lākou iwi iwi maikaʻi ʻole, a he mea maikaʻi ia i ka hoʻomaikaʻi ʻana i ka ʻōnaehana pale o kahi keiki. ^[5] .

Me nā plethora o ke keu pono o ka transplant i ka hoʻomaikaʻi ʻana i ke olakino o kekahi kanaka, loaʻa pū kekahi mau pilikia e like me ka maʻi graft-versus-host, ka hopena o ke koʻokoʻo, nā maʻi, nā maʻi, nā cataract, infertility, nā maʻi ʻaʻai hou a me ka make pū kekahi. ^{[6] [1]} .

ʻO Busulfan kahi lāʻau chemotherapy anti-cancer i hoʻohana ʻia i ke kaʻina transplant iwi. Eia nō naʻe, aia kekahi mau moʻolelo o ka lāʻau i loaʻa ka hopena maikaʻi ʻole i ka poʻe i hana i ke transplant, me ka fibrosis pulmonary ka mea nui loa.

I kēia manawa ua uhi mākou i ka papa, e nānā i ke kumuhana nui, 'Busulfan-induced lung harm' a no ia mea, pono mākou e makaʻala i ke ʻano o Busulfan a pehea e hana ai. ^[7] .

He aha ia ʻo Busulfan?

Hoʻohana ʻia kahi lāʻau lapaʻau e mālama ai i nā maʻi ʻaʻai a luku i ka iwi iwi i makemake ʻole ʻia ma mua o ka hoʻoneʻe ʻana o ka iwi iwi. ʻO ka lāʻau chemotherapy i kahi hui o nā lāʻau i kapa ʻia nā ʻākela alkylating a hana ʻia e ka hoʻolohi ʻana a i ʻole ke kū ʻana i ka ulu ʻana o nā hunaola maʻi ʻaʻai i loko o kou kino. ^[8] . Hoʻohana ʻia ia no ka mālama ʻana i kekahi mau maʻi koko e like me polycythemia vera a me myeloid metaplasia a e mālama i ka leukemia myelogenous mau loa (CML) ^[9] .

Eia nō naʻe, ʻo ka lāʻau chemotherapy, e like me ka mea i ʻōlelo ʻia ma luna, he hopena ʻino ia e hopena ai i ka fibrosis pulmonary i kapa ʻia ʻo Busulfan ^{[9] [10]} .

He aha ka hopena o Busulfan Lung?

ʻO ka busulfan lung, kapa ʻia ma ke ʻano he fibrosis pulmonary e ka lāʻau i hōʻino ʻia a makaʻu ʻia kaʻiʻo māmā. ʻOiai laha ʻole, lethal ke ʻano a hiki ma kahi o 5 kenetene o nā maʻi i mālama ʻia me Busulfan ^{[umikumākahi]} . ʻO ka hōʻike mua o ka hōʻino ʻia o Busulfan lung i hōʻike ʻia ma 1961.

pehea e hoemi ai i na poo keokeo ma ka ihu

Hoʻokumu ke ʻano i nā olonā paʻakikī a paʻakikī hoʻi i loko o nā akemāmā, a i ia manawa he mea paʻakikī ia i ka hanu ʻana o kēlā me kēia.

Akā, e hoʻomaopopo ʻia he hopena ʻē aʻe nā lāʻau ʻē aʻe, e like me ka mea aʻe ^[12] :

• Haemorrhages
• Impotence
• Kāleka
• Hoʻoiho atricular
• Amenorrhoea
• Malformation o fetus
• Sterility
• Pulrosary fibrosis
• Deposition o uricʻakika i nā puʻupaʻa tubules
• Anemia
• Alopecia
• Gynaecomastia

ʻO kekahi o nā hopena ʻoluʻolu o ka lāʻau e like me kēia ^{[umikumākahi]} :

• Kaumaha emi
• Nusea
• Hyperpigmentation o ka ʻili
• ʻO ka luaʻi
• Pohā
• ʻO Rashes
• Ka luhi
• Waha maloo

Pehea e ʻike ʻia ai kahi hōʻino o Busulfan Lung?

Ke noʻonoʻo nei ua hoʻokomo ʻia ke ʻano e kahi lāʻau, paʻakikī e ʻike i ke ʻano. A i ka hapanui o nā hihia, aia ke kūlana i kona pae holomua.

ʻO ke tomography compute hoʻonā kiʻekiʻe (CT Scan) a me ka histopathology nā ʻano kokoke loa e hiki ke kōkua i ke kuhi ʻana i ke ʻano ^[13] . I kekahi mau hihia o ka busulfan lung pohō, ua hōʻike nā diagnoses i kēia:

• Hoʻemi ʻia ke kalapona kalapona (CO) i ka hiki ke hoʻokuʻu
• Hoʻopau i ka hanana hanu ma ka hoʻāʻo ʻana i ka hana pulmonary
• Hiki paha i ka umauma X-ray ke hōʻike i nā kikoʻī a me nā pākuʻi ma nā ʻaoʻao ʻelua o nā māmā
• Ke kūʻana o nā rales
• ʻO ka waihoʻoluʻu polū o nā lehelehe, ka ʻili, a i ʻole nā ​​manamanalima ma muli o ke kiʻekiʻe o ka oxygen pae
• Hoʻonui ākea o ke kumu o nā manamana lima

Ma kahi palapala hope loa ...

