E kamaʻilio kākou e pili ana i kahi maʻi laha ʻole i kapa ʻia ʻo C3G (Complement 3 Glomerulopathy), ka mea e hoʻopilikia ai i nā puʻupaʻa?

Ke ʻike nei ʻoe i ke koko i loko o kou mimi i kekahi manawa, a i ʻole ka pehu ʻana i kou mau wāwae? A i ʻole ke luhi mau nei ʻoe? Hiki i kēia ke kumu o kahi pilikia me kou mau puʻupaʻa. I kēia lā e kamaʻilio mākou e pili ana i kahi kūlana kakaikahi akā koʻikoʻi loa e hoʻopilikia ai i nā puʻupaʻa. ʻO ia hoʻi ʻo C3G (C Three G) a i ʻole "Complement 3 Glomerulopathy".

He aha ka C3G (Complement 3 Glomerulopathy)?

I ka ʻōlelo maʻalahi, ʻo C3G kahi hui o nā maʻi e hoʻopilikia i ka hana maikaʻi o kou mau puʻupaʻa. Ua ʻike ʻoe, ua like nā puʻupaʻa me nā kānana ʻelua i loko o ko mākou kino. ʻO kā lākou hana nui ke kānana i ke koko, wehe i nā huahana ʻōpala pono ʻole a me ka wai keu, a hana i ka mimi.

He protein kūikawā ʻo "Complement 3" i loko o ko kākou koko. He mea nui loa ia no ka ʻōnaehana pale e hakakā nei i nā maʻi i loko o ko kākou kino. ʻO "Glomerulopathy" ka hōʻino ʻia ʻana o nā glomeruli, ʻo ia hoʻi nā kīʻaha koko liʻiliʻi i loko o nā puʻupaʻa, e like me nā ʻupena liʻiliʻi. ʻO kēia mau glomeruli nā mea e kānana i ke koko.

A laila, he maʻi ʻo C3G e pili ana i ka protein `complement 3`, e hōʻino ana i ka `glomeruli` a e hoʻopilikia ana i ka hana o nā puʻupaʻa. I ka wā ma mua, ʻo ia hoʻi, ma mua o 2013, ua kapa ʻia kēia e nā kauka ʻo ``Membranoproliferative glomerulonephritis (MPGN) ʻano I, II a i ʻole III`` a i ʻole ``Mesangioproliferative glomerulonephritis``. Akā i kēia manawa, ua kapa ʻia kēia hui o nā maʻi ʻo C3G.

Aia kekahi mau ʻano C3G?

ʻAe, ʻelua ʻano nui o C3G. Hoʻoholo nā kauka i kēia mau ʻano ʻelua ma ka lawe ʻana i kahi ʻāpana liʻiliʻi o ka puʻupaʻa (kapa mākou ia he 'kidney biopsy ') a nānā iā ia ma lalo o kahi microscope, ma muli o ka hōʻino a me ka mumū i ka 'glomeruli'.

• ʻO ka maʻi waiho paʻa (DDD): I ʻike mua ʻia ʻo MPGN ʻano II, ua hōʻailona ʻia kēia ʻano maʻi e ka hoʻokumu ʻia ʻana o nā waiho paʻa, e like me ka lipine i loko o ka membrane basement glomerular (GBM), ka uhi o nā glomeruli. Hoʻopilikia pinepine ia i nā keiki a me nā ʻōpio i ko lākou mau makahiki he 20 mua.

• ʻO `C3 glomerulonephritis` (C3GN): Ua kapa ʻia ma mua ʻo `MPGN ʻano I` a i ʻole `III`. Ma kēia, ʻaʻole ʻike ʻia nā waihona paʻa e like me nā mea i ʻike ʻia ma DDD. Hoʻopilikia nui kēia i ka poʻe ma luna o 30 mau makahiki.

He maʻi mau anei ʻo C3G?

ʻAe, he maʻi mau loa ʻo C3G. ʻO ke ʻano kēia, ʻaʻole ia he mea e ulu koke a maikaʻi aʻe, a hiki ke hoʻonui ʻia ka pōʻino i nā puʻupaʻa i ka hala ʻana o ka manawa. No laila, he mea nui loa e makaʻala i kēia.

