Koj puas paub txog tus kab mob no uas cuam tshuam rau koj ob lub ntsws thiab lub raum tib lub sijhawm? Cia peb tham txog Goodpasture Syndrome!

Koj puas muaj tej lub sij hawm koj hnoos tsis tu ncua? Tej lub sij hawm koj hnoos ntawd puas muaj ntshav me ntsis? Los yog koj puas xav tias koj ua tsis taus pa, los yog koj tsuas yog nkees thiab tso zis tsawg xwb? Tej zaum cov no yog cov cim qhia tias muaj teeb meem loj heev. Hnub no peb yuav tham txog ib qho mob uas tsis tshua muaj, tab sis yog ib qho uas koj yuav tsum paub txog.

Goodpasture Syndrome yog dab tsi?

Hais yooj yim xwb, Goodpasture Syndrome yog ib yam kab mob autoimmune uas tsis tshua muaj tab sis muaj peev xwm ua rau tuag taus. "Autoimmune" txhais tau hais tias peb lub cev tiv thaiv kab mob, lub cev tiv thaiv kab mob, pib tawm tsam peb cov hlwb thiab cov nqaij noj qab haus huv tsis raug.

Hauv tus kab mob no, peb lub cev tiv thaiv kab mob tawm tsam ib qho protein hu ua collagen. Collagen zoo li lub pob zeb hauv peb lub cev. Collagen pab kom peb daim tawv nqaij, cov leeg, cov leeg nqaij, cov leeg, thiab cov pob txha muaj zog.

Yog li, yog tias koj muaj Goodpasture syndrome, qee cov protein uas koj lub cev tsim, peb hu ua lawv cov tshuaj tiv thaiv kab mob, mus thiab txuas rau collagen hauv koj lub ntsws thiab lub raum. Thaum qhov no tshwm sim, kev o pib hauv cov cheeb tsam ntawd, thiab cov ntaub so ntswg ntawd pib maj mam tawg. Yog tias tsis kho, qhov no tuaj yeem ua rau mob hnyav ntawm lub raum (glomerulonephritis), uas tuaj yeem ua rau lub raum tsis ua haujlwm. Koj kuj tseem tuaj yeem pib los ntshav ntau ntawm lub ntsws (pulmonary hemorrhage). Hauv ntau qhov xwm txheej, qhov ua rau tuag los ntawm tus kab mob no yog los ntshav ntawm lub ntsws.

Lwm lub npe rau qhov no yog:

• Kab mob tiv thaiv glomerular basement membrane (anti-GBM)
• Kab mob Goodpasture

Qhov no feem ntau tshwm sim li cas?

Qhov no yog ib yam kab mob tsis tshua muaj . Raws li cov kws kho mob hais, tsuas muaj ib mus rau ob tug neeg mob tshiab uas tau tshaj tawm hauv txhua 100,000 tus neeg hauv ib xyoos. Yog li koj tuaj yeem xav txog nws tsis tshua muaj npaum li cas.

Cov tsos mob yog dab tsi?

Goodpasture syndrome yog ib qho mob '(pulmonary-renal)'. Qhov no txhais tau tias ob lub ntsws thiab lub raum raug cuam tshuam tib lub sijhawm. Feem ntau, cov tsos mob thawj zaug uas tshwm sim yog cuam tshuam nrog lub ntsws. Cov no yog:

• Ua pa nyuaj (ua tsis taus pa): Tsis yog thaum nce ntaiv xwb, tab sis txawm tias thaum koj so.
• Mob hauv siab : Mob hauv siab thaum ua pa lossis hnoos.
• Hnoos : Hnoos tas li, qee zaum mob.
• Muaj suab nrov los ntawm hauv siab thaum ua pa : Tej zaum nws yuav yog lub suab gurgling, zoo li muaj ib yam dab tsi daig hauv siab.
• Kuv xav tias nkees heev.`(qaug zog)`: Ib qho kev xav tias koj tsis muaj zog ua dab tsi li.
• Ntshav ntswg (epistaxis).
• Hnoos ntshav : Qhov no yog ib qho cim uas ua rau ntau tus neeg ntshai thiab xav tau kev kho mob tam sim ntawd.
• Pallor : Daim tawv nqaij hloov daj vim tsis muaj ntshav.

