Ubuwazi ngalesi sifo esithinta amaphaphu akho nezinso ngesikhathi esisodwa? Ake sixoxe nge-Goodpasture Syndrome!

Ingabe ngezinye izikhathi ukhwehlela njalo? Ingabe lokho kukhwehlela ngezinye izikhathi kuphakamisa igazi elincane? Noma uzizwa uphelelwa umoya, ukhathele, futhi ukhipha umchamo ophansi? Lokhu kungaba izimpawu zento ethile engathi sína. Namuhla sizokhuluma ngesimo esisodwa esingavamile, kodwa okufanele usiqaphele.

Iyini i-Goodpasture Syndrome?

Kalula nje, i-Goodpasture Syndrome iyisifo esingavamile kodwa esingasongela impilo sokuzivikela komzimba . "I-Autoimmune" isho ukuthi uhlelo lokuzivikela lomzimba wethu, uhlelo lokuzivikela komzimba, luqala ngephutha ukuhlasela amangqamuzana nezicubu zethu ezinempilo.

Kulesi sifo, amasosha omzimba wethu ahlasela iphrotheni ebizwa ngokuthi i-collagen. I-Collagen ifana nesakhiwo emizimbeni yethu. I-Collagen isiza ukugcina isikhumba sethu, imisipha, imisipha, imisipha kanye namathambo kuqinile.

Ngakho-ke, uma une-Goodpasture syndrome, amanye amaprotheni akhiqizwa umzimba wakho, siwabiza ngokuthi ama-antibodies, ayahamba ayonamathela ku-collagen emaphashini nasezinso. Uma lokhu kwenzeka, ukuvuvukala kuqala kulezo zindawo, futhi lezo zicubu ziqala ukuwohloka kancane kancane. Uma zingalashwa, lokhu kungaholela ekuvuvukeni okukhulu kwezinso (i-glomerulonephritis), okungaholela ekuhlulekeni kwezinso. Ungaqala futhi ukopha kakhulu emaphashini (ukuphuma kwegazi emaphashini). Ezimweni eziningi, imbangela enkulu yokufa ngenxa yalesi sifo ukopha emaphashini.

Elinye igama lalokhu lithi:

• Isifo se-anti-glomerular basement membrane (anti-GBM)
• Isifo samadlelo amahle

Kuvamile kangakanani lokhu?

Lesi yisifo esingavamile kakhulu . Ngokusho kodokotela, kubikwe icala elilodwa noma amabili kuphela lalesi sifo kubantu abayi-100,000 ngonyaka. Ngakho ungacabanga ukuthi singavamile kangakanani.

Ziyini izimpawu?

I-Goodpasture syndrome iyisimo `(sezinso-zezinso)`. Lokhu kusho ukuthi amaphaphu nezinso zombili ziyathinteka ngesikhathi esisodwa. Ngokuvamile, izimpawu zokuqala ezibonakala zihlobene namaphaphu. Lezi yizi:

• Ubunzima bokuphefumula (ukuphelelwa umoya): Hhayi nje kuphela lapho ukhuphuka izitebhisi, kodwa ngisho nalapho uphumule.
• Ubuhlungu besifuba : Ubuhlungu besifuba uma uphefumula noma ukhwehlela.
• Ukukhwehlela : Ukukhwehlela okuqhubekayo, ngezinye izikhathi okubuhlungu.
• Umsindo uvela esifubeni lapho uphefumula : Kungaba umsindo wokubhonga, njengokungathi kukhona okunamathele esifubeni.
• Ngizizwa ngikhathele kakhulu.`(ukukhathala)`: Umuzwa wokuba buthakathaka kangangokuthi awukwazi ukwenza lutho.
• Ukopha ekhaleni (i-epistaxis).
• Ukukhwehlela igazi : Lokhu kuyisibonakaliso esethusa abantu abaningi futhi esidinga usizo lwezokwelapha ngokushesha.
• Umbala okhanyayo : Isikhumba siyashintsha sibe mhlophe ngenxa yokuntuleka kwegazi.

Izimpawu ezihlobene nezinso nazo zingase zivele kamuva. Zihlanganisa:

• Ukwehla kwamangqamuzana abomvu egazini, okungukuthi, i-anemia .
• Igazi emchameni (i-hematuria): Umchamo ungase ube bomvu noma ube nsundu ngokumnyama.
• Ukuphuma komchamo okuncane kunokujwayelekile.
• Umfutho wegazi ophakeme .
• Isicanucanu nokuhlanza : Lokhu kungenzeka futhi ngokulahlekelwa yisifiso sokudla.

Yini ebangela lokhu?

I-Goodpasture syndrome yenzeka lapho ama-antibodies akhiqizwa amasosha ethu omzimba ehlasela i-collagen ku-glomerular basement membrane (GBM) ezinso. Le GBM iyingxenye ye-glomeruli, amayunithi amancane okuhlunga igazi ezinso zethu. La ma-antibodies ahlasela ne-collagen emasakeni omoya emaphashini. Lokhu kubangela ukuthi izicubu zamaphaphu zibhujiswe, okubangela ukopha futhi kwenze kube nzima ukuphefumula.