Pehea ka maʻamau o kēia C3G?

He maʻi laha ʻole kēia.Ua manaʻo nā kauka e hana ʻia kēia ma kahi o ʻelua a ʻekolu mau kānaka no kēlā me kēia haneli tausani. No laila, ʻaʻole paha ʻoe i lohe nui e pili ana i ia mea. Akā, ʻoiai he mea kakaikahi ia, he mea nui e makaʻala i ia mea.

He aha nā hōʻailona o ka maʻi C3G?

I kēia manawa, e nānā kākou i nā hōʻailona o kēia maʻi C3G. Loaʻa i nā ʻano ʻelua kekahi mau hiʻohiʻona maʻamau, he mea nui e ʻike:

• Koko i loko o ka mimi ( hematuria ): I kekahi manawa ʻulaʻula paha ka mimi, a he liʻiliʻi paha ia e ʻike wale ʻia ma lalo o kahi microscope.
• ʻO ka protein keu i loko o ka mimi (proteinuria): Hiki i kēia ke hana i ka mimi e ʻike ʻia he huʻa.
• Ka emi ʻana o ka mimi ma mua o ka maʻamau (oliguria).
• Ua emi ka nui o ka protein i loko o ke koko (hypoalbuminemia).
• Pehu (edema): ʻOi loa aku ma nā lima, nā kuʻekuʻe wāwae, a me nā wāwae. E noʻonoʻo, inā ala ʻoe i ke kakahiaka a pehu kou mau wāwae, hiki ke like me kēia.
• Ke koko kiʻekiʻe (hypertension).
• Nā maʻi hoʻi e kū hou ana.
• Manaʻo luhi loa ('Luhi'): ʻAʻole wale ka luhi, akā, he manaʻo o ka luhi loa me ka nānā ʻole i ka nui o kāu hiamoe.
• Hiki i kekahi poʻe ke loaʻa i nā maʻi e like me ka gout .

Hiki i kēia mau hōʻailona ʻelua ke alakaʻi i ka hāʻule ʻana o nā puʻupaʻa . Hiki i ka hāʻule ʻana o nā puʻupaʻa ke hana i nā hōʻailona e like me:

• Luhi mau.
• Ka luaʻi, ka luaʻi.
• Ka paʻakikī i ka hoʻoholo ʻana i ka manaʻo, ka manaʻolana ʻole.
• Pehu kino.
• Ka hele ʻana o ka mimi ma mua o ka maʻamau (hiki ke ʻike ʻia kēia i kekahi manawa i nā pae mua o ka hāʻule ʻana o nā puʻupaʻa).

Ma waho aʻe o nā puʻupaʻa, hiki i ka C3G ke hana i nā hōʻailona ʻē aʻe. ʻO kēia mau mea:

• Nā pilikia ʻike: Hiki ke hōʻino ʻia ka ʻike ma muli o ka hōʻiliʻili ʻana o nā waihona protein a me ka calcium (drusen), ʻoi aku hoʻi ma ka macula.
• Lipodystrophy: He ʻano kēia e nalowale ai ka momona ma kekahi mau wahi o ke kino (ʻoi aku ka maka a me nā lālā).

No ke aha i hoʻokumu ʻia ai kēia C3G?

ʻAe, e nānā kākou i ke kumu i hoʻokumu ʻia ai kēia C3G. He mea paʻakikī iki kēia, akā e wehewehe maʻalahi wau. Hana ʻia kēia ke hana pono ʻole ka "ʻōnaehana hoʻopihapiha" i loko o ko mākou kino. ʻO kēia "ʻōnaehana hoʻopihapiha" he hōʻiliʻili o nā protein i loko o ko mākou koko. He ʻāpana kēia o kā mākou ʻōnaehana pale. Kōkua kēia i ka hakakā ʻana i nā mea e like me ka bacteria a me nā virus e hele mai ana mai waho.