Cov tsos mob ntsig txog lub raum kuj tseem yuav tshwm sim tom qab. Lawv suav nrog:

• Kev txo qis ntawm cov qe ntshav liab hauv cov ntshav, uas yog, ntshav tsis txaus .
• Ntshav nyob hauv cov zis (hematuria): Cov zis yuav hloov xim liab los yog xim av tsaus.
• Cov zis tawm tsawg dua li niaj zaus.
• Ntshav siab .
• Xeev siab thiab ntuav : Qhov no kuj tuaj yeem tshwm sim nrog kev tsis qab los noj mov.

Dab tsi ua rau qhov no?

Goodpasture syndrome tshwm sim thaum cov tshuaj tua kab mob uas peb lub cev tiv thaiv kab mob tsim tawm tsam cov collagen hauv glomerular basement membrane (GBM) hauv lub raum. GBM no yog ib feem ntawm glomeruli, cov chav lim ntshav me me hauv peb lub raum. Cov tshuaj tua kab mob no kuj tawm tsam cov collagen hauv cov hnab cua hauv lub ntsws. Qhov no ua rau cov ntaub so ntswg hauv lub ntsws raug rhuav tshem, ua rau los ntshav thiab ua rau nws nyuaj rau ua pa.

Tseem tsis tau paub meej tias yog vim li cas lub cev tiv thaiv kab mob thiaj li tawm tsam nws cov collagen. Tej zaum cov yam ntxwv ib puag ncig thiab peb cov noob caj noob ces yuav koom nrog rau hauv qhov mob no. Cov kev tshawb fawb tau pom tias cov neeg uas muaj tus kab mob no muaj kev sib raug zoo nrog cov cim qhia txog noob caj noob ces hu ua Human Leukocyte Antigen (HLA) DR15. Qhov no yog ib qho protein uas pab lub cev tiv thaiv kab mob paub qhov txawv ntawm peb cov nqaij thiab cov khoom txawv teb chaws.

Qee zaum, tus kab mob no tuaj yeem tshwm sim tom qab muaj kev kis kab mob, xws li mob khaub thuas lossis mob ntsws. Tsis tas li ntawd, koj:

• Yog koj haus luam yeeb .
• Yog koj haus cov tshuaj yeeb xws li cocaine .
• Qhov kev pheej hmoo ntawm kev mob no yuav siab dua yog tias koj raug cov hmoov hlau lossis cov tshuaj hydrocarbon xws li methane thiab propane.

Qhov no cuam tshuam rau leej twg tshaj plaws?

Tus mob Goodpasture syndrome no tshwm sim tau rau txhua lub hnub nyoog, tab sis feem ntau nws tshwm sim rau cov neeg hluas, ntawm kaum thiab peb caug xyoo, thiab tom qab ntawd dua rau cov neeg uas muaj hnub nyoog 60 thiab 70 xyoo.

Yuav kuaj mob li cas?

Thaum koj mus ntsib kws kho mob, lawv yuav nug txog koj cov tsos mob thiab kuaj koj lub cev. Tsis tas li ntawd xwb, lawv kuj tseem yuav ua cov kev kuaj mob hauv qab no kom paub tseeb tias koj muaj tus mob Goodpasture syndrome:

• Kev kuaj ntshav : Cov no xyuas koj qhov kwv yees glomerular filtration rate (eGFR), uas ntsuas seb koj lub raum ua haujlwm zoo npaum li cas.
• Kev kuaj zisKev kuaj zis: Qhov no kuaj seb puas muaj ntshav hauv koj cov zis lossis muaj cov protein ntau (proteinuria).
• Kev kuaj duab : Yuav ua ib qho kev ntsuas X-ray hauv siab lossis CT scan los xyuas seb puas muaj kev puas tsuaj rau lub ntsws.
• Bronchoscopy : Hauv cov txheej txheem no, tus kws kho mob ntxig ib lub raj nyias, ntev (bronchoscope) nrog lub koob yees duab thiab lub teeb txuas los ntawm koj lub qhov ntswg lossis qhov ncauj los xyuas seb puas muaj kev puas tsuaj hauv koj lub ntsws.
• Kev kuaj mob raum : Hauv qhov no, ib daim ntaub me me raug coj los ntawm lub raum thiab kuaj xyuas hauv qab lub tshuab microscope kom paub tseeb tias puas muaj kab mob raum.

