BUSULFAN-INDUCED LUNG DAMAGE: MEA UMA E TATAU ONA E ILOA E UIGA I LE VAVEGA FAAMAʻI I LE LAGI E LANUMOLI - PAMPEREDPEOPLENY.COM

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Aua le misia

Fale Soifua Maloloina Faamaloloina faaletonu Faʻaletonu Fofo oi-Amritha K E Amritha K. ia Oketopa 15, 2019

O le ata tifaga fou o le 'Sky Is Pink' o loʻo i tala fou mo mafuaʻaga tonu uma. Faʻavae i luga o le tala moni o le olaga o Aisha Chaudhary, o le tifaga e faʻataʻamilomilo i se tulaga le masani ai faʻafomaʻi, Busulfan-mafua ai mama afaina po o pulmonary fibrosis na mafua ai le vave maliu o Aisha i le talavou tausaga o 18.

Fanau mai ma le tuʻufaʻatasia o le puipuia o le tino (SCID), sa manaʻomia le teineititi e faia se ponaivi o le ponaivi i le 6 masina le matua, ina ua tuʻuina ia Busulfan (o le kanesa fualaʻau). Ona alu ai lea o le vailaʻau e mafua ai le pulmonary fibrosis ose aafiaga seasea o vailaʻau.

Ae tatou te leʻi oʻo i le malamalama pe o le a le Busulfan-faʻatosina mama afaina ma ona auiliiliga, seʻi tatou vaʻai i le SCID.

O le a le Severe Combined Immunodeficiency (SCID)?

O se faʻalavelave e seasea tupuʻa, o le SCID e faʻamatalaina e le faʻaletonu o le atinaʻe o sela T ma sela B, o se faʻaiuga o le tele o suiga o ituaiga. O le tulaga e mafua ai le tamaititi i le maualalo tele pe leai foi se puipuiga i uma, mafua ai le tino o le tamaititi e maua i le tele o ituaiga o faamai ^[1] ^[lua] .

O tamaiti e maua i lenei faʻamaʻi masani, maʻi ua maua mai ua mamaʻi lava i maʻi pei o le niumonia, fiva faiʻai, ma tanesusu ma e mafai ona feoti i totonu o le tausaga muamua o lo latou olaga. Peitaʻi, o vailaʻau faʻaonapo nei ma togafiti togafitiga ua fesoasoani i se faʻaleleia atili.

Ole togafitiga ole gaʻo ole togafitiga sili ona aoga mo le SCID ^[3] .

Ia, Ole a Lenei Suʻega Ivi?

O le gaʻo o le ponaivi o se togafitiga e suia le suauʻu le maloloina i se maloloina. O le ponaivi ponaivi o le ponaivi, spongy pepa i totonu o ou ponaivi e faia sela-faia sela (toto totoga sela) lea e avea ma sela toto e tau ai faʻamaʻi, ave le okesene i le tino ma faʻatonu le toto ^[4] .

O le taotoga e faia e totoina maloloina ponaivi ponaivi ponaivi i se tagata ina ua uma ona latou mama maloloina ponaivi ponaivi ua faʻateʻaina, ma e aoga i le faʻaleleia atili o le sisitema sisitema o se tamaititi ^[5] .

Faʻatasi ai ma le anoanoaʻi o penefiti o loʻo i ai le faʻaleleia i le soifua maloloina o se tagata, o loʻo i ai foʻi ni faʻafitauli e pei o le graft-versus-host disease, gagau sela o le sela, faʻaleagaina o totoga, faʻamaʻi, papala, maʻitaga, kanesa fou ma e oʻo lava i le oti ^[6] ^[1] .

Busulfan o se vailaʻau e puipuia ai le kanesa i le kanesa lea e faʻaaogaina i le gaʻo o le gaʻo o le ponaivi. Peitai, ua i ai lipoti o le vailaʻau na i ai se leaga aafiaga i luga o tagata na faia i le taotoga, ma pulmonary fibrosis o le sili ona ogaoga tasi.

O lenei ua uma ona tatou ufiufiina le fanua, seʻi o tatou vaʻavaʻai atu i le autu autu, 'Busulfan-faʻatosina mama afaina' ma mo lena, tatou manaʻomia le nofouta o le a Busulfan ma pe faʻafefea ona aoga ^[7] .

O le a le Busulfan?