ʻO ka mea e hana ʻia i kekahi me C3G, ʻo ia ka hoʻāla nui ʻia ʻana o kēia 'ʻōnaehana hoʻopihapiha', a i ʻole ma waho o ke kaohi. A laila e hōʻino ʻia ka protein 'complement 3 (C3)'. Hoʻopaʻa ʻia kēia mau ʻāpana protein C3 i hōʻino ʻia i loko o nā 'glomeruli'. A laila lilo ia i mea paʻakikī no nā 'glomeruli' ke kānana i ke koko a hana i ka mimi. Inā ʻaʻole mālama ʻia, hiki i kēia hōʻino ke hoʻomau a alakaʻi i ka hāʻule ʻana o nā puʻupaʻa.

I ka hapa nui o ka manawa, he paʻakikī ke kuhikuhi pono i ke kumu e hele hewa ai kēia "ʻōnaehana hoʻomaikaʻi". Akā i kekahi manawa:

• Hiki ke ulu aʻe ʻo C3G ma muli o nā loli i loko o kā mākou mau genes (nā loli i loko o nā genes e pili ana i ka ʻōnaehana complement, e like me ``C3`` a me ``CFH``).
• ʻO kahi maʻi koko i kapa ʻia ʻo "Monoclonal gammopathy" hiki ke hōʻino pololei ʻole i nā puʻupaʻa, hoʻopilikia i ka hana o ka ʻōnaehana "complement", a alakaʻi i ka hoʻokumu ʻia ʻana o C3G. ʻO kēia "monoclonal gammopathy" kahi kūlana e hoʻopilikia ai i nā cell plasma i loko o ko kākou iwi iwi.
• Manaʻo nā kānaka noiʻi lapaʻau he mea i kapa ʻia ʻo "autoantibodies" paha kekahi e pāʻani i kahi kuleana. ʻO nā "Autoantibodies" he mau protein i hana ʻia e kā mākou ʻōnaehana pale ponoʻī e hoʻouka hewa ana i kā mākou mau ʻiʻo olakino ponoʻī. Eia nō naʻe, ʻaʻohe noiʻi lawa e ʻōlelo maopopo ai ʻo kēia mau "autoantibodies" ke kumu o C3G.

ʻO wai ka mea i hoʻopilikia nui ʻia e kēia maʻi C3G?

Hiki i kēia maʻi C3G ke ulu i loko o kekahi. Hoʻopilikia like ia i nā kāne a me nā wahine. ʻO ka awelika o ka makahiki o ka ʻike ʻia ʻana ma kahi o 23 mau makahiki. Eia nō naʻe, hiki ke ʻike ʻia ke ʻano `DDD` a mākou i ʻōlelo ai ma mua i ka wā ʻōpio ma mua o ke ʻano `C3GN`.

He aha nā pilikia e hiki mai ana o C3G?

ʻO ka pilikia nui a weliweli loa, ʻo ia ka hiki ʻana o kahi 50% o ka poʻe me C3G, a i ʻole hoʻokahi i loko o ʻelua, i ka hāʻule ʻana o ka puʻupaʻa i loko o 10 mau makahiki o ka hōʻoia ʻana. ʻO kēia ka mea a kākou e makaʻu nui ai.

Pehea e ʻike ʻia ai ka maʻi C3G?

E hana kekahi kauka i kekahi mau hoʻokolohua e nānā i ke olakino o kou mau puʻupaʻa a me ka maikaʻi o kā lākou hana. Hiki i kēia mau mea ke komo pū me:

