ʻO ka maʻi membrane anti-glomerular basement

ʻO nā maʻi membrane anti-glomerular basement membrane (anti-GBM maʻi) kahi maʻi laha ʻole e hiki ke hoʻopili koke i ka hōʻino ʻana o ka puʻupaʻa a me ka maʻi māmā.

Pili kekahi ʻano o ka maʻi i ka lung a i ʻole ka hakuʻala. ʻO ka maʻi Anti-GBM i kapa ʻia ʻo Goodpasture Syndrome.

ʻO ka maʻi Anti-GBM kahi maʻi autoimmune. Kū i ka wā i hōʻeha hewa ka ʻōnaehana pale a hōʻino i nā kino o ke kino. Hoʻomohala ka poʻe me kēia maʻi i nā mea e hoʻouka i kahi protein i kapa ʻia collagen i loko o nā ʻeke ea liʻiliʻi i nā akemāmā a me nā wae kānana (glomeruli) o nā puʻupaʻa.

Kapa ʻia kēia mau mea waiwai ant anticlomerular basement membrane antibodies. ʻO ka membrane papa lalo glomerular kahi ʻāpana o nā puʻupaʻa e kōkua i ka kānana ʻōpala a me nā wai keu mai ke koko. ʻO nā antivlomerular basement membrane antibodies he mau antiodies e kūʻē i kēia membrane. Hiki iā lākou ke hōʻino i ka membrane o lalo, kahi e hiki ai ke hōʻeha i nā puʻupaʻa.

I kekahi manawa, hoʻonāukiuki ʻia kēia maʻi e ka maʻi hanu viral a i ʻole e ka hanu ʻana i nā mea hoʻoheheʻe hydrocarbon. I nā hihia pēlā, hiki i ka ʻōnaehana pale pale ke hoʻouka aku i nā ʻōnaehana a i ʻole nāʻaʻaʻa no ka mea kuhihewa ia iā lākou no kēia mau virus a i ʻole nā ​​kemika haole.

ʻO ka pane hewa o ka ʻōnaehana pale ke kumu o ke kahe ʻana o ke koko i loko o nā ʻeke lewa o nā akemāmā a me ka mumū ma nā wahi kānana o ka hakuʻala.

Lohi mālie paha nā ʻōuli ma nā mahina a i ʻole mau makahiki, akā ulu pinepine lākou i mau lā i mau pule.

ʻO ka lilo o ka makemake, ka luhi, a me ka nāwaliwali he mau ʻōuli mua ia.

Pākuʻi ʻia paha nā ʻōuli pūpū:

• ʻApu ʻana i ke koko
• Mū maloo
• Pokole o ka hanu

ʻO nā hakuʻala a me nā ʻōuli ʻē aʻe e pili pū me:

• Mimi koko
• ʻO ke ahi wela ke ʻimi
• ʻO Nusea a me ka luaʻi
• ʻIli pale
• ʻO ka pehu (edema) i kēlā me kēia wahi o ke kino, keu hoʻi i nā wāwae

Hiki i kahi hoʻokolokolo kino ke hōʻike i nā hōʻailona o ke kahe kiʻekiʻe a me ka hoʻoheheʻe o ka wai. Lohe paha ka mea mālama ola i nā kani a me nā puʻuwai maikaʻi ʻole ke hoʻolohe nei i ka umauma me kahi stethoscope.

ʻO nā hopena urinalysis pinepine ʻole, a hōʻike i ke koko a me ka protein i ka mimi. ʻIke ʻia paha nā hunaola ʻulaʻula maʻamau.

