Kuchedwa kwachinyamata kwa Cystic Fibrosis

Popanda kuonedwa kakhanda, cystic fibrosis (CF) sichipezeka mpaka zizindikiro zikuyamba kuonekera ndipo zakula kwambiri moti zimayambitsa kukula mofulumira ndi mavuto opuma . Kawirikawiri, izi zimachitika zaka ziwiri zoyambirira za moyo - koma nthawi yomwe matendawa apangidwa, kuwonongeka kwachitika kale.

Komabe, pokhala ndi ana obadwa kumene, CF imapezeka m'masabata angapo oyamba a moyo ndi makanda angayambe kulandira chithandizo mwamsanga, motero kupeŵa matenda osoŵa zakudya ndi kupuma kwabwino komwe sikukanatha kupezeka.

Kuyezetsa magazi ndi matenda am'tsogolo kumatha kuwonjezera chiyembekezo cha moyo wa anthu omwe ali ndi CF. Anthu omwe ali ndi cystic fibrosis amatha kuyembekezera kuti azikhala pakati pa zaka makumi atatu, komabe maphunziro ena amanena kuti ana omwe akubadwa lero ndi CF adzakhala ndi zaka makumi asanu chifukwa cha matenda oyambirira komanso mankhwala abwino.

Kusanthula Njira

Kuonedwa kwa khanda kwa cystic fibrosis ndiyeso kuti aone ngati angathe kukhala ndi cystic fibrosis. Imeneyi ndiyo njira yoyamba yomwe imayambitsa matenda a cystic fibrosis, kudziwika kwa odwala CF, kapena kuthetsa zonsezi. Ngati zotsatira za kuyang'ana koyamba ndi zabwino, sizikutanthauza kuti mwana ali ndi cystic fibrosis. Kuwonetsa bwino kumangotanthauza kuti kuyesedwa kwina kuyenera kuchitidwa kuti mudziwe tanthauzo la zotsatira zabwino.

Choyamba - Kukulitsa Red Flag

Gawo loyamba la kuwonetseredwa kwa mwana watsopano ndi kuyezetsa magazi komwe kumachitika masiku angapo atabadwa.

Magazi amachokera kwa mwanayo ndipo amatumizidwa ku labu la boma kuti awone mawonekedwe osiyanasiyana. Kuyezetsa magazi kwa CF kumafuna kuchuluka kwa chinthu chomwe chimatchedwa immunoreactive trypsinogen (IRT), yomwe ndi puloteni yomwe imapangidwa ndi mphukira. Ana amene amalembedwa ndi cystic fibrosis nthawi zambiri amakhala ndi IRT m'magazi awo, koma zinthu zina zingayambitsenso nyamayi.

Ngati kuyang'ana kwa kanthawi kochepa kwa CF kulibwino, zikutanthauza kuti zigawo zapamwamba za IRT zinadziwika. Dziko lirilonse limapanga zitsogozo zake pazochitika zachilendo ndi zachilendo za IRT. Mayiko ena ali ndi mtengo wapatali umene umakhala wabwino kwambiri ndipo ena amati amasankha kuchuluka kwa miyeso ya IRT yopambana tsiku lililonse.

Gawo Lachiwiri - Mayeso a Chibadwa

Ngati kuyang'ana kobadwa kumene kwa IRT kuli kolimbikitsa, ambiri amachitanso mayeso ena pamwazi kuti aone ngati mwanayo wasinthidwa ndi Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). Pali zamoyo zoposa 1,200 zomwe zimadziwika m'thupi la CFTR zomwe zimachititsa cystic fibrosis. Sizothandiza kapena ndalama zimayesedwa kuti ziyesedwe kwa onse, koma zambiri zimayesa kusintha kwa mitundu ingapo. Kachiwiri, boma lirilonse limasintha zomwe zingasinthe muzowunikira.

Ngati kusintha kwa mtundu wa CFTR kumapezeka, mwanayo amakhala wothandizira CF kapena ali ndi matenda a cystic fibrosis. Bungwe la boma lidzadziwitsa dokotala wamkulu wa mwanayo ndi zina, kuti adziwitse dipatimenti yaumoyo yachipatala kapena bungwe lina lomwe likuvomerezedwa ndi kuphunzitsidwa kuti apereke zotsatila ndi mabanja.

Ngati miyeso ya IRT imakwera koma palibe kusintha kwa CFTR yowunikira, zotsatira za mayesero onsewa zimatumizidwa kwa dokotala wamkulu wa chisamaliro cha mwana yemwe angadziwe ngati kuyesedwa kotheka kuli kofunika.

Chinthu Chachitatu - Mayeso Otukwana

Thuku la chloride , kapena thukuta, ndilo mayeso a golide omwe amagwiritsidwa ntchito kuti apeze chithandizo cha cystic fibrosis kwa zaka zambiri. Chiyesochi chimayesa kuchuluka kwa mchere mu thukuta la munthu, chomwe chili chapamwamba kwambiri kuposa anthu omwe ali ndi CF. Ma chloride okhutira oposa 60 mmol / lita amalingalira ngati zotsatira zabwino.

Pamene dokotala wamkulu akulandira zotsatira za kuyang'anitsitsa kumene kuchokera ku laboratori ya boma, iye adzasankha ngati kuyesedwa kwina kuyenera kuchitidwa. Ngati miyeso ya IRT inali kukwezedwa koma palibe kusintha kwa CFTR kunadziwika, dokotala wamkulu akhoza kulamula kuyesa thukuta ngakhale kuti mwanayo ali ndi kusintha kosaoneka bwino komwe sikudaphatikizidwe mu gulu la kuyesera kwa majini.

Ngati kanema kamene kamadziwoneka kamene kamasintha kusintha kwa CFTR , dokotala wamkulu akuyesa kuyesa thukuta kuti adziwe ngati mwanayo ali ndi matenda a CF. Ngati thukuta la chloride ndilobwino, mwanayo ali ndi cystic fibrosis ndipo nthawi zambiri amatumizidwa ku malo ovomerezeka a CF kuti ayambe kumwa mankhwala. Ngati kuyezetsa kwa majini kunawonetsa kusintha kwa jini la CFTR koma thukuta la chloride ndiloipa, mwanayo ndi chithandizo cha CF koma alibe matenda ndipo safuna mankhwala. Mulimonsemo, uphungu wokhudzana ndi majini kawirikawiri umachitidwa ndi banja kuti afotokoze zotsatira za nthawi yaitali za zotsatira.

Chitsime:
Comeau, AM, PhD, Accurso, FJ, MD, White, TB, PhD, Campbell, III, PW, MD, Hoffman, G., BS, Parad, RB, MD, Roshfeld, M ., MD, MPH, Sontag, MK, PhD, Massie, J, MBBS, FRACP, PhD, Farrell, PM, MD, PhD, ndi O'Sullivan, BP, MD. "Malangizo Othandizira Kugwiritsa Ntchito Ndondomeko Zowonongeka Zachibadwa Zatsopano za Cystic Fibrosis: Cystic Fibrosis Foundation Workshop Report". Matenda. 2007. 119: 2 e495-e518. 6 July 2008.