• Nā hoʻāʻo koko: Lawe ʻia kahi hāpana koko liʻiliʻi mai kahi aa koko ma kou lima a nānā ke keʻena hoʻokolohua inā maʻamau kāu mau protein complement a i ʻole he nui nā huahana ʻōpala (e like me ka creatinine) i loko o kou koko.
• ʻO ka helu kānana glomerular i manaʻo ʻia (eGFR): He hoʻāʻo kēia e ana i ka maikaʻi o ke kānana ʻana o kou mau puʻupaʻa i kou koko, ma muli o ka nui o ka creatinine serum a i ʻole cystatin C i loko o kahi hāpana koko. Hiki i kēia ke hāʻawi iā ʻoe i kahi manaʻo maikaʻi o ka hana maikaʻi o kou mau puʻupaʻa.
• ʻIke mimi: Pono ʻoe e mimi i loko o ke kīʻaha kūikawā a hāʻawi i ka hāpana i kāu kauka. E nānā ke keʻena hoʻokolohua no ka protein a i ʻole ke koko i loko o ka mimi, hiki ke ʻike wale ʻia ma lalo o kahi microscope.
• ʻO ka biopsy puʻupaʻa: ʻO kēia ka meaʻO ke ala maikaʻi loa e ʻike maopopo ai i ka maʻi C3G ma o ka biopsy. Ma kēia, lawe ke kauka i kahi ʻāpana liʻiliʻi o ka ʻiʻo mai kou puʻupaʻa me ka hoʻohana ʻana i kahi nila lahilahi a i ʻole kahi ʻoki liʻiliʻi a hoʻāʻo iā ia ma kahi lab. Inā loaʻa i kēia hāpana nā ʻāpana o ka protein C3 i hōʻino ʻia i loko o kou glomeruli, hiki ke lilo i maʻi C3G. Hiki i kēia biopsy ke haʻi iā ʻoe i ke ʻano o C3G āu (DDD a i ʻole C3GN), ma muli o ka loaʻa ʻana o kēlā mau waihona paʻa.

Hiki ke ho'ōla loa ʻia ʻo C3G?

ʻO ka mea pōʻino, ʻaʻohe lāʻau lapaʻau no ka maʻi C3G i kēia manawa. Akā, mai hopohopo, ke hoʻomau nei nā kauka a me nā mea noiʻi i ke aʻo ʻana i kēia maʻi a ke hana nei e ʻimi i nā lāʻau lapaʻau hou. Ke manaʻolana nei mākou e hiki mai ana kahi lāʻau lapaʻau maikaʻi aʻe i ka wā e hiki mai ana.

He aha nā lāʻau lapaʻau no C3G?

ʻAʻohe lāʻau lapaʻau kikoʻī i ʻāpono ʻia e ka U.S. Food and Drug Administration (FDA) no C3G i kēia manawa. Eia nō naʻe, hiki i nā kauka ke kuhikuhi i nā ʻano lāʻau lapaʻau like ʻole a me ka mālama kākoʻo e hoʻolohi i ka holomua o C3G, kaohi i nā hōʻailona, ​​a hōʻemi i ka hōʻino ʻana o nā puʻupaʻa. ʻO kēia mau mea:

• Nā lāʻau lapaʻau koko: ʻO nā lāʻau lapaʻau e like me nā angiotensin-converting enzyme (ACE) inhibitors a me nā angiotensin II receptor blockers (ARBs) e hoʻemi i ke kaomi i loko o ka glomeruli. Hoʻemi kēia i ka nui o ka protein i loko o ka mimi a hoʻemi i ke kaumaha ma luna o nā puʻupaʻa.
• Nā lāʻau hoʻohaʻahaʻa kolesterol: Hiki i kāu kauka ke kuhikuhi i nā lāʻau lapaʻau e like me nā statins e hoʻohaʻahaʻa i ka cholesterol a hōʻemi i ka hōʻino ʻana i kou mau puʻupaʻa.
• Nā hoʻololi ʻana i ka ʻai: E kōkua kekahi meaʻai no nā puʻupaʻa iā ʻoe e hoʻomohala i kahi hoʻolālā ʻai kūpono iā ʻoe a kōkua iā ʻoe e mālama i ke kaumaha olakino. ʻO ka hōʻemi ʻana i kāu ʻai ʻana i ka paʻakai (sodium), ka momona saturated, a me ka cholesterol hiki ke kōkua i ka hōʻemi ʻana i ka pōʻino o nā puʻupaʻa.
• ʻO Corticosteroids: ʻIke ʻia hoʻi he "steroids," he mau lāʻau anti-inflammatory kēia e hana ana ma ka hoʻōki ʻana i ka ʻōnaehana pale mai ka hoʻouka ʻana i kāu glomeruli.
• ʻEculizumab: He lāʻau antibody monoclonal kēia i hoʻohana ʻia e mālama i nā maʻi koko kakaikahi. Hiki iā ia ke kōkua i ka hoʻomaikaʻi ʻana i ka hana o nā puʻupaʻa ma ka hōʻemi ʻana i ka protein i loko o ka mimi a me ka hoʻonui ʻana i ka helu kānana glomerular i kekahi mau maʻi C3G.
• Dialysis: Ma kahi o 50% o ka poʻe me C3G ua ʻōlelo ʻia he hemahema ko lākou puʻupaʻa. ʻO ka dialysis ka wā e hāʻule ai nā puʻupaʻa, a ʻo ka hana a nā puʻupaʻa e hana ai (e hoʻomaʻemaʻe i ke koko, wehe i nā huahana ʻōpala, a me ke kahe keu) e hana ʻia me ke kōkua o kahi mīkini.
• Hoʻoili puʻupaʻa: He lāʻau lapaʻau ʻē aʻe kēia no ka hāʻule ʻana o ka puʻupaʻa. Wehe ke kauka lapaʻau i kou puʻupaʻa hāʻule a hoʻololi iā ia me kahi puʻupaʻa olakino mai kahi mea hāʻawi.

