Ua ʻike anei ʻoe e pili ana i kēia maʻi e hoʻopilikia ana i kou mau māmā a me nā puʻupaʻa i ka manawa like? E kamaʻilio kākou e pili ana i ka Goodpasture Syndrome!

Loaʻa iā ʻoe kekahi manawa ka ʻū mau? Ke hoʻāla mai nei kēlā ʻū i kekahi manawa i ke koko iki? A i ʻole ke manaʻo nei ʻoe e pilikia ana ʻoe i ka hanu ʻana, a luhi wale paha ʻoe a emi iho ka mimi? He mau hōʻailona paha kēia o kahi mea koʻikoʻi. I kēia lā e kamaʻilio mākou e pili ana i kekahi ʻano maʻi kakaikahi, akā hoʻokahi āu e makaʻala ai.

He aha ka Goodpasture Syndrome?

I ka ʻōlelo maʻalahi, ʻo Goodpasture Syndrome kahi maʻi autoimmune kakaikahi akā hiki ke hoʻoweliweli i ke ola. ʻO "Autoimmune" ke ʻano o ka ʻōnaehana pale o ko mākou kino, ka ʻōnaehana pale, e hoʻomaka hewa ana e hoʻouka i kā mākou mau hunaola a me nā ʻiʻo olakino ponoʻī.

I loko o kēia maʻi, hoʻouka kā mākou ʻōnaehana pale i kahi protein i kapa ʻia ʻo collagen. Ua like ke Collagen me kahi poloka hale i loko o ko mākou kino. Kōkua ke Collagen i ka mālama ʻana i ko mākou ʻili, nā ʻiʻo, nā tendon, nā ligaments, a me nā iwi i ikaika.

No laila, inā loaʻa iā ʻoe ka maʻi Goodpasture, ʻo kekahi o nā protein a kou kino e hana ai, kapa mākou iā lākou he antibodies, e hele a hoʻopili i ka collagen i loko o kou mau māmā a me nā puʻupaʻa. Ke hana ʻia kēia, hoʻomaka ka mumū ma kēlā mau wahi, a hoʻomaka kēlā ʻiʻo e haki mālie. Inā waiho ʻole ʻia, hiki i kēia ke alakaʻi i ka mumū nui o nā puʻupaʻa (glomerulonephritis), hiki ke alakaʻi i ka hāʻule ʻana o ka puʻupaʻa. Hiki iā ʻoe ke hoʻomaka e kahe nui ke koko mai nā māmā (pulmonary hemorrhage). I nā hihia he nui, ʻo ke kumu nui o ka make mai kēia maʻi, ʻo ia ke kahe ʻana mai nā māmā.

ʻO kekahi inoa ʻē aʻe no kēia:

• ʻO ka maʻi anti-glomerular basement membrane (anti-GBM)
• Maʻi Goodpasture

Pehea ka maʻamau o kēia?

He maʻi laha ʻole kēia. Wahi a nā kauka, hoʻokahi a ʻelua wale nō hihia hou o kēia maʻi i hōʻike ʻia i kēlā me kēia 100,000 poʻe i kēlā me kēia makahiki. No laila hiki iā ʻoe ke noʻonoʻo i kona laha ʻole.

He aha nā hōʻailona?

ʻO ka maʻi Goodpasture kahi maʻi `(pulmonary-renal)`. ʻO ke ʻano kēia, ua hoʻopilikia ʻia nā māmā a me nā puʻupaʻa i ka manawa like. ʻO ka pinepine, ʻo nā hōʻailona mua e ʻike ʻia e pili ana i nā māmā. Eia kēia:

• Paʻakikī ka hanu ʻana (dyspnea): ʻAʻole wale i ka wā e piʻi ai i nā alapiʻi, akā, i ka wā e hoʻomaha ai ʻoe.
• ʻEha umauma : ʻEha umauma i ka wā e hanu ana a i ʻole ke kuʻi ʻana.
• ʻŪhū : He ʻūhū mau, ʻeha i kekahi manawa.
• He walaʻau mai ka umauma mai i ka wā e hanu ana : He leo gurgling paha ia, me he mea lā ua paʻa kekahi mea i loko o ka umauma.
• Ua luhi loa wau.`(luhi)`: He manaʻo o ka nāwaliwali loa a ʻaʻole hiki iā ʻoe ke hana i kekahi mea.
• Kahe koko ihu (epistaxis).
• ʻUmi ʻana i ke koko : He hōʻailona kēia e hoʻoweliweli ai i nā poʻe he nui a pono e mālama koke ʻia e ke kauka.
• Keʻokeʻo : Lilo ka ʻili i ke keʻokeʻo ma muli o ka nele o ke koko.

