Idiopathic pulmonary fibrosis ( IPF ) ndi mtundu wa matenda akuluakulu a mapapu omwe amachititsa kuti dyspnea (kupuma pang'ono) kuwonjezere pang'onopang'ono. Anthu omwe ali ndi IPF angakhalenso ndi chifuwa chouma komanso chokhazikika, kutopa kwapang'onopang'ono, kapena kutaya thupi kosawerengeka-komanso nthawi zambiri, kufa msanga.
IPF ndi matenda osadziwika, koma sichikuwoneka kuti ndi osowa. Anthu pafupifupi 15,000 amafa kuchokera ku IPF chaka chilichonse ku United States.
Zimakhudza amuna nthawi zambiri kusiyana ndi amayi, kusuta fodya kuposa omwe osuta fodya, ndipo kawirikawiri anthu oposa zaka 50.
Chifukwa cha IPF sichinagwiritsidwe ntchito mwangwiro ("idiopathic" amatanthawuza "chosadziwika chifukwa"), ndipo palibe chithandizo chake. Komabe, palifukufuku ochulukirapo kuti amvetsetse vutoli, komanso kuti apange chithandizo chabwino kwa IPF . Chizindikiro cha anthu omwe ali ndi IPF chakhala chikupita patsogolo kwambiri m'zaka zingapo zapitazi.
Njira zatsopano zothandizira IPF zikupangidwa, ndipo ena ali kale m'mayesero a kachipatala. Kuyambira mofulumira kwambiri kunena motsimikizirika kuti kupambana kwa mankhwala kumakhala kozungulira pangodya, koma pali zifukwa zambiri zowonjezera chiyembekezo kuposa momwe zinalili kanthawi kochepa chabe.
Kuzindikira kwathu kwa IPF
IPF imayambitsidwa ndi fibrosis yodabwitsa (scarring) ya minofu ya mapapo. Mu IPF, maselo osakhwima a alveoli (mapepala a mpweya) amayamba m'malo mwa maselo akuluakulu, omwe amatha kusinthanitsa mpweya.
Chifukwa chake, ntchito yaikulu yamapapu-kusinthanitsa mpweya, kutulutsa oksijeni kuchokera mlengalenga kulowa m'magazi, ndi carbon dioxide kuchoka mwazi-zimasokonezeka. Kupita patsogolo kochepa kuti mupeze mpweya wabwino m'magazi ndimene zimayambitsa zizindikiro zambiri za IPF.
Kwa zaka zambiri, chiphunzitso chogwiritsira ntchito chifukwa cha IPF chinali chimodzi chochokera ku kutupa.
Izi zikutanthauza kuti china chake chinayambitsa kutupa kwa mapapo a mapapo, zomwe zimawombera kwambiri. Njira zamakono zochiritsira kwa IPF zinali makamaka pofuna kupewa kapena kuchepetsa njira yotupa. Mankhwala oterewa aphatikizapo steroids , methotrexate , ndi cyclosporine . Kawirikawiri, mankhwalawa anali othandiza kwambiri (ngati alipo), ndipo anali ndi zotsatira zoopsa.
Pofotokoza chifukwa cha IPF, ofufuza masiku ano asintha maganizo awo pa njira yowonongeka yotengera, ndikuyang'ana pa zomwe tsopano zikukhulupiriridwa kuti ndizochiritso chakumphasa kwa anthu omwe ali ndi vutoli. Izi ndizo, vuto lalikulu lomwe limayambitsa IPF sangakhale lopweteka kwambiri mthupi, koma machiritso osadziwika kuchokera ku (mwina mwina ndibwino). Ndi machiritso osazolowereka, fibrosis yochulukira imapezeka, zomwe zimayambitsa kuwonongeka kwa mapapo a mapapo.
Kuchira kwabwino kwa mapapo a mapapo kumakhala njira yovuta kwambiri, kuphatikizapo kugwirizana kwa mitundu yosiyanasiyana ya maselo ndi zinthu zambiri za kukula, cytokines ndi ma molekyulu ena. Kuchuluka kwa fibrosis mu IPF tsopano kumaganizidwa kuti kumagwirizana ndi kusiyana pakati pa zinthu zosiyanasiyana pa nthawi ya machiritso.
Ndipotu, palizinthu zina zomwe zimagwiritsidwa ntchito poyambitsa matenda a pulmonary fibrosis.
Mamolekyu awa tsopano ndi omwe akuwunikira kufufuza kwakukulu, ndipo mankhwala ambiri akukambidwa ndi kuyesedwa poyesera kubwezeretsa njira yowonongeka yowonjezereka kwa anthu omwe ali ndi IPF. Pakalipano, kufufuza uku kwachititsa kuti pakhale zochepa zolephera ndi zolephera zingapo-koma zopambana zakhala zolimbikitsa kwambiri, ndipo ngakhale zolephereka zapita patsogolo podziwa kwathu za IPF.
Zimapindula Kwambiri
Mu 2014, a FDA adalandira mankhwala awiri atsopano kuti azitsatira IPF , nintendanib (Ofev) ndi pirfenidone (Esbriet).
Mankhwalawa amagwira ntchito poletsa machipatala a tyrosine kinases, mamolekyu omwe amachititsa kukula kwa mitundu yosiyanasiyana ya fibrosis. Mankhwala onsewa asonyezedwa kuti ayambe kuchepetsa kukula kwa IPF.