Puas kho tau Goodpasture syndrome?

Yog, qhov no kho tau yog tias kuaj pom tus kab mob kom raug thiab pib kho sai.

Yuav kho li cas?

Kev kho mob rau Goodpasture syndrome sib txawv nyob ntawm seb qhov mob hnyav npaum li cas. Yog tias qhov mob tsis hnyav dhau, cov kws kho mob yuav sau ntawv kom noj tshuaj xws li:

• Cov tshuaj Corticosteroids : Piv txwv li, cov tshuaj xws li prednisone. Cov no pab tiv thaiv kev los ntshav hauv lub ntsws.
• Cov tshuaj tiv thaiv kab mob : Cov tshuaj xws li cyclophosphamide. Cov no tiv thaiv koj lub cev tiv thaiv kab mob kom tsis txhob tawm tsam nws cov nqaij.
• Cov tshuaj txo cov ntshav siab (antihypertensives): Cov no pab tswj cov ntshav siab thiab txo qhov kev puas tsuaj rau lub raum.

Ntxiv rau qhov ntawd, cov kws kho mob kuj siv kev kho mob hu ua plasmapheresis . Qhov no suav nrog kev siv koob txhaj ntshav los ntawm cov hlab ntsha hauv koj txhais caj npab, cais cov kua ntawm cov ntshav, hu ua plasma, ntawm cov qe ntshav. Cov tshuaj tua kab mob anti-GBM uas tsis zoo nyob hauv cov plasma no. Tom qab ntawd cov plasma raug tshem tawm, thiab cov plasma los ntawm tus neeg noj qab haus huv raug ntxiv rov qab rau hauv koj cov qe ntshav thiab muab rov qab rau koj. Hais yooj yim, nws tshem tawm cov tshuaj tua kab mob uas tsis zoo ntawm koj lub cev.

Yuav siv sijhawm ntev npaum li cas thiaj rov zoo tom qab kho tas?

Koj yuav tau noj tshuaj tiv thaiv kab mob li ntawm 6 txog 12 lub hlis. Feem ntau, koj yuav tau ua plasmapheresis txhua hnub rau ob peb lub lis piam.

Yuav muaj dab tsi tshwm sim tom ntej?

Yog tias tsis kho, Goodpasture syndrome tuaj yeem ua rau muaj kev phom sij rau lub neej los ntawm kev ntshav ntawm lub ntsws thiab lub raum tsis ua haujlwm. Txawm li cas los xij, yog tias tus kab mob raug kuaj pom thaum ntxov thiab pib kho, qhov tshwm sim feem ntau zoo. Koj lub cev tsuas yog tsim cov tshuaj tua kab mob phem rau lub sijhawm luv luv - txij li ob peb lub lis piam mus txog li ob xyoos. Kev rov qab mob lossis rov tshwm sim dua yog tsawg heev.

Qhov teeb meem loj tshaj plaws ntawm qhov no yog kev ua haujlwm ntawm lub raum. Yog tias qhov ntawd tshwm sim, yuav tsum tau siv tshuaj dialysis los lim cov ntshav.Yuav tsum tau hloov raum.

Kuv muaj lus dab tsi hais txog kuv lub neej?

Kwv yees li ntawm 80% ntawm cov neeg uas muaj tus kab mob Goodpasture syndrome muaj feem yuav muaj sia nyob tau tsib xyoos yog 80%. Yog tias kuaj pom tus kab mob thaum ntxov thiab kho kom zoo.

Qhov no puas tiv thaiv tau?