Hiki ke ʻike ʻia nā hōʻailona pili i ka puʻupaʻa ma hope. ʻO lākou pū kekahi:

• ʻO ka emi ʻana o nā hunaola koko ʻulaʻula i loko o ke koko, ʻo ia hoʻi, anemia .
• Koko i loko o ka mimi (hematuria): Hiki i ka mimi ke ʻulaʻula a ʻeleʻele paha.
• ʻEmi ka mimi i hoʻopuka ʻia ma mua o ka maʻamau.
• Ke koko kiʻekiʻe .
• Ka luaʻi a me ka luaʻi ʻana : Hiki ke hana pū kēia me ka nalowale o ka makemake.

He aha ke kumu o kēia?

Hoʻomaka ka maʻi Goodpasture i ka wā e hoʻouka ai nā antibodies i hana ʻia e kā mākou ʻōnaehana pale i ka collagen i loko o ka glomerular basement membrane (GBM) i loko o nā puʻupaʻa. ʻO kēia GBM kekahi ʻāpana o ka glomeruli, nā ʻāpana kānana koko liʻiliʻi i loko o ko mākou mau puʻupaʻa. Hoʻouka pū kēia mau antibodies i ka collagen i loko o nā ʻeke ea i loko o nā māmā. ʻO kēia ke kumu o ka luku ʻia ʻana o nā ʻiʻo māmā, e hoʻoulu ai i ke kahe ʻana o ke koko a me ka paʻakikī o ka hanu ʻana.

ʻAʻole maopopo loa ke kumu e hoʻouka ai ka ʻōnaehana pale i kāna collagen ponoʻī. He hiki paha i nā mea pili i ke kaiapuni a me ko mākou mau genes ke komo i ke ʻano. Ua ʻike ʻia nā noiʻi he pilina ikaika ko ka poʻe me ka maʻi me kahi māka genetic i kapa ʻia ʻo Human Leukocyte Antigen (HLA) DR15. He protein kēia e kōkua ana i ka ʻōnaehana pale e hoʻokaʻawale ma waena o ko mākou mau ʻiʻo ponoʻī a me nā mea haole.

I kekahi manawa, hiki ke ʻike ʻia kēia maʻi ma hope o kahi maʻi, e like me ke anu a i ʻole ka maʻi palū. Eia kekahi, ʻo ʻoe:

• Inā ʻoe e puhipaka ana .
• Inā ʻoe e hanu i nā lāʻau lapaʻau e like me ka cocaine .
• ʻOi aku ke kiʻekiʻe o ka pilikia o ka loaʻa ʻana o kēia maʻi inā ʻoe e pili ana i ka lepo metala a i ʻole nā ​​​​kemika hydrocarbon e like me ka methane a me ka propane.

ʻO wai ka mea e hoʻopilikia nui nei i kēia?

Hiki ke loaʻa ka maʻi Goodpasture i kēlā me kēia makahiki, akā ʻoi aku ka maʻamau i ka poʻe ʻōpio, ma waena o nā makahiki he ʻumi a me kanakolu, a laila hou i ka poʻe i ko lākou mau makahiki 60 a me 70.

Pehea e hana ʻia ai ka hōʻailona?

Ke ʻike ʻoe i ke kauka, e nīnau lākou e pili ana i kāu mau hōʻailona a hana i kahi hoʻokolokolo kino. Eia kekahi, hiki iā lākou ke hana i kēia mau hoʻokolohua e hōʻoia i ka maʻi Goodpasture:

• Nā hoʻāʻo koko : Nānā kēia i kāu helu kānana glomerular i manaʻo ʻia (eGFR), kahi e ana ai i ka hana maikaʻi o kou mau puʻupaʻa.
• Nā hoʻāʻo mimiUrinalysis: Nānā kēia i ke koko i loko o kou mimi a i ʻole nā ​​​​pae protein kiʻekiʻe (proteinuria).
• Nā hoʻāʻo kiʻi : Hana ʻia kahi X-ray umauma a i ʻole CT scan e nānā no ka hōʻino ʻana o ka māmā.
• Bronchoscopy : Ma kēia kaʻina hana, hoʻokomo ke kauka i kahi ʻōmole lahilahi a lōʻihi (bronchoscope) me kahi kāmela a me ke kukui i hoʻopili ʻia ma kou ihu a waha paha e nānā i ka hōʻino i loko o kou mau māmā.
• ʻO ka biopsy puʻupaʻa : Ma kēia, lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻiʻo mai ka puʻupaʻa a nānā ʻia ma lalo o kahi microscope e hōʻoia inā loaʻa ka maʻi puʻupaʻa.