Akukaziwa kahle ukuthi kungani isimiso somzimba sokuzivikela sihlasela i-collagen yaso. Kungenzeka ukuthi izici zemvelo kanye nezakhi zofuzo zethu zingase zihileleke kulesi simo. Ucwaningo luthole ukuthi abantu abanalesi sifo banobudlelwano obuqinile nophawu lwezakhi zofuzo olubizwa ngokuthi i-Human Leukocyte Antigen (HLA) DR15. Lena iphrotheni esiza isimiso somzimba sokuzivikela ukuhlukanisa phakathi kwezicubu zethu nezinto zangaphandle.

Ngezinye izikhathi, lesi sifo singavela ngemva kokutheleleka, njengomkhuhlane noma umkhuhlane. Futhi, wena:

• Uma ubhema .
• Uma uphefumula izidakamizwa ezifana ne -cocaine .
• Ingozi yokuthola lesi sifo iphakeme uma ubhekene nothuli lwensimbi noma amakhemikhali e-hydrocarbon njenge-methane ne-propane.

Ubani lokhu okuthinta kakhulu?

I-Goodpasture syndrome ingavela kunoma yimuphi ubudala, kodwa ivame kakhulu kubantu abasebasha, abaneminyaka ephakathi kweyishumi namashumi amathathu, bese kuba ngabantu abaseminyakeni yabo yama-60 nangama-70.

Kwenziwa kanjani ukuxilongwa?

Uma ubona udokotela, bazobuza ngezimpawu zakho futhi bahlole umzimba. Ngaphezu kwalokho, bangenza nezivivinyo ezilandelayo ukuqinisekisa i-Goodpasture syndrome:

• Ukuhlolwa kwegazi : Lokhu kuhlola izinga lakho lokuhlunga le-glomerular (eGFR) elilinganisiwe, elilinganisa ukuthi izinso zakho zisebenza kahle kangakanani.
• Ukuhlolwa komchamoUkuhlolwa komchamo: Lokhu kuhlola igazi emchameni wakho noma amazinga aphezulu amaprotheni (i-proteinuria).
• Ukuhlolwa kwezithombe : Kwenziwa i-X-ray yesifuba noma i-CT scan ukuhlola umonakalo wamaphaphu.
• I-Bronchoscopy : Kule nqubo, udokotela ufaka ithubhu elincane, elide (i-bronchoscope) elinekhamera kanye nokukhanya okunamathiselwe ngekhala lakho noma emlonyeni wakho ukuhlola umonakalo ngaphakathi kwamaphaphu akho.
• Ukuhlolwa kwezinso : Kulokhu, kuthathwa ingxenye encane yezicubu ezinso bese ihlolwa ngaphansi kwe-microscope ukuqinisekisa ukuthi isifo sezinso sikhona yini.

Ingabe i-Goodpasture syndrome ingelapheka?

Yebo, lokhu kungelapheka uma kutholakale ukuthi unesifo esifanele futhi ukwelashwa kuqala ngokushesha.

Iphathwa kanjani?

Ukwelashwa kwe-Goodpasture syndrome kuyahlukahluka kuye ngokuthi isimo sibucayi kangakanani. Uma isimo singesibi kakhulu, odokotela bangase bakunikeze imithi efana nalokhu:

• Ama-Corticosteroids : Isibonelo, imithi efana ne-prednisone. Lokhu kusiza ekumiseni ukopha emaphashini.
• Imithi yokucindezela amasosha omzimba : Imithi efana ne-cyclophosphamide. Le mithi ivimba amasosha omzimba wakho ekuhlaseleni izicubu zawo.
• Imithi yokucindezela kwegazi (imithi yokunciphisa umfutho wegazi): Le mithi isiza ukulawula umfutho wegazi nokunciphisa umonakalo ezinso.

Ngaphezu kwalokho, odokotela basebenzisa nokwelashwa okubizwa ngokuthi i-plasmapheresis . Lokhu kuhilela ukuthatha igazi emthanjeni osengalweni yakho ngenaliti, ukuhlukanisa ingxenye ewuketshezi yegazi, ebizwa ngokuthi i-plasma, kumaseli egazi. Ama-antibody ayingozi e-anti-GBM akule plasma. I-plasma iyasuswa, bese i-plasma evela kumuntu ophilile ifakwa emaseli akho egazi bese ibuyiselwa kuwe. Kalula nje, isusa ama-antibodies ayingozi emzimbeni wakho.

Kuthatha isikhathi esingakanani ukululama ngemva kokwelashwa?

Kuzodingeka uphuze imithi yokucindezela amasosha omzimba cishe izinyanga eziyi-6 kuya kweziyi-12. Ezimweni eziningi, kungase kudingeke wenze i-plasmapheresis nsuku zonke amasonto amaningana.

