Ubusazi ngesi sifo esichaphazela imiphunga yakho kunye nezintso ngaxeshanye? Masithethe ngeGoodpasture Syndrome!

Ngaba ngamanye amaxesha ukhwehlela rhoqo? Ngaba loo khohlela ngamanye amaxesha inyusa igazi elincinci? Okanye uziva uphelelwa ngumoya, udiniwe, kwaye umchamo wakho unciphile? Ezi zinokuba ziimpawu zento enzulu. Namhlanje siza kuthetha ngesinye sezifo ezingaqhelekanga, kodwa ekufuneka uziqaphele.

Yintoni iGoodpasture Syndrome?

Ngamafutshane, iGoodpasture Syndrome sisifo esingaqhelekanga kodwa esinokubeka ubomi esichengeni . "I-Autoimmune" ithetha ukuba inkqubo yokuzikhusela yomzimba wethu, inkqubo yokuzikhusela yomzimba, ngempazamo iqala ukuhlasela iiseli kunye nezicubu zethu ezisempilweni.

Kwesi sifo, amajoni ethu omzimba ahlasela iproteni ebizwa ngokuba yi-collagen. I-Collagen ifana nebhloko yokwakha emizimbeni yethu. I-Collagen inceda ukugcina ulusu lwethu, izihlunu, imisipha, ii-ligaments kunye namathambo omelele.

Ngoko ke, ukuba une-Goodpasture syndrome, ezinye zeeproteni ezenziwa ngumzimba wakho, sizibiza ngokuba zii-antibodies, ziya kuthi zinamathele kwi-collagen emiphungeni nasezintsoni zakho. Xa oku kusenzeka, ukudumba kuqala kwezo ndawo, kwaye ezo zicubu ziqala ukuwohloka kancinci. Ukuba azinyangwa, oku kunokukhokelela ekuvuvukeni okukhulu kwezintso (i-glomerulonephritis), okunokukhokelela ekungasebenzini kwezintso. Ungaqala ukopha kakhulu emiphungeni (i-pulmonary hemorrhage). Kwiimeko ezininzi, unobangela oyintloko wokufa kwesi sifo kukopha emiphungeni.

Elinye igama lale nto ngu:

• Isifo se-anti-glomerular basement membrane (anti-GBM)
• Isifo sedlelo elihle

Kuqheleke kangakanani oku?

Esi sisifo esingaqhelekanga kakhulu . Ngokutsho koogqirha, kukho amatyala amatsha amabini kuphela esi sifo axelwayo kubantu abayi-100,000 ngonyaka. Ngoko ke ungacinga ukuba sinqabile kangakanani.

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Zithini iimpawu?

I-Goodpasture syndrome yimeko ye-(pulmonary-renal). Oku kuthetha ukuba imiphunga kunye nezintso zichaphazeleka ngaxeshanye. Rhoqo, iimpawu zokuqala ezibonakala zinxulumene nemiphunga. Nazi:

• Ubunzima bokuphefumla (ukuphelelwa ngamandla): Kungekuphela nje xa unyuka izinyuko, kodwa kwanaxa uphumle.
• Intlungu yesifuba : Intlungu yesifuba xa uphefumla okanye ukhohlela.
• Ukukhwehlela : Ukukhwehlela okungapheliyo, ngamanye amaxesha okubuhlungu.
• Ingxolo ivela esifubeni xa uphefumla : Isenokuba yingxolo egubhayo, ngokungathi kukho into enamathele esifubeni.
• Ndiziva ndidiniwe kakhulu.(ukudinwa): Ukuziva ubuthathaka kangangokuba awukwazi ukwenza nto.
• Ukopha kwempumlo (epistaxis).
• Ukukhohlela igazi : Olu luphawu olubangela uloyiko kubantu abaninzi kwaye lufuna unyango olukhawulezileyo.
• Ulusu lujika lube mhlophe ngenxa yokunqongophala kwegazi.

Iimpawu ezinxulumene nezintso nazo zinokuvela kamva. Ziquka:

• Ukuncipha kweeseli ezibomvu zegazi egazini, oko kukuthi, i-anemia .
• Igazi kumchamo (hematuria): Umchamo unokuba bomvu okanye ube mdaka ngebala.
• Ukuphuma komchamo kuncinci kunesiqhelo.
• Igazi elonyukayo .
• Isicaphucaphu nokuhlanza : Oku kunokwenzeka nangokuphelelwa ngumdla wokutya.

Yintoni ebangela oku?

I-Goodpasture syndrome yenzeka xa ii-antibodies eziveliswa yinkqubo yethu yomzimba zihlasela i-collagen kwi-glomerular basement membrane (GBM) kwizintso. Le GBM yinxalenye ye-glomeruli, iiyunithi ezincinci zokucoca igazi kwizintso zethu. Ezi antibodies zihlasela ne-collagen kwi-air sacs emiphungeni. Oku kubangela ukuba izicubu zemiphunga zitshatyalaliswe, nto leyo ebangela ukopha kwaye kwenze kube nzima ukuphefumla.