Hiki ke ho'ōla ʻia ka maʻi Goodpasture?

ʻAe, hiki ke ho'ōla ʻia kēia inā ʻike pololei ʻia ka maʻi a hoʻomaka koke ka lāʻau lapaʻau.

Pehea e mālama ʻia ai?

ʻOkoʻa ka lāʻau no ka maʻi Goodpasture ma muli o ke koʻikoʻi o ke kūlana. Inā ʻaʻole koʻikoʻi loa ke kūlana, hiki i nā kauka ke kuhikuhi i nā lāʻau lapaʻau e like me:

• ʻO Corticosteroids : No ka laʻana, nā lāʻau lapaʻau e like me prednisone. Kōkua kēia i ka hoʻōki ʻana i ke kahe koko i loko o nā māmā.
• Nā lāʻau pale kino : Nā lāʻau e like me ka cyclophosphamide. Hoʻōki kēia i kāu ʻōnaehana pale mai ka hoʻouka ʻana i kona mau ʻiʻo ponoʻī.
• Nā lāʻau hoʻohaʻahaʻa koko (nā lāʻau antihypertensive): Kōkua kēia i ka hoʻomalu ʻana i ke koko a hōʻemi i ka hōʻino ʻana i nā puʻupaʻa.

Eia kekahi, hoʻohana pū nā kauka i kahi lāʻau i kapa ʻia ʻo plasmapheresis . Pili kēia i ka lawe ʻana i ke koko mai kahi aʻa koko i loko o kou lima ma o kahi nila, e hoʻokaʻawale ana i ka ʻāpana wai o ke koko, i kapa ʻia ʻo plasma, mai nā hunaola koko. Aia nā antibodies anti-GBM ʻino i loko o kēia plasma. A laila wehe ʻia ka plasma, a hoʻohui ʻia ka plasma mai kahi kanaka olakino i kāu mau hunaola koko a hāʻawi ʻia iā ʻoe. I ka ʻōlelo maʻalahi, wehe ia i nā antibodies ʻino mai kou kino.

ʻEhia ka lōʻihi e hoʻōla ai ma hope o ka lāʻau lapaʻau?

Pono ʻoe e lawe i nā lāʻau immunosuppressant no kahi o 6 a 12 mau mahina. I nā manawa he nui, pono paha ʻoe e hana i ka plasmapheresis i kēlā me kēia lā no kekahi mau pule.

He aha ka mea e hiki mai ana?

Inā ʻaʻole mālama ʻia, hiki i ka maʻi Goodpasture ke alakaʻi i ke kahe koko e hoʻoweliweli ana i ke ola mai nā māmā a me ka hāʻule ʻana o nā puʻupaʻa. Eia nō naʻe, inā ʻike mua ʻia ka maʻi a hoʻomaka ʻia ka mālama ʻana, maikaʻi ka hopena. Hoʻopuka wale kou kino i kēlā mau antibodies ʻino no ka manawa pōkole - mai kekahi mau pule a i ʻelua mau makahiki. He mea kākaʻikahi loa nā relapses a i ʻole nā ​​​​recurrences.

ʻO ka pilikia koʻikoʻi loa o kēia, ʻo ia ka hāʻule ʻana o nā puʻupaʻa. Inā hana ʻia kēlā, pono ka dialysis e kānana i ke koko.He mea pono ka hoʻoili ʻana o ka puʻupaʻa.

He aha kaʻu e ʻōlelo ai e pili ana i koʻu ola?

Ma kahi o 80% o ka poʻe me ka maʻi Goodpasture he 80% ka nui o ke ola ʻana no ʻelima mau makahiki. ʻO ia hoʻi inā ʻike mua ʻia ka maʻi a mālama pono ʻia.

Hiki ke pale ʻia kēia?