Inā loaʻa iā ʻoe ka 'DDD' a i ʻole 'C3GN' a loaʻa pū kekahi iā ʻoe ka 'monoclonal gammopathy', e paipai kāu kauka i nā hoʻokolohua hou aʻe e nānā i nā maʻi ʻē aʻe.

He aha ka nānā ʻana no ka maʻi C3G?

Ma kahi o ka hapalua o nā maʻi me C3G e loaʻa i ka hāʻule ʻana o nā puʻupaʻa i loko o 10 mau makahiki o ka ʻike ʻana. ʻAʻohe lāʻau lapaʻau no ka hāʻule ʻana o nā puʻupaʻa i kēia manawa. Eia nō naʻe, me ka ʻike pololei a me ka mālama koke ʻana, hiki iā ʻoe ke ola i kahi ola lōʻihi a olakino me ka ʻole o nā loli nui. No laila, he mea nui e noho mālie a hahai i ka ʻōlelo aʻo a kāu kauka.

Hiki ke pale ʻia ka maʻi C3G?

ʻO ka mea pōʻino, ʻaʻohe ala e pale aku ai i ka maʻi C3G i kēia manawa, no ka mea, hoʻoulu pinepine ʻia e nā kumu i waho o ko mākou mana.

Pehea wau e mālama ai iaʻu iho?

Inā loaʻa iā ʻoe ʻo C3G, na kāu kauka e hoʻomohala i ka hoʻolālā lapaʻau maikaʻi loa nāu. Hiki i kēia ke komo pū me ka lāʻau lapaʻau, ka mālama kākoʻo, a me nā hoʻololi ʻana i ke ʻano o ka nohona. Hiki i kāu hoʻolālā lapaʻau ke komo pū me kēia mau mea, a he mea nui e hahai pono i kēia mau ʻanuʻu:

• E lawe i kāu lāʻau lapaʻau e like me ke kauoha a kāu kauka. Mai haʻalele i hoʻokahi lā.
• Ke hoʻololi nei i kāu meaʻai, ʻoiai ka hōʻemi ʻana i ka paʻakai, ka ʻaila, a me ke kō, a me ka ʻai ʻana i nā huaʻai a me nā mea kanu hou aʻe.
• E hōʻalo i ka hoʻohana ʻana i nā lāʻau anti-inflammatory non-steroidal (NSAIDs) (e.g., nā lāʻau ʻeha e like me ibuprofen a me diclofenac) me ka ʻole o ke aʻo ʻana a ke kauka, no ka mea hiki iā lākou ke hōʻino i nā puʻupaʻa a hoʻolalelale i ka hāʻule ʻana o ka puʻupaʻa.
• Ke mālama nei i kou olakino noʻonoʻo. ʻO ka loaʻa ʻana o ia ʻano maʻi laha ʻole hiki ke hoʻolilo iā ʻoe i mehameha a makaʻu. Eia kekahi, ʻo ka ʻike ʻana e hiki i ka C3G ke hoʻoulu i ka hāʻule ʻana o ka puʻupaʻa e hoʻonui i kou pilikia o ka loaʻa ʻana o ke kaumaha. E kamaʻilio me kāu kauka e pili ana i kou olakino noʻonoʻo a e ʻimi i ke aʻo a me ka mālama ʻana inā pono. E nīnau i kāu kauka e pili ana i nā hui kākoʻo no ka poʻe me C3G. ʻO ka hālāwai ʻana me nā poʻe ʻē aʻe me C3G hiki ke kōkua i ka hōʻemi ʻana i ka mehameha a kaʻana like i nā ʻike.