Tej zaum yuav tsis muaj peev xwm tiv thaiv tau tag nrho. Txawm li cas los xij, koj tuaj yeem txo koj qhov kev pheej hmoo los ntawm kev zam qee yam:

• Hydrocarbons, uas txhais tau tias tej yam xws li methane, propane, roj av, kerosene, thiab tar.
• Hmoov hlau.
• Tshuaj yeeb.
• Cov khoom siv pleev xim rau plaub hau.

Kuv yuav saib xyuas kuv tus kheej li cas?

Yog tias koj muaj tus mob Goodpasture syndrome, nws yog ib lub tswv yim zoo los ua cov no:

• Tsum haus luam yeeb tag nrho. Tsis txhob nyob rau hauv qhov chaw uas lwm tus neeg haus luam yeeb (piv txwv li, tsis txhob raug 'pa luam yeeb thib ob').
• Ua si nawv tsawg kawg yog 30 feeb txhua hnub.
• Sim txo kev ntxhov siab .

Kuv yuav tsum mus ntsib kws kho mob thaum twg?

Yog koj hnoos tawm ntshav, ua pa nyuaj, tso zis tsawg dua, lossis muaj lwm yam tsos mob uas teev saum toj no, mus ntsib kws kho mob tam sim ntawd. Kev kuaj pom thaum ntxov ua rau koj muaj feem rov zoo dua. Yog tias tsis kho, nws tuaj yeem ua rau lub raum puas tsuaj mus tas li thiab txawm tias muaj teeb meem ntsws tuag taus.

Koj yuav tsum nug tus kws kho mob li cas?

Thaum koj mus ntsib kws kho mob, koj tuaj yeem nug cov lus nug no:

• Kuv yuav paub tseeb li cas yog tias kuv muaj tus kab mob Goodpasture Syndrome?
• Kuv tus mob hnyav npaum li cas?
• Koj pom zoo kom muaj kev kho mob dab tsi?
• Kuv ua tau dab tsi los saib xyuas kuv ob lub raum?
• Kuv puas yuav tsum mus ntsib kws kho mob nephrologist lossis kws kho mob pulmonologist?
• Koj puas qhia tau kuv txog ib pawg neeg txhawb nqa uas pab cov neeg uas muaj tus kab mob no?

Peb yam tseem ceeb (Triad) ntawm Goodpasture syndrome yog dab tsi?

Hauv kev kho mob, "triad" txhais tau tias yog peb yam tsos mob tseem ceeb uas piav qhia txog ib qho mob. Peb yam tsos mob tseem ceeb ntawm Goodpasture Syndrome yog:

• Ntshav los ntawm lub ntsws.
• Kev mob ntawm lub raum nce ntxiv sai.
• Qhov muaj cov tshuaj tiv thaiv kab mob hu ua "anti-GBM antibodies" nyob rau hauv lub cev.

Muaj tus kab mob no ua rau koj nyuaj siab thiab kho siab heev, tshwj xeeb tshaj yog vim tseem tsis tau paub meej tias yog vim li cas. Nws tuaj yeem cuam tshuam rau koj tej dej num niaj hnub, thiab tej zaum koj yuav tau hloov haujlwm yog tias koj ua haujlwm nrog hmoov hlau, roj av, lossis roj av nplaum.

Thaum kawg, yuav tsum nco ntsoov li cas

Yog li ntawd, Goodpasture Syndrome yog ib yam dab tsi uas yuav tsum ntshai, tab sis yog tias koj paub cov tsos mob thaum ntxov, mus ntsib kws kho mob sai sai, thiab pib kho, koj tuaj yeem tau txais cov txiaj ntsig zoo heev. Yuav tsum paub txog kev hloov pauv hauv koj lub cev. Yog tias koj xav tias muaj qee yam txawv txawv, nws yog ib qho tseem ceeb uas yuav tsum nrhiav kev pab kho mob yam tsis txhob xav tias nws yog ib yam me me. Koj tsis nyob ib leeg, thiab cov kws kho mob tuaj yeem pab koj.

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