O se vailaʻau talavai e faʻaogaina e togafitia ai kanesa ma faʻaleagaina le gaʻo o le ponaivi leʻo manaʻomia muamua le gaʻo o le ponaivi. O le vailaʻau o le chemotherapy o se vaega o vailaʻau e taʻua o alkylating agents ma e galue e ala i le faifai lemu pe taofi le tuputupu aʻe o sela kanesa i lou tino. ^[8] . O loʻo faʻaaogaina foi mo le togafitia o faʻamaʻi toto pei o polycythemia vera ma myeloid metaplasia ma ia togafitia ai myelogenous leukemia masani (CML) ^[9] .

Peitai, o le vailaʻau o le chemotherapy, pei ona taʻua muamua, e i ai se aafiaga ogaoga ogaoga mafua ai pulmonary fibrosis lea e taua o Busulfan-faʻaosofia mama afaina ^[9] ^[10] .

O le a le mea leaga o Busulfan Lung?

Pulu Busulfan, e taua o le pulmonary fibrosis e mafua mai ile vailaʻau e afaina ai ma maʻila le tino o le tino. E ui e seasea maua, o le tulaga e leaga ma e tupu i lalo ifo o le 5 pasene o tagata mamaʻi na togafitia ma Busulfan ^{[sefulu ma le tasi]} . O le lipoti muamua o Busulfan mama na faaleagaina na lipotia mai i le 1961.

O le tulaga e mafua ai ona faia ni uaua malo ma malo i totonu o mama, lea e faigata ai ona manava le tagata.

Ae, e tatau ona maitauina o le vailaʻau e i ai isi itu oona matuia, pei o nei ^[12] :

Fuafua
Faʻaleagaina
Cataract
Avea faʻapitoa
Amenorrhoea
Malformation o fetus
Sterility
Falaʻau fibrosis
Teuina o uric acid i fatuga tubules
Anemia
Alopecia
Gynaecomastia

O nisi o faʻamalieina o aʻafiaga o vailaʻau o loʻo taʻua i lalo ^{[sefulu ma le tasi]} :

Paʻu pauna
Faanoi
Hyperpigmentation o le paʻu
Talosaga
Manava tata
Rashes
Le lavava
Gutu mago

Faʻafefea ona faʻamaonia le faʻaleagaina o Busulfan Lung?

Mafaufau o le tulaga e mafua mai i se vailaʻau, e faigata ona faʻailoaina le tulaga. Ma i le tele o tulaga, o le tulaga o le ai ai i lona tulaga alualu i luma tulaga.

O le tomography fuafuaina maualuga (CT Scan) ma le histopathology o ni auala lata mai ia e mafai ona fesoasoani e faʻailoaina le tulaga ^[13] . I nisi tulaga o Busulfan mama faʻaleagaina, o faʻamaoniga ua faʻaalia mea nei:

Faʻaitiitia le carbon monoxide (CO) faʻamalolo avanoa
Laʻititi manava manava luga pulmonary galuega faʻataʻitaʻiga
O le pusa X-ray atonu e faʻaali mai ni avanoa ma fasi i itu uma o le mama
Le iai o rales
O le lanumoana lanu o laugutu, paʻu, poʻo atigilima ona o le maualalo ole toto i le maualuga ole okesene
Faʻateleina le faʻateleina o le faʻavae o atigilima

I Se Faaiuga Mulimuli ...

Afai o le tulaga ua maua i le amataga o laʻasaga, e sili atu faigofie le puleaina o le fualaʻau-faʻaosofia pulmonary fibrosis - pei o le taimi nei vailaʻau togafitiga mafai ona taofi ma se fou vailaʻau mafai ona amataina. ^[14] ^{[sefulu ma le lima]} .

Peitai, sili atu suʻesuʻega e tatau ona faia i luga o le malamalamaʻaga i le ala o le oti faʻafitauli-mafuaʻaga tulaga ma ia atiaʻe se lelei togafitiga faʻafomaʻi mo le tutusa.