Āhea wau e ʻike ai i ke kauka?

Inā loaʻa iā ʻoe nā hōʻailona o C3G, ʻoiai nā loli i loko o kou mimi (koko, huʻa, emi), pehu, a luhi nui paha, e ʻike koke i ke kauka. He mau hōʻailona paha kēia e hana pono ʻole ana kou mau puʻupaʻa.

Inā ua ʻike ʻia ʻoe me C3G, e kuhikuhi aku kāu kauka iā ʻoe i kahi loea i nā maʻi puʻupaʻa (he nephrologist). ʻO ia ka mea nāna e hāʻawi iā ʻoe i ka hoʻolālā lapaʻau maikaʻi loa.

He aha nā nīnau e pono iaʻu e nīnau aku i kaʻu kauka?

Ke ʻike ʻoe i ke kauka, mai kānalua e nīnau i nā nīnau e like me kēia:

• Pehea e ʻike pono ai iaʻu inā loaʻa iaʻu ʻo C3G?
• Inā ʻaʻohe oʻu C3G, he aha nā maʻi ʻē aʻe e loaʻa iaʻu?
• He aha ke ʻano o ka C3G iaʻu (DDD a i ʻole C3GN)?
• He aha nā lāʻau lapaʻau āu e ʻōlelo mai ai noʻu? Aia kekahi mau hopena ʻaoʻao?
• He aha nā hoʻololi e pono iaʻu e hana i kaʻu papaʻai?
• Manaʻo ʻoe e hāʻule ana koʻu mau puʻupaʻa? He aha kaʻu e hana ai e pale aku ai i kēlā hana?
• Hiki iaʻu ke ʻike i kahi nephrologist?

ʻO ka mea hope loa, pono wau e ʻōlelo ...

He maʻi laha ʻole a paʻakikī hoʻi ʻo C3G e hoʻopilikia ana i nā ʻāpana kānana liʻiliʻi i loko o nā puʻupaʻa. Inā loaʻa iā ʻoe ʻo C3G, hiki iā ʻoe ke ʻike i nā loli i kou mimi, ka pehu ʻana, ka ʻeha o nā hono, nā pilikia ʻike, a me ka luhi.

Hiki iā ʻoe ke ʻike i nā ʻano manaʻo like ʻole i kou ʻae ʻana i kēia hōʻailona a hoʻololi i ke ʻano o ka hopena o C3G i kou ola. Mai hopohopo, ʻaʻole ʻoe hoʻokahi. E hāʻawi iā ʻoe iho i ka manawa a me kahi e aʻo ai e pili ana iā C3G a me kāu mau koho lapaʻau. ʻO ke aʻo ʻana e pili ana i kāu mau koho a me nā mea e manaʻo ai e hiki ke kōkua iā ʻoe e hana me kou mau manaʻo ma ke ʻano olakino a huahua. Aia nā kauka ma laila e hāʻawi i ka ʻike a me ke alakaʻi. Inā he mau nīnau kāu, pono i ka ʻōlelo aʻoaʻo a i ʻole ke kākoʻo, e kamaʻilio me lākou. E hoʻomanaʻo, me ka ʻike mua a me ka mālama kūpono, hiki iā ʻoe ke kāohi i kēia maʻi a ola i kahi ola maikaʻi.

ʻO C3G, maʻi puʻupaʻa, complement 3, glomerulopathy, hāʻule puʻupaʻa, protein mimi, dialysis