Iinkonzo ezintsha ze-Idiopathic Pulmonary Fibrosis

I-FDA I-Esbriet evunyelweyo nje kunye ne-Ofev ye-Idiopathic Pulmonary Fibrosis.

Ngo-Oktobha 2014, i-FDA ivume izidakamizwa ezintsha ezimbini, ipirfenidone (Esbriet) kunye ne-nintedanib (Ofev), kunyango lwe-idiopathic pulmonary fibrosis. Idiopathic pulmonary fibrosis sisifo esibi esibulala abaninzi abantu abaxilongwa ngabo kwiminyaka embalwa emfutshane. Ngaphambi kokufumanisa i-pirfenidone kunye ne-nintedanib, kwakungekho nonyango olululo lwezonyango kubantu abanesi sifo esibulalayo.

Yintoni i-idiopathic Pulmonary Fibrosis?

Idiopathic pulmonary fibrosis yisifo esingapheliyo okanye esihlala ixesha elide esibhubhisa iindawo ezincinci ngaphakathi kwemiphunga (interstitia). Nangona i-data engapheliyo ikhona kwi-propulence ngokuthe ngqo kwesi sifo, i-American Lung Association iqikelela ukuba ixabana nama-140,000 aseMerika. Ngelixa ndisebenzisa le ncwadi, ndabona ukuqikelela okuphantsi kwexesha xa isifo sichazwa ngokuthe ngqo (nceda uxolele i-pun), echaphazela naphi na abantu aba-14 no-28 ngabangama-100,000. Abantu abanesi sifo baneminyaka engama-50 ubudala nangaphezulu, kwaye isifo sichaphazela amadoda angaphezu kwamabhinqa.

Akukho mntu owaziyo obangela idiopathic pulmonary fibrosis (oko kutheni kuthiwa yi- idiopathic disorder). Into esiyaziyo kukuba esi sifo esibulalayo sinyuka siphumelele kwimiphunga-imiphunga yinto eyenza ukuba kube nzima ukuphefumula. Ingqalelo, idiopathic pulmonary fibrosis yintlupheko yesifo somphunga esinciphisa inani lomphunga.

Xa sele sele ifunyanwe, iminyaka yobudala yokuphila kubantu abanesifo sisithuba seminyaka emine kuphela.

Idiopathic pulmonary fibrosis ibangela ukuvuvukala kunye noshintsho lwe-fibrotic (ukutshatyalaliswa) kwimizimba yamaphaphu (parenchyma) edibeneyo kunye nama-capillari e-pulmonary kwi-alveoli. I-capillaries ye-pulmonary e-alveoli yonakaliswa; Ama-capillary e-pulmonary abandakanyeka ekutshintsheni kwe-oksijeni kwaye azisa igazi elityebileyo elithini-oksijini emzimbeni.

Ngamanye amazwi, kubantu abane-puldiary fibrosis, i-scarf in tishu yamaphaphu iyakwazi ukucoca i-oksijini evela emoyeni esiphefumulayo.

Idiopathic pulmonary fibrosis yenza kube nzima ukuphefumula emva kokuzama. Kwakhona kubangela ukukhwehlela kunye noshintsho kwizandi zomphunga kwiimviwo. Uninzi lweempawu zesifo sisistim okanye umzimba wonke, kwakhona, kwaye ziquka ukukhathala, ukulahleka kwesisindo kunye ne-clubbing yeminwe neenzwane. Isisombululo se-CT esiphezulu (cinga i-x-rays x-rays) ibonisa i-patchy okanye pied kunye ne-"honeycomb" ekubonakala kuyo utshintsho lwe-fibrotic. I-biopsy yokuphanda inikezela ngokuchanekileyo kwesi sifo. Njengoko i-pulmonary fibrosis iyaqhubeka, ikhokelela kwimimandla enokudibanisa okanye edibeneyo kuquka ukungaphumeleli kokuphefumula, ukungaphumeleli kwintliziyo kunye negazi lomnxeba .

Nangona singaqinisekanga ukuba yintoni eyenza i-idiopathic pulmonary fibrosis, siyazi ukuba kukho ezinye izinto ezinobungozi zesifo:

Idiopathic pulmonary fibrosis iyaziwayo ngenxa yokungabi naluphi unyango olufanelekileyo nolululo.

Nangona abanye abantu bahlala bezinzile ixesha elide emva kokuxilongwa, baninzi bahlaziye okanye baxakeke ngakumbi nangonyango. U kunyango lwangoku lujoliswe kwiimpawu zesifo okanye uzame ukunciphisa ukuvutha nokunyuka. I-Prednisone, i-steroid, isetyenziselwa ukukhusela ukutshaya. Abantu abaninzi abane-idiopathic pulmonary fibrosis bafumana i-oksijeni eyongezelelweyo kunye nokuvuselelwa kwepmonta. Ekugqibeleni, ukhetho oluphela lonyango kubantu abano-idiopathic pulmonary fibrosis ngumthamo wokupakisha.