Kuzokwenzekani ngokulandelayo?

Uma ingelashwa, i-Goodpasture syndrome ingaholela ekuphumeni kwegazi okusongela ukuphila okuvela emaphashini kanye nokwehluleka kwezinso. Kodwa-ke, uma lesi sifo sibonakala kusenesikhathi futhi ukwelashwa kuqalwe, umphumela uvame ukuba muhle. Umzimba wakho ukhiqiza lawo ma-antibodies ayingozi isikhathi esifushane kuphela - kusukela emavikini ambalwa kuya eminyakeni emibili. Ukubuyela emuva noma ukuphinda kwenzeke akuvamile kakhulu.

Inkinga enkulu kakhulu yalokhu ukwehluleka kwezinso. Uma lokho kwenzeka, ukwelashwa nge-dialysis kuyadingeka ukuze kuhlungwe igazi.Kudingeka ukufakelwa izinso.

Ngithini ngempilo yami?

Cishe abantu abangu-80% abane-Goodpasture syndrome banesilinganiso sokusinda seminyaka emihlanu esingu-80%. Lokho kwenzeka uma lesi sifo sitholakala kusenesikhathi futhi selashwa ngendlela efanele.

Ingabe lokhu kungavinjelwa?

Kungase kungenzeki ukukuvimbela ngokuphelele. Kodwa-ke, unganciphisa ingozi yakho ngokugwema izinto ezithile:

• Ama-hydrocarbon, okusho izinto ezifana ne-methane, i-propane, uphethiloli, i-parafini, kanye ne-tar.
• Uthuli lwensimbi.
• I-cocaine.
• Imikhiqizo yokupenda izinwele.

Ngingazinakekela kanjani?

Uma unesifo se-Goodpasture, kuwumqondo omuhle ukwenza lezi zinto:

• Yeka ukubhema ngokuphelele. Gwema ukuba sezindaweni lapho abanye abantu bebhema khona (okungukuthi, gwema ukuchayeka entuthwini yokubhema esetshenziswa abanye abantu).
• Yenza umsebenzi womzimba okungenani imizuzu engama-30 nsuku zonke.
• Zama ukunciphisa ukucindezeleka .

Kufanele ngimbone nini udokotela?

Uma ukhwehlela igazi, unenkinga yokuphefumula, unciphile umchamo, noma unezinye izimpawu ezibalwe ngenhla, bona udokotela ngokushesha. Ukutholwa kusenesikhathi kwandisa kakhulu amathuba akho okululama. Uma kungelashwa, kungabangela ukulimala kwezinso okungapheli ngisho nezinkinga zamaphaphu ezibulalayo.

Yimiphi imibuzo okufanele uyibuze udokotela?

Uma ubona udokotela, ungabuza le mibuzo:

• Ngingazi kanjani ukuthi nginesi sifo se-Goodpasture?
• Isimo sami sibi kangakanani?
• Yikuphi ukwelashwa okuncomayo?
• Yini engingayenza ukuze nginakekele izinso zami?
• Ingabe kufanele ngibone udokotela wezinzwa noma udokotela wamaphaphu?
• Ungangitshela ngeqembu lokusekela elisiza abantu abanalesi sifo?

Yiziphi izici ezintathu eziyinhloko (i-Triad) ze-Goodpasture syndrome?

Kwezokwelapha, "u-triad" ubhekisela ezimpawini ezintathu eziyinhloko ezichaza isimo sezokwelapha. Izimpawu ezintathu eziyinhloko ze-Goodpasture Syndrome yilezi:

• Ukopha okuvela emaphashini.
• Ukwanda okusheshayo kokuvuvukala kwezinso.
• Ukuba khona kwalawo ma-antibodies abizwa ngokuthi ``ama-anti-GBM antibodies'' emzimbeni.

Ukuba nalesi sifo kungaba yinto ekhathazayo futhi enesizungu, ikakhulukazi njengoba imbangela eqondile ingakaziwa. Kungaphazamisa imisebenzi yakho yansuku zonke, futhi kungadingeka ushintshe imisebenzi uma usebenza ngempuphu yensimbi, uphethiloli, noma i-tar.

Okokugcina, okufanele ukukhumbule

Ngakho-ke, i-Goodpasture Syndrome yinto okufanele uyesabe, kodwa uma ubona izimpawu kusenesikhathi, ubonane nodokotela ngokushesha, bese uqala ukwelashwa, ungathola imiphumela emihle kakhulu. Qaphela izinguquko emzimbeni wakho. Uma uzizwa into engavamile, kubalulekile ukufuna iseluleko sezokwelapha ngaphandle kokuyilahla njengento encane. Awuwedwa, futhi odokotela bangakusiza.

I- Goodpasture syndrome, isifo se-Anti-GBM, ukopha kwamaphaphu, isifo sezinso, izifo zokuzivikela komzimba, ubunzima bokuphefumula, igazi emchameni