Akukaziwa ukuba kutheni inkqubo yomzimba yokuzikhusela ihlasela i-collagen yayo. Kusenokwenzeka ukuba izinto ezingqongileyo kunye ne-genes zethu zinokuba negalelo kule meko. Izifundo zifumanise ukuba abantu abanesifo banolwalamano oluqinileyo nophawu lwe-genetic olubizwa ngokuba yi-Human Leukocyte Antigen (HLA) DR15. Le yiproteni enceda inkqubo yomzimba yokuzikhusela ukuba yahlule phakathi kwezicubu zethu kunye nezinto zangaphandle.

Ngamanye amaxesha, esi sifo sinokuvela emva kosulelo, njengomkhuhlane okanye umkhuhlane. Kwakhona, wena:

• Ukuba uyatshaya .
• Ukuba uphefumla iziyobisi ezifana ne-cocaine .
• Umngcipheko wokufumana esi sifo uphezulu ukuba ujongene nothuli lwesinyithi okanye iikhemikhali ze-hydrocarbon ezifana ne-methane kunye ne-propane.

Ngubani ochaphazela kakhulu oku?

I-Goodpasture syndrome inokwenzeka nakubani na ubudala, kodwa ixhaphake kakhulu kubantu abancinci, abaphakathi kweminyaka elishumi ukuya kumashumi amathathu, kwaye kwakhona kubantu abakwiminyaka yabo yama-60 ukuya kuma-70.

Yenziwa njani le nto yokuxilonga?

Xa ubona ugqirha, baya kukubuza ngeempawu zakho baze bakuhlole ngokwasemzimbeni. Ukongeza, banokwenza olu vavanyo lulandelayo ukuqinisekisa i-Goodpasture syndrome:

• Uvavanyo lwegazi : Olu lujonga izinga lakho lokucoca i-glomerular (eGFR) eliqikelelweyo, elilinganisa ukuba izintso zakho zisebenza kakuhle kangakanani.
• Uvavanyo lomchamoUhlalutyo lomchamo: Oku kujonga igazi kumchamo wakho okanye amanqanaba aphezulu eeproteni (proteinuria).
• Uvavanyo lwemifanekiso : Kwenziwa i-X-ray yesifuba okanye i-CT scan ukujonga umonakalo emiphungeni.
• I-Bronchoscopy : Kule nkqubo, ugqirha ufaka ityhubhu ende ebhityileyo (i-bronchoscope) enekhamera kunye nokukhanya okufakwe ngempumlo okanye emlonyeni wakho ukujonga umonakalo ngaphakathi kwimiphunga yakho.
• I-biopsy yezintso : Kule nto, kuthathwa iqhekeza elincinci lesicubu esisezintsoni lize lihlolwe phantsi kwe-microscope ukuqinisekisa ukuba isifo sezintso sikhona na.

Ngaba isifo seGoodpasture sinokunyangeka?

Ewe, oku kunokunyangeka ukuba kufunyaniswe isifo ngokuchanekileyo kwaye unyango luqala ngokukhawuleza.

Iphathwa njani?

Unyango lwe-Goodpasture syndrome luyahluka ngokuxhomekeke kubukhali bemeko. Ukuba imeko ayinzima kakhulu, oogqirha banokunika amayeza anjengala:

• Ii-Corticosteroids : Umzekelo, amayeza afana ne-prednisone. La mayeza anceda ekumiseni ukopha emiphungeni.
• Amayeza okunciphisa umzimba : Amayeza afana ne-cyclophosphamide. La athintela inkqubo yakho yomzimba yokuzikhusela ekuhlaseleni izicubu zayo.
• Amayeza oxinzelelo lwegazi (amayeza okunciphisa uxinzelelo lwegazi): La mayeza anceda ukulawula uxinzelelo lwegazi nokunciphisa umonakalo kwizintso.

Ukongeza, oogqirha basebenzisa unyango olubizwa ngokuba yi-plasmapheresis . Oku kuquka ukuthatha igazi elivela kumthambo osengalweni yakho ngenaliti, ukwahlula inxalenye yolwelo lwegazi, ebizwa ngokuba yi-plasma, kwiiseli zegazi. Ii-anti-GBM antibodies eziyingozi zikwi-plasma. Emva koko i-plasma iyasuswa, kwaye i-plasma evela kumntu ophilileyo yongezwa kwiiseli zakho zegazi kwaye ibuyiselwe kuwe. Ngamafutshane, isusa ii-antibodies eziyingozi emzimbeni wakho.

Kuthatha ixesha elingakanani ukuchacha emva konyango?

Kuya kufuneka usele amayeza okunciphisa amajoni omzimba kangangeenyanga ezi-6 ukuya kwezi-12. Kwiimeko ezininzi, kunokufuneka wenze i-plasmapheresis yonke imihla kangangeeveki eziliqela.

Kuza kwenzeka ntoni emva koko?