Yintoni iPirfenidone (Esbriet) neNintedanib (Ofev)?

Kwizilingo zekliniki, i-pirfenidone (i-Esbriet) ne-nintedanib (i-Ofev) iboniswe ukunciphisa i-fibrosis okanye inqabana, kwaye ngoko ke ukunyuka kweso sifo.

I-Pirfenidone ineendlela ezininzi ezinokuthi zenze isenzo kwaye yonyango lomlomo. Kwimvavanyo yeekliniki zeSigaba 3, malunga nesiqingatha sabathathi-nxaxheba abane-fibrodium idiopathic pulmonary pulmonary reception babona i-pirfenidone yehla ukunciphisa amandla okunyanzeliswa. Lo mlinganiselo ubonisa ukunyanzeliswa kokuphuculwa kwesi sifo. Abathathi-nxaxheba bafumana ezinye iinjongo ezintle kuquka ukuphuculwa kwimizuzu engama-6 yokuhamba kunye nokunciphisa ekufeni (inani labantu abafayo) ngexesha lokufunda isonto eli-52. Imiphumo enokuthi imiphumo yonyango ilawulwe kwaye iyancitshiswa kwi-photosensitive, isisu, isisu kunye nokuhlanza.

I-Nintenanib i-tyrosine-kinase inhibitor kunye ne-pirfenidone yonyango lomlomo. Phakathi kwezinye iinkqubo ze-biologic, i-tyrosine kinase ibandakanyeka ekutshintsheni nasekutshintsheni kwe-fibrotic. Kwimvavanyo yesigaba se-3, njenge-pirfenidone, i-nintenanib nayo yanciphisa intuthuko ye-idiopathic pulmonary fibrosis njengoko ilinganiselwe ukunciphisa izinga lokunciphisa amandla okunyanzeliswa. Imiphumo emibi ingabandakanya ukuhanjiswa, isicathulo, ukuhlanza, intloko, ukunyuka kwengcinezelo yegazi, kunye nokwandisa i-enzyme yesibindi (isalathisi sokungasebenzi kwesibindi). Ekugqibeleni, i-Ofev i-teratogen engafanele ithathwe ngexesha lokukhulelwa ngenxa yokwesaba ukukhubazeka komntwana okanye ukufa.

Yintoni iPirfenidone (Esbriet) neNintedanib (Ofev) Ethethayo kuwe?

Ukuba wena okanye umntu owaziyo une-puldiary pulmonary fibrosis, imvume ye-pirfenidone (Esbriet) kunye ne-nintedanib (i-Ofev) Ngaba uphumelelo lwezokwelapha olufanele ukwamkelwa njengeendaba ezilungileyo. Nangona kunjalo, ezinye iimfuno kufuneka zicaciswe phambi kokuba ezi zonyango zifumana indawo yazo kwiyeza lonyango. Okokuqala, akucaci ukuba i-pirfenidone kunye ne-nintendanib kufuneka isetyenziswe ngokwahlukileyo okanye ngexesha elifanayo xa unyanga i-idiopathic pulmonary fibrosis. Ukuba sisetyenzisene ndawonye, ​​asiqinisekanga ukuba yiyiphi imichiza kufuneka isetyenziswe kuqala. Ukuba zisetyenziselwa ngokwahlukileyo, asiqinisekanga ukuba amayeza aya kubonisa umbane okanye isiphumo esongeziweyo. Okwesibini, asiqinisekanga ukuba ngaba abanye abantu bayayiphendula ("abaphenduli") kuye okanye zombini la mayeza. Okwesithathu, asiyazi ukuba iziyobisi ziya kuqhubeka nokunciphisa ukuqhubela phambili kwe-idiopathic pulmonary fibrosis edlulileyo ixesha elivavanyayo.

Kwinqaku yokugqibela, ukuba wena okanye othandekayo unemithi ye-idiopathic pulmonary fibrosis, cinga ukufunda ngokubanzi malunga namaqela enkxaso yesi sifo kwi-American Lung Association okanye iNational Heart, Lung, ne-Blood Institute.

Imithombo ekhethiweyo

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I-Kemp WL, i-Burns DK, i-Brown TG. Isahluko 13. I-Pulmonary Pathology. Ku: Kemp WL, Burns DK, Brown TG. eds. I-Pathology: I-Big Picture . New York, NY: McGraw-Hill; 2008 Kufikeleleke ngoNovemba 04, 2014.

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"Ukufudumala komphefumlo: inyathelo elikhulu elingaphambili kwi-idiopathic pulmonary fibrosis" nguCJ Ryerson kunye no-HR Collard ukusuka kwi-BMJ epapashwe ngoSeptemba 2014. Ukufikelela kwi-PubMed ngo-11/4/2014.