Mwamwayi, anthu amatha kuyankha bwino kwa mankhwalawa, kapena pakadali pano palibe njira yokonzera mankhwala omwe angakhale abwino kwa munthu aliyense. Komabe, mayesero odalirika angakhale pambali pofotokoza momwe munthu angayankhire kwa mankhwala awiriwa. (Zambiri pa izi pansipa.)
Kuonjezera apo, tsopano zadziwika kuti anthu ambiri omwe ali ndi IPF (mpaka 90 peresenti) ali ndi matenda a reflux a gastroesphageal (GERD) omwe angakhale ochepa kwambiri moti sakudziwa. Komabe, "microreflux" yosakhoza kukhala chinthu chachikulu chomwe chimayambitsa kuwonongeka kwazing'ono m'magazi a mapapo-ndi kwa anthu omwe ali ndi njira yowonongeka ya mapapo, kupweteka kwambiri kwa fibrosis kungabweretse.
Mayesero ang'onoang'ono apanga kuti anthu omwe ali ndi IPF omwe amachiritsidwa ndi GERD akhoza kupeza pang'onopang'ono kupita patsogolo kwa IPF yawo. Ngakhale kuti mayesero akuluakulu ndi aakulu a nthawi yayitali akufunika, akatswiri ena amakhulupirira kuti mankhwala "achizolowezi" a GERD ali kale ndi maganizo abwino kwa anthu omwe ali ndi IPF.
Zomwe Zidzachitike Posachedwapa
Mayeso a Zachibadwa
Zimadziwika kuti anthu ambiri omwe amapanga IPF ali ndi chibadwa choyambirira pa chikhalidwe ichi. Kafukufuku wogwira ntchito akuchitidwa poyerekeza ndi ma genetic m'matenda omwe amapezeka m'mapapo m'magazi amtundu wa anthu omwe ali ndi IPF. Kusiyana kwa mitundu yosiyanasiyana ya majini m'matenda a IPF adadziwika kale. Izi zizindikiro za majini zimapereka ochita kafukufuku zokhudzana ndi chithandizo cha mankhwala pochiza IPF. Zaka zingapo, mankhwala osokoneza bongo "oyenerera" kuti awononge IPF amatha kufika pa siteji yoyesera yachipatala.
Mankhwala Osokoneza Bongo Akuyesedwa
Pamene tikudikirira mankhwala othandizira, pakalipano mankhwala osokoneza bongo akuyesedwa kale:
- Imatinib: Imatinib ndi tyrosine kinase inhibitor, yofanana ndi nintendanib.
- FG-3019: Mankhwalawa ndi anti-monoclonal antibody omwe amagwiritsidwa ntchito pazitsulo zopangira tizilombo toyambitsa matenda, ndipo cholinga chake ndi kuchepetsa fibrosis.
- Thalidomide: Mankhwala awa awonetsedwa kuti achepetse mapapu a fibrose mu zinyama, ndipo akuyesedwa kwa odwala omwe ali ndi IPF.
Pulmospheres
Ochita kafukufuku pa yunivesite ya Alabama adalongosola njira yatsopano yomwe amasonkhanitsira "pulmospheres" -madontho akuluakulu opangidwa kuchokera ku mapaipi a munthu amene ali ndi IPF-ndipo amawonetsa pulmospheres ku anti-IPF mankhwala nintendanib ndi pirfenidone. Kuchokera ku mayeserowa, amakhulupirira kuti akhoza kudziwa nthawi yomwe wodwalayo angayankhe bwino mankhwalawa kapena onsewa. Ngati chidziwitso choyambirira ndi pulmospheres chikutsimikiziridwa ndi kuyesedwa koonjezereka, izi zikhoza kukhala ngati njira yowonetsera kuyesa kuyesa mitundu yosiyanasiyana ya mankhwala kwa anthu omwe ali ndi IPF.
Mawu Ochokera
IPF ndi chiwopsezo chachikulu cha mapapu, ndipo zingakhale zovulaza kuti izi zitheke. Ndipotu, munthu amene ali ndi IPF amene amachititsa Google kufufuza pa matendawa akhoza kuchoka kwambiri. Komabe, m'zaka zingapo zapitazi pakhala kuchuluka kwachulukidwe kogwira IPF. Mankhwala awiri atsopano amavomerezedwa kale kuti athe kuchiritsidwa, othandizira angapo atsopano akuyesedwa mu mayesero a zamankhwala, ndipo kafukufuku wogwirizana akulonjeza kuti apereke njira zatsopano zothandizira posachedwa.
Ngati inu kapena wokondedwa wanu ndi IPF mukufuna kuti muyesedwe kuchipatala chimodzi mwa mankhwala atsopano, zowonongeka pazitsulo zamakono zingapezeke ku clinicaltrials.gov.
> Zotsatira:
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> Raghu G, Rochwerg B, Zhang Y, et al. Malamulo Otsogolera ATS / ERS / JRS / ALAT: Chithandizo cha Idiopathic Pulmonary Fibrosis. Kusintha kwa Malangizo Othandizira Otsogolera a 2011. Am J Respir Crit Care Med 2015; 192: e3.
> Surolia R, Li FJ, Wang Z, ndi al. 3D Pulmospheres Zimatumikira Monga Zomwe Zimakukhudzani ndi Zomwe Zimagwiritsidwa Ntchito Poyesa Mankhwala Osokoneza Bongo. JCI Insight 2017 .; 2 (2): e91377.
> Xu Y, Mizuno T, Sridharan A, et al. Kachilombo kamodzi ka RNA Kuyeza Kuzindikiritsa Ntchito Zambiri za Maselo a Eitheiyi mu Idiopathic Pulmonary Fibrosis. JCI Insight. 2017; 1 (20): e 90558.