Ukuba ayinyangwa, i-Goodpasture syndrome inokukhokelela ekuphumeni kwegazi okusongela ubomi kwimiphunga kunye nokungasebenzi kakuhle kwezintso. Nangona kunjalo, ukuba isifo siqatshelwe kwangethuba kwaye unyango luqalisiwe, isiphumo sihlala silungile. Umzimba wakho uvelisa ezo antibodies ziyingozi ixesha elifutshane - ukusuka kwiiveki ezimbalwa ukuya kwiminyaka emibini. Ukubuyela umva okanye ukuphinda ubuye kunqabile kakhulu.

Eyona ngxaki inkulu yoku kukungasebenzi kakuhle kwezintso. Ukuba oko kuyenzeka, unyango lwe-dialysis luyafuneka ukuze kuhluzwe igazi.Kuyimfuneko ukufakelwa izintso.

Ndithini ngobomi bam?

Malunga nama-80% abantu abane-Goodpasture syndrome banomlinganiselo wokusinda weminyaka emihlanu wama-80%. Oko kwenzeka ukuba esi sifo sichongiwe kwangethuba kwaye sinyangwa ngokufanelekileyo.

Ngaba oku kungathintelwa?

Kusenokungabi lula ukuyithintela ngokupheleleyo. Nangona kunjalo, ungayinciphisa ingozi yakho ngokuphepha izinto ezithile:

• Iihydrocarbon, oko kuthetha izinto ezifana ne-methane, i-propane, ipetroli, i-parafini, kunye ne-tar.
• Uthuli lwesinyithi.
• I-cocaine.
• Iimveliso zokufaka imibala kwizinwele.

Ndingazinyamekela njani?

Ukuba une-Goodpasture syndrome, kungcono ukwenza ezi zinto zilandelayo:

• Yeka ukutshaya ngokupheleleyo. Kuphephe ukuba kwiindawo apho abanye abantu batshaya khona (oko kukuthi, kuphephe ukudibana 'nomsi osetyenziswa ngomnye umntu').
• Yenza umthambo ubuncinane imizuzu engama-30 yonke imihla.
• Zama ukunciphisa uxinzelelo .

Ndifanele ndimbone nini ugqirha?

Ukuba ukhohlela igazi, unengxaki yokuphefumla, unciphile umchamo, okanye unezinye iimpawu ezidweliswe apha ngasentla, bona ugqirha ngokukhawuleza. Ukufunyaniswa kwangoko kwandisa kakhulu amathuba akho okuchacha. Ukuba akunyangwa, kunokubangela umonakalo osisigxina wezintso kunye neengxaki ezibulalayo zemiphunga.

Yeyiphi imibuzo omele uyibuze ugqirha?

Xa ubona ugqirha, ungabuza le mibuzo:

• Ndingaqiniseka njani ukuba ndineGoodpasture Syndrome?
• Ingxaki yam inzulu kangakanani?
• Loluphi unyango olucebisayo?
• Ndingenza ntoni ukuze ndinyamekele izintso zam?
• Ngaba kufuneka ndidibane nogqirha wezifo zengqondo okanye ingcali yezifo zemiphunga?
• Ungandixelela ngeqela lenkxaso elinceda abantu abanesi sifo?

Ziziphi iimpawu ezintathu eziphambili (iTriad) zeGoodpasture syndrome?

Kwizonyango, "unxantathu" ubhekisa kwiimpawu ezintathu eziphambili ezichaza imeko yempilo. Iimpawu ezintathu eziphambili zeGoodpasture Syndrome zezi:

• Ukopha okuvela emiphungeni.
• Ukwanda ngokukhawuleza kokudumba kwezintso.
• Ubukho bezo antibodies ezibizwa ngokuba yi-"anti-GBM antibodies" emzimbeni.

Ukuba nesi sifo kunokuba yinto ebangela uxinzelelo olukhulu kwaye ibe sisizungu, ingakumbi kuba isizathu esichanekileyo asikaziwa. Sinokuphazamisana nemisebenzi yakho yemihla ngemihla, kwaye kusenokufuneka utshintshe imisebenzi ukuba usebenza ngomgubo wesinyithi, ipetroli, okanye itha.

Okokugqibela, yintoni ekufuneka uyikhumbule

Ngoko ke, i-Goodpasture Syndrome yinto omele uyoyike, kodwa ukuba uzibona iimpawu kwangethuba, ubone ugqirha ngokukhawuleza, kwaye uqale unyango, ungafumana iziphumo ezilungileyo kakhulu. Qaphela utshintsho emzimbeni wakho. Ukuba uziva into engaqhelekanga, kubalulekile ukufuna ingcebiso yezonyango ngaphandle kokuyithatha njengento encinci. Awuwedwa, kwaye oogqirha banokukunceda.

Isifo seGoodpasture , isifo se-Anti-GBM, ukopha emaphaphu, isifo sezintso, izifo zokuzikhusela komzimba, ubunzima bokuphefumla, igazi emchameni