Isifo se-membrane se-anti-glomerular engaphansi

Izifo ezingama-anti-glomerular basement ulwelwesi (izifo ezilwa ne-GBM) yisifo esingajwayelekile esingahle sihlanganise ukusilela kwezinso ngokushesha kanye nesifo samaphaphu.

Ezinye izinhlobo zalesi sifo zibandakanya iphaphu noma izinso kuphela. Isifo se-Anti-GBM sasaziwa ngokuthi yi-Goodpasture syndrome.

Isifo se-Anti-GBM yisifo sokuzimela komzimba. Kwenzeka lapho amasosha omzimba ngephutha ehlasela futhi abhubhise izicubu zomzimba ezinempilo. Abantu abanalesi sifo bahlakulela izinto ezihlasela iphrotheni okuthiwa i-collagen ezingxenyeni ezincane zomoya emaphashini nasezakhi zokuhlunga (i-glomeruli) zezinso.

Lezi zinto zibizwa ngokuthi ngama-antibodies ulwelwesi lwangaphansi kwamagciwane. Ulwelwesi lwangaphansi kwe-glomerular luyingxenye yezinso ezisiza ukuhlunga imfucuza noketshezi olungeziwe egazini. Ama-antibodies ulwelwesi lwangaphansi kwamagciwane angama-antibodies alwa nale ulwelwesi. Bangalimaza ulwelwesi olungaphansi, olungaholela ekulimaleni kwezinso.

Kwesinye isikhathi, lesi sifo sibangelwa ukutheleleka okuphefumula ngegciwane noma ngokuphefumula kumakhemikhali e-hydrocarbon. Ezimweni ezinjalo, amasosha omzimba angahlasela izitho noma izicubu ngoba ziwenza amaphutha kula magciwane noma amakhemikhali angaphandle.

Ukuphendula okuyiphutha kohlelo lokuzivikela komzimba kubangela ukopha emasakeni emoyeni amaphaphu nokuvuvukala ezingxenyeni zokuhlunga izinso.

Izimpawu zingavela kancane kakhulu ezinyangeni noma ngisho neminyaka, kepha zivame ukukhula ngokushesha okukhulu ezinsukwini kuya emasontweni.

Ukuphelelwa isifiso sokudla, ukukhathala nobuthakathaka yizimpawu ezivamile zokuqala.

Izimpawu zamaphaphu zingafaka:

• Akhwehlele igazi
• Ukukhwehlela okomile
• Ukuphelelwa umoya

Izinso nezinye izimpawu zifaka:

• Umchamo onegazi
• Umuzwa ovuthayo lapho uchama
• Isicanucanu nokuhlanza
• Isikhumba esiphaphathekile
• Ukuvuvukala (edema) kunoma iyiphi indawo yomzimba, ikakhulukazi emilenzeni

Ukuhlolwa komzimba kungaveza izimpawu zomfutho wegazi ophakeme kanye nokugcwala ngokweqile koketshezi. Umhlinzeki wezempilo angayizwa imisindo yenhliziyo namaphaphu engajwayelekile lapho elalele isifuba nge-stethoscope.

Imiphumela ye-urinalysis imvamisa ayijwayelekile, futhi ikhombisa igazi namaprotheni emchameni. Kungabonakala amaseli abomvu angajwayelekile.

Ukuhlolwa okulandelayo kungenziwa futhi:

• Ukuhlolwa kwe-membrane engaphansi kwe-antiglomerular
• Igazi legazi lomzimba
• BUN
• I-x-ray yesifuba
• I-Creatinine (serum)
• I-biopsy yamaphaphu
• Ukucubungula izinso

Inhloso enkulu ukususa ama-antibody ayingozi egazini. Ukwelashwa kungafaka:

• I-Plasmapheresis, esusa ama-antibody ayingozi ukusiza ukunciphisa ukuvuvukala ezinso nasemaphashini.
• Imithi ye-Corticosteroid (efana ne-prednisone) nezinye izidakamizwa, ezicindezela noma zithulisa amasosha omzimba.
• Imithi efana ne-angiotensin-converting enzyme (ACE) inhibitors kanye ne-angiotensin receptor blockers (ARBs), esiza ukulawula umfutho wegazi.
• I-Dialysis, engenziwa uma ukwehluleka kwezinso kungasakwazi ukwelashwa.
• Ukufakelwa kwezinso, okungenziwa lapho izinso zakho zingasasebenzi.

Ungatshelwa ukuthi unciphise ukudla kwakho usawoti noketshezi ukulawula ukuvuvukala. Kwezinye izimo, kunganconywa iprotheni ephansi ukuya kokulingene.

Lezi zinsizakusebenza zinganikeza imininingwane ethe xaxa ngesifo se-anti-GBM:

• National Institute of Diabetes and Digestive and Kidney Diseases - www.niddk.nih.gov/health-information/kidney-disease/glomerular-diseases/anti-gbm-goodpastures-disease
• Isisekelo Sezinso Sikazwelonke - www.kidney.org/atoz/content/goodpasture
• Inhlangano Kazwelonke Yezinkinga Ezingajwayelekile - rarediseases.org/rare-diseases/goodpasture-syndrome

Ukuxilongwa kusenesikhathi kubaluleke kakhulu. Isimo sibi kakhulu uma izinso sezivele zonakaliswe kakhulu lapho ukwelashwa kuqala. Ukulimala kwamaphaphu kungahle kusuka kubumnene kuya kokubi.

Abantu abaningi bazodinga i-dialysis noma ukufakelwa izinso.

Ukungalashwa, lesi simo singaholela kunoma yikuphi okulandelayo:

• Isifo sezinso esingalapheki
• Isifo sezinso sokugcina
• Ukwehluleka kwamaphaphu
• I-glomerulonephritis eqhubeka ngokushesha
• Ukopha kakhulu kwamaphaphu (ukuphuma kwegazi emaphashini)

Shayela i-aphoyintimenti nomhlinzeki wakho uma ukhiqiza umchamo omncane, noma unezinye izimpawu zesifo sokulwa ne-GBM.

Ungalokothi uhogele i-glue noma i-siphon uphethiloli ngomlomo wakho, obeka amaphaphu kuma-solvents we-hydrocarbon futhi angadala isifo.

I-Goodpasture syndrome; I-glomerulonephritis eqhubekayo ngokushesha ene-pulmonary hemorrhage; Isifo se-renal syndrome; I-Glomerulonephritis - ukuphuma kwegazi kwamaphaphu

• Ukunikezwa kwegazi lezinso
• I-Glomerulus nephron

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I-Phelps RG, i-Turner AN. Isifo se-membrane se-anti-glomerular engaphansi nesifo se-Goodpasture. Ku: Feehally J, Floege J, Tonelli M, Johnson RJ, abahleli. I-Nephrology Yomtholampilo Ephelele. Umhlaka 6. IPhiladelphia, PA: Elsevier; 2019: isahluko 24.

URadhakrishnan J, Appel GB, D'Agati VD. Isifo se-glomerular sesibili. Ku: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, ama-eds. IBrenner neRector's The Kidney. Umhla ka-11. IPhiladelphia, PA: Elsevier; 2020: isahluko 32.