Inā hopu ʻia ke ʻano i nā kau mua, ʻoi aku ka maʻalahi o ka mālama ʻana i ka fibrosis pulmonary i hoʻokomo ʻia i loko o ka lāʻau - e like me ka hiki ke hoʻokū ʻia ka lāʻau lapaʻau o kēia manawa a hoʻomaka ʻia kahi lāʻau hou. ^{[14] [umikumālima]} .

Eia nō naʻe, pono e mālama ʻia i nā ʻike hou aʻe e pili ana i ka hoʻomaopopo ʻana i ka papa o ka hopena o ka lāʻau make a me ka hoʻomohala ʻana i kahi lāʻau lapaʻau kūpono no ia mea.

1. [1]De Ravin, S. S., Wu, X., Moir, S., Kardava, L., Anaya-O'Brien, S., Kwatemaa, N., ... & Marquesen, M. (2016). ʻO ka Lentiviral hematopoietic stem cell therapy therapy no ka hoʻopili ʻia ʻana me ka X i hoʻopili ʻia me ka ikaika. Lapaʻau unuhi ʻepekema, 8 (335), 335ra57-335ra57.
2. [elua]Palacios, T. V., Vergales, B., Wisniewski, J., Borish, L., & Lawrence, M. G. (2016). ʻO ke Kulanui o Virginia ʻike ma ka hoʻokō ʻana i ka loiloi hou o nā keiki no ka pale nui i hoʻohui ʻia (SCID). Pai no ka maʻi ʻaʻai a me ka Immunology Haumāna, 137 (2), AB216.
3. [3]Sundin, M., Marits, P., Ramme, K., Kolios, A. G., & Nilsson, J. (2019). ʻO ka immunodeficiency (SCID) hui pū ʻia e hōʻike ana i ka wā kamaliʻi, me ka agammaglobulinemia, e pili pū ana me nā hoʻololi heterozygous hui pū kekahi ma DCLRE1C. ʻO Immunology Haumāna, 200, 16-18.
4. [4]Klein, O. R., Buddenbaum, J., Tucker, N., Chen, A. R., Gamper, C. J., Loeb, D., ... & Holuba, M. J. (2017). Nonmyeloablative haploidentical iwi ʻōiwi me ka post-transplantation cyclophosphamide no nā keiki pediatric a me nā ʻōpio makua me nā maʻi maʻi hematologic kiʻekiʻe. ʻO Biology of Blood and Marrow Transplantation, 23 (2), 325-332.
5. [5]Robinson, T. M., O'Donnell, P. V., Fuchs, E. J., & Luznik, L. (2016, ʻApelila). ʻO ka iwi iwi Haploidentical a me ka transplantation cell stem: ʻike me ka cyclophosphamide post-transplantation. I nā Seminars i ka 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). ʻO ke koʻikoʻi biological o HLA locus e kūlike ana i ka hoʻohuli iwi o ka iwi hāʻawi pili ʻole. Koko, 125 (7), 1189-1197.
7. [7]Ikeda, J., Scipione, C., Hyduk, S., Althagafi, M. G., Gao, X., Jongstra-Bilen, J., & Cybulsky, M. I. (2019). Hoʻopili ka iwi o ka iwi i ka pae mua o ka Atherosclerotic Lesion Formation. Arteriosclerosis, Thrombosis, and 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). ʻO ka nānā ʻana i ka salivary salivary o IV busulfan i nā mea maʻi e hana ana i ka hematopoietic stem cell transplantation: kahi noiʻi pailaka. Ke kuʻi ʻana o ka iwi iwi, 52 (10), 1384.
9. [9]Tutschka, P. J., Copelan, E. A., & Klein, J. P. (1987). ʻO ka hoʻoneʻe iwi iwi no ka leukemia ma hope o ke ʻano hou o ka busulfan a me ka cyclophosphamide. Koko, 70 (5), 1382-1388.
10. [10]ʻO Jain, R., Gupta, K., Bhatia, A., Bansal, A., & Bansal, D. (2017). Hepatic sinusoidal-obstruction maʻi maʻi a me ka busulfan i hoʻokomo ʻia i ka ʻeha ma loko o kahi mea loaʻa transplant cell post-autologous. Nā pediatrics India, 54 (9), 765-770.
11. [umikumākahi]Matijasic, N., Bonevski, A., Tokic Pivac, V., & Pavic, I. (2019). ʻO Busulfan i hōʻeha ʻia e ka maʻi ʻeha ma nā mea maʻi Oncology Pediatric - Nānā i nā puke me kahi hihia kiʻi. Pediatric Allergy, Immunology, a me Pulmonology, 32 (3), 86-91.
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