ʻAʻole paha e hiki ke pale loa iā ia. Eia nō naʻe, hiki iā ʻoe ke hōʻemi i kou pilikia ma ka pale ʻana i kekahi mau mea:

• ʻO nā hydrocarbons, ʻo ia hoʻi nā mea e like me ka methane, propane, gasoline, kerosene, a me ke tar.
• Lepo hao.
• Kokaini.
• Nā huahana hoʻoluʻu lauoho.

Pehea e hiki ai iaʻu ke mālama iaʻu iho?

Inā loaʻa iā ʻoe ka maʻi Goodpasture, he mea maikaʻi ke hana i kēia mau mea:

• E hoʻōki loa i ka puhipaka. E pale i ka noho ʻana ma nā wahi e puhipaka ai kekahi poʻe (e pale i ka pili ʻana i ka 'uahi lua').
• E hana i ka hana kino no ka liʻiliʻi he 30 mau minuke i kēlā me kēia lā.
• E ho'āʻo e hōʻemi i ke kaumaha .

Āhea wau e ʻike ai i ke kauka?

Inā ʻoe e ʻū ana i ke koko, pilikia ka hanu ʻana, emi ka mimi ʻana, a i ʻole kekahi o nā hōʻailona ʻē aʻe i helu ʻia ma luna, e ʻike koke i ke kauka. ʻO ka ʻike mua ʻana e hoʻonui nui i kou manawa e hoʻōla ai. Inā ʻaʻole mālama ʻia, hiki iā ia ke hōʻeha mau i nā puʻupaʻa a me nā pilikia make o ka māmā.

He aha nā nīnau āu e nīnau ai i ke kauka?

Ke ʻike ʻoe i ke kauka, hiki iā ʻoe ke nīnau i kēia mau nīnau:

• Pehea e ʻike pono ai iaʻu inā loaʻa iaʻu ka Goodpasture Syndrome?
• Pehea ke koʻikoʻi o koʻu kūlana?
• He aha nā lāʻau lapaʻau āu e paipai nei?
• He aha kaʻu e hana ai e mālama ai i koʻu mau puʻupaʻa?
• Pono anei au e ʻike i kahi nephrologist a i ʻole kahi pulmonologist?
• Hiki iā ʻoe ke haʻi mai iaʻu e pili ana i kahi hui kākoʻo e kōkua ana i nā poʻe me kēia maʻi?

He aha nā hiʻohiʻona nui ʻekolu (Triad) o ka maʻi Goodpasture?

I ka lāʻau lapaʻau, ʻo ka "triad" e pili ana i ʻekolu mau hōʻailona nui e wehewehe ana i kahi maʻi. ʻO nā hōʻailona nui ʻekolu o Goodpasture Syndrome:

• Ke kahe ʻana o ke koko mai nā māmā.
• Ka piʻi wikiwiki ʻana o ka mumū o nā puʻupaʻa.
• ʻO ke alo o kēlā mau antibodies i kapa ʻia ʻo "anti-GBM antibodies" i loko o ke kino.

Hiki i ka loaʻa ʻana o kēia maʻi ke hoʻoluhi nui a mehameha hoʻi, ʻoiai ʻaʻole ʻike ʻia ke kumu pololei. Hiki iā ia ke hoʻopilikia i kāu mau hana o kēlā me kēia lā, a pono paha ʻoe e hoʻololi i nā hana inā ʻoe e hana me ka pauka metala, ka ʻaila, a i ʻole ke kēpau.

ʻO ka mea hope loa, he aha e hoʻomanaʻo ai

No laila, he mea makaʻu ka Goodpasture Syndrome, akā inā ʻike mua ʻoe i nā hōʻailona, ​​e ʻike koke i ke kauka, a hoʻomaka i ka mālama ʻana, hiki iā ʻoe ke loaʻa nā hopena maikaʻi loa. E makaʻala i nā loli i kou kino. Inā manaʻo ʻoe i kahi mea ʻē, he mea nui e ʻimi i ka ʻōlelo aʻoaʻo lapaʻau me ka ʻole o ka hoʻokuʻu ʻana iā ia he mea liʻiliʻi. ʻAʻole ʻoe hoʻokahi, a hiki i nā kauka ke kōkua iā ʻoe.

ʻO ka maʻi Goodpasture, ka maʻi Anti-GBM, ke kahe koko ʻana o ka pulmonary, ka maʻi puʻupaʻa, nā maʻi autoimmune, ka paʻakikī o ka hanu ʻana, ke koko i loko o ka mimi