Vaʻai Tusitusiga Faʻasino

[1]De Ravin, S. S., Wu, X., Moir, S., Kardava, L., Anaya-O’Brien, S., Kwatemaa, N., ... & Marquesen, M. (2016). Lentiviral hematopoietic stem cell gene togafitiga mo le X-fesoʻotaʻi tuʻufaʻatasia tuʻufaʻatasia puipuia. Faʻasaienisi faʻaliliuga faʻaliliuga, 8 (335), 335ra57-335ra57.
[lua]Palacios, T. V., Vergales, B., Wisniewski, J., Borish, L., & Lawrence, M. G. (2016). O le Iunivesite o Virginia poto masani i le faʻatinoina o le pepe fou e suʻesuʻeina mo le tuʻufaʻatasia o tuʻufaʻatasia o puipuiga (SCID). Tusi o tala o le Allergy ma Clinical Immunology, 137 (2), AB216.
[3]Sundin, M., Marits, P., Ramme, K., Kolios, A. G., & Nilsson, J. (2019). Ogaoga tuʻufaʻatasia o le puipuiga (SCID) o loʻo faʻaalia i le taimi o tamaititi, ma le agammaglobulinemia, e fesoʻotaʻi ma suiga o le heterozygous compound i DCLRE1C. Togafitiga Puipuiga, 200, 16-18.
[4]Klein, O. R., Buddenbaum, J., Tucker, N., Chen, A. R., Gamper, C. J., Loeb, D., ... & Holuba, M. J. (2017). Nonmyeloablative haploidentical ponaivi tuiina ma post-transplantation cyclophosphamide mo tamaiti ma talavou matutua tagata mamaʻi ma maualuga-lamatiaga hematologic malignancies. Paiolo o le Totoina ma le Tauiina o le Sini, 23 (2), 325-332.
[5]Robinson, T. M., O'Donnell, P. V., Fuchs, E. J., & Luznik, L. (2016, Aperila). Haploidentical ponaivi gaʻo ma totoga sela totoina: poto masani ma post-transplantation cyclophosphamide. I Seminars i le hematology (Vol. 53, Nu. 2, i. 90-97). WB Saunders.
[6]Morishima, Y., Kashiwase, K., Matsuo, K., Azuma, F., Morishima, S., Onizuka, M., ... & Mori, T. (2015). Taua o meaola o le HLA locus tutusa i le fesoʻotaʻi fesoʻotaʻi ponaivi ponaivi totoina. Toto, 125 (7), 1189-1197.
[7]Ikeda, J., Scipione, C., Hyduk, S., Althagafi, M. G., Gao, X., Jongstra-Bilen, J., & Cybulsky, M. I. (2019). Ole gaosiga ole ponaivi e afaina ai le tulaga muamua ole Atherosclerotic Lesion Formation. Arteriosclerosis, Thrombosis, ma Vascular Biology, 39 (Suppl_1), A534-A534.
[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). Vaʻaia togafitiga salivary o le IV busulfan i tagata mamaʻi o loʻo faia hematopoietic stem cell transplantation: o se pailate suʻesuʻe. Tuiina o ponaivi ponaivi, 52 (10), 1384.
[9]Tutschka, P. J., Copelan, E. A., & Klein, J. P. (1987). Fesuiaiga o ponaivi o le ponaivi mo leukemia i le maeʻa ai o se tulafono fou o le busulfan ma le cyclophosphamide. Toto, 70 (5), 1382-1388.
[10]Jain, R., Gupta, K., Bhatia, A., Bansal, A., & Bansal, D. (2017). Hepatic sinusoidal-obstruction syndrome ma busulfan-faʻatupuina manua mama i se post-autologous gagau sela sela mauaina. Pediatrics Initia, 54 (9), 765-770.
[sefulu ma le tasi]Matijasic, N., Bonevski, A., Tokic Pivac, V., & Pavic, I. (2019). Busulfan-Induced Lung Injury in Pediatric Oncology Patients-Iloiloina o Tusitusiga ma se Ata Faʻatusa. Pediatric Allergy, Immunology, ma Pulmonology, 32 (3), 86-91.
[12]Mourad, M., & Crosser, M. S. (2016). A48 MAFUFUAGA O MAU FUA FAʻATONU O MAU LELEI: Faʻasolosolo mama i le maeʻaina o le sela o le peripheral Stem cell, ae leʻo taimi uma na afaina ai le niumonia. O se mataupu o le Busulfan-Induced Pulmonary Manua. American Journal of Respiratory and Critical Care Medicine, 193, 1.
[13]Rix, A., Drude, N. I, Mrugalla, A., Baskaya, F., Pak, K. Y., Gray, B., ... & Mottaghy, F. M. (2019). Suʻesuʻega o le Chemotherapy-Induced Organ Damage ma le Ga-68 Faʻailogaina Duramycin. Molecular Imaging and Biology, 1-11.
[14]Themanns, M., Koban, F., Bergmayr, C., Chrzan, A., Strohmaier, W., Haybaeck, J., ... & Zebedin-Brandl, E. (2019). Treprostinil faʻaitiitia endothelial faʻaleagaina i murine sinusoidal obstruction syndrome. Tusi o Faʻafomaʻi Molecular, 97 (2), 201-213.
[sefulu ma le lima]Averyanov, A., Kogan, E., & Lesnyak, V. (2020). Faʻamaʻi pipisi na mafua mai i fualaʻau. I Faigata e Suʻesuʻe Faʻamaʻi Eseese Faʻamaʻi o le mama (i. 393-408). Faʻasalalauga Nusipepa.