Kuzvarwa patsva kuongorora kweCystic Fibrosis

Pasina kuonekwa kuchangobva kuzvarwa, cystic fibrosis (CF) haisati yaongororwa kusvikira zviratidzo zvichatanga kuonekwa uye zvakanyatsokura zvakakonzera kukurumidza kukura uye zvinetso zvokufema . Kazhinji, izvi zvinowanikwa mumakore maviri ekutanga ehupenyu - asi nenguva iyo kuongororwa kwaitwa, kukanganisa kwave kwaitwa.

Nekuzvarwa patsva, CF inowanikwa mumavhiki mashomanana okutanga ehupenyu uye vacheche vanogona kutanga kurapwa pakarepo, nekudaro kudzivisa kusagadzikana uye kuporesa kunetseka kwaizove kusingagoni kudzivirirwa.

Kunyanya kuongororwa uye kurapwa kunogonawo kuwedzera kukwanisa kurarama kwevanhu vane CF. Vanhu vari kurarama ne cystic fibrosis vanogona kutarisira kuti vararame pakati pemakore matatu, asi zvimwe zvidzidzo zvinofanotaura kuti vana vanozvarwa nhasi neCF vachagara mukati mavo makumi mashanu nekuda kwekurapwa kwekutanga uye kurapwa kwakanaka.

Kuchenesa Process

Kuzvarwa patsva kuongorora kwe cystic fibrosis muedzo kuti uone kuti kune kestic fibrosis. Ndiro danho rokutanga mune imwe nzira iyo pakupedzisira inotungamirira kuongorororwa kwe cystic fibrosis, kujekeswa kwevatakuri veCF, kana kubvisa zvose zvingaita. Kana zviitiko zvekuongorora kwekutanga zvakanaka, izvo hazvirevi kuti mwana ane cystic fibrosis. Kuongorororwa kwakanaka zvinoreva kuti kuedza kwakawedzerwa kunofanira kuitwa kuti uone kukosha kweshoko rakanaka.

Chokutanga Chekutanga - Kurera Murape Mutsvuku

Danho rekutanga rekuzvarwa patsva kuongororwa nderopa rekuongorora ropa rinoitwa mazuva mashoma mushure mekuberekwa.

Ropa rinotorwa kubva kumucheche uye rinotumirwa kubazi rehurumende kuti riongorore zvinetso zvakasiyana-siyana. Kuongorora kuongorora kweCF kunotarisa mazinga akakwirira echinhu chinonzi immunoreactive trypsinogen (IRT), iyo inyme inoumbwa ne pancreas. Vacheche vanoberekwa ne cystic fibrosis vanowanzova nepamusoro ye IRT muropa ravo, asi mamwe mamiriro ezvinhu anogonawo kuita kuti enzyme iwedzere.

Kana kuonekwa patsva kweCF kwakanaka, izvo zvinoreva kuti mazinga akareba eRT akawanikwa. Hurumende imwe neimwe inosarudza itsananguro yezvakagadziriswa uye dzisina kujairika dze IRT. Dzimwe nyika dzine huwandu hunokwana hunoita huno hwakanaka uye humwe hunoti hunosarudza huwandu hwehukuru hweHIV hunozivikanwa zuva rega rega.

Chipiri Chepiri - Kuedza Kuzvarwa

Kana kuonekwa kwekuzvarwa patsva kweRTT kwakanaka, vazhinji vanoita imwe yero kuropa kuti vaone kana mwana ane mutsa weCystic Fibrosis Transmembrane Conductance Regulator (CFTR). Kune zviitiko zvinodarika 1 200 zvinozivikanwa muCFTR gene inokonzera cystic fibrosis. Hazvibatsiri kana mari inokwanisika kuedza ivo vose, asi vazhinji vanoongorora kuwanda kwezvinhu zvakasiyana-siyana. Zvakare, humwe hurumende inosarudza kuti kuchinja kupi kunowanikwa mujeri rekuedza.

Kana kuchinjwa kweCFTR gene kuchionekwa, mwana anenge ari CF carrier kana ane cystic fibrosis chirwere. Bhuku rezvematongerwo enyika richazivisa chiremba wevana wekutanga uye mune dzimwe nyika, ivo vachazivisa zvakare dhipatimendi rezvehutano rwehutano kana imwe sangano inobvumirwa uye yakadzidziswa kugoverana nemhuri.

Kana mizinga yeTT yakakwirira uye asi hapana kuchinja kweCFTR gene inowanikwa, mhinduro dzematambudziko ose anotumirwa kuchiremba wevana vekuda vana vanozoona kana kuongorora kunodiwa.

Danho rechitatu - Muedzo Wokutuka

I sweat chloride test , kana sweat test, yakave iyo yegoridhe yemuedzo inoshandiswa kuongororwa ne cystic fibrosis kwemakore mazhinji. Chiyero chinoyera huwandu hwemunyu mumhutu wemunhu, unove wakakwirira kudarika unowanikwa nevanhu vane CF. Chloridhi yakagadzirirwa kupfuura 60 mmol / litita inonzi yakakosha.

Apo chiremba mukuru wekuchengetedza vana anogamuchira migumisiro yekuongorora kunozvarwa patsva yebazi rehurumende, iye achasarudza kana mamwe maedzo achifanira kuitwa. Kana mizinga yeRT yakasimudzirwa asi hapana CFTR mutation yakaonekwa, chiremba mukuru anogona kuraira chirwere che sweat chero zvakadaro kana mwana aine chimwe chezvinhu zvisingawanzogadziriswa zvaisina kuiswa mujecha rekuongorora mazita.

Kana kuongorora kunozvarwa patsva kuchiona kuchinja kweCFTR , chiremba mukuru wekurapa acharonga chirwere chekutuka kuti aone kana mwana ane chirwere cheCF. Kana chirwere che chloride test chiri chakanaka, mwana ane cystic fibrosis uye kazhinji anoendeswa kunzvimbo yakagamuchirwa CF yekutanga kurapwa. Kana kuongorora kwemafuroko kwakaratidza kuchinja kweCFTR gene asi sweat chloride test haina kunaka, mwana ndiye mutakuri weCF asi haana chirwere uye haadi chirwere. Mune zvimwe zviitiko, mazano ekubereka maitiro anowanzoitwa nemhuri kuti atsanangure zvinorehwa kwenguva refu yemigumisiro.

Kunobva:
Comeau, AM, PhD, Accurso, FJ, MD, White, TB, PhD, Campbell, III, PW, MD, Hoffman, G., BS, Parad, RB, MD, MPH, Wilfond, BS, MD, Rosenfeld, M ., MD, MPH, Sontag, MK, PhD, Massie, J., MBBS, FRACP, PhD, Farrell, PM, MD, PhD, uye O'Sullivan, BP, MD. "Mirayiridzo Yekushandiswa kweCystic Fibrosis Ichangobva Kuongorora Kuongorora Zvirongwa: Cystic Fibrosis Foundation Workshop Report". Pediatrics. 2007. 119: 2 e495-e518. 6 July 2008.