E hana ʻia paha nā hōʻike aʻe:

• ʻO ka hoʻāʻo ʻana o ka membrane Antiglomerular basement
• Ke kinoea koko arterial
• BUNU
• Pākuʻi x-ray
• Creatinine (serum)
• ʻO Biopsy Lung
• ʻO Biopsy hakuʻala

ʻO ka pahuhopu nui ka wehe ʻana i nā mea ʻino ʻino mai ke koko. Hiki ke hoʻopili ʻia i ka mālama ʻana:

• ʻO Plasmapheresis, kahi e hemo ai i nā mea ʻino e hōʻemi ai i ka mumū ma nā puʻupaʻa a me nā akemāmā.
• Nā lāʻau Corticosteroid (e like me prednisone) a me nā lāʻau ʻē aʻe, kahi e kaohi a hoʻomaha paha i ka ʻōnaehana pale.
• ʻO nā lāʻau e like me ka angiotensin-hoʻololi i ka enzyme (ACE) mea kāohi a me nā angiotensin receptor blockers (ARBs), i kōkua i ke kaohi i ke kahe o ke koko.
• Dialysis, i hiki ke hana inā ʻaʻole hiki ke mālama hou ʻia ka hoʻokō ʻana o ka hakuʻala.
• ʻO kahi transplan kidney, hiki ke hana ʻia ke hana ʻole kāu mau puʻupaʻa.

ʻ toldlelo ʻia paha ʻoe e kaupalena i kāu lawe ʻana i ka paʻakai a me nā wai e kaohi ai i ka pehu. I kekahi mau hihia, koi ʻia kahi papa protein protein haʻahaʻa a haʻahaʻa paha.

Hāʻawi paha kēia mau kumuwaiwai i ka ʻike hou aku e pili ana i ka maʻi 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
• National Kidney Foundation - www.kidney.org/atoz/content/goodpasture
• National Organization for Rare Disorder - rarediseases.org/rare-diseases/goodpasture-syndrome

He mea nui kahi ʻike kakahiaka. ʻOi aku ka maikaʻi o ka nānā inā ua hōʻino nui ʻia nā puʻupaʻa ke hoʻomaka ka lapaʻau. Hiki ke hōʻiliʻili ʻia ka hōʻino ʻana o ka maʻi mai ka palu a i ke koʻikoʻi.

Pono nā kānaka he nui i ka dialysis a i ʻole ke kuʻi ʻōiwi.

ʻAʻole mālama ʻia, hiki i kēia ʻano ke alakaʻi i kekahi o kēia mau mea:

• Maʻi maʻi ʻōniʻoniʻo
• ʻO ka maʻi hōʻeha hope loa
• Hoka maikaʻi ʻole
• ʻO glomerulonephritis holomua wikiwiki
• ʻO ka hemorrhage pulmonary nui

Kāhea i kahi manawa me kāu mea hāʻawi inā ʻoe e hōʻemi i ka mimi, a i ʻole he mau hōʻailona ʻē aʻe o ka maʻi anti-GBM.

Mai honi iki i ke kopa a i ʻole siphon gasolina me kou waha, kahi e hōʻike ai i nā akemāmā i nā mea hoʻoheheʻe hydrocarbon a hiki ke kumu i ka maʻi.

ʻO Goodpasture Syndrome; ʻO ka glomerulonephritis holomua wikiwiki me ka hemorrhage pulmonary; Pulmonary renal syndrome; Glomerulonephritis - pulemary hemorrhage

• Hāʻawi ke koko
• ʻO Glomerulus a me nephron

Collard HR, King TE, Schwarz MI. ʻO ka hemorrhage Alveolar a me nā maʻi infiltrative kakaikahi. I: Broaddus VC, Mason RJ, Ernst JD, et al, eds. ʻO Murray a me Nadel's Textbook of Respiratory Medicine. ʻElua huaʻōlelo Piladelapia, PA: Elsevier Saunders; 2016: chap 67.

ʻO Phelps RG, Turner AN. ʻO ka maʻi anti-glomerular basement membrane a me ka maʻi Goodpasture. I: Feehally J, Floege J, Tonelli M, Johnson RJ, eds. Nā Nephrology Haumāna piha. ʻElua huaʻōlelo Piladelapia, PA: Elsevier; 2019: chap 24.

Radhakrishnan J, Appel GB, DʻAgati VD. Maʻi glomerular lua. I: Yu ASL, Chertow GM, Luyckx VA, Marsden PA, Skorecki K, Taal MW, eds. ʻO Brenner a me Rector's The Kidney. 11th hua. Piladelapia, PA: Elsevier; 2020: chap 32.