Nā maʻi koʻikoʻi pinepine: Pono anei mākou e makaʻala i kēia maʻi laha ʻole (Maʻi Granulomatous Maʻi - CGD)?

Ke maʻi mau nei kāu keiki liʻiliʻi? Ke hoʻomaikaʻi kekahi maʻi, hele mai kekahi. Loaʻa iā ia i kekahi manawa nā maʻi koʻikoʻi ma ka ʻili, nā māmā, a i loko paha o ke kino? Hopohopo ka nui o nā mākua e pili ana i ia mau pilikia. I kēia lā e kamaʻilio mākou e pili ana i kahi ʻano genetic kakaʻikahi akā koʻikoʻi e hiki ai ke hoʻoulu i ia mau maʻi pinepine. Kāhea mākou i kēia maʻi Chronic Granulomatous, a i ʻole CGD no ka pōkole i waena o nā kauka.

I ka ʻōlelo maʻalahi, he aha ka CGD?

I mea e hoʻomaopopo ai i kēia, e kamaʻilio mua kākou e pili ana i ka ʻōnaehana pale o ko kākou kino. E noʻonoʻo ua like ko kākou kino me kahi ʻāina. Loaʻa iā ia kahi pūʻali koa i hiki ke pale aku iā ia mai nā ʻenemi haole (nā germs). ʻO ia ko kākou ʻōnaehana pale kino . ʻO kahi ʻāpana kūikawā o kēia pūʻali koa i kapa ʻia he mau keʻokeʻo koko .

I kēia manawa, eia ka mea e hana ʻia i kahi kanaka me CGD. Aia kahi hui kūikawā o nā hunaola koko keʻokeʻo i kapa ʻia he mau koa, no ka laʻana, `(neutrophils)`, `(monocytes)`, `(macrophages)` a me `(eosinophils)`. ʻO ka hana nui o kēia mau hunaola, ʻo ia ka loaʻa ʻana o kekahi mau ʻano bacteria a me nā fungi e komo i loko o ke kino, hopu iā lākou, a luku iā lākou.

Akā i loko o kekahi me CGD, ʻaʻole hiki i kēia mau koa kūikawā, a i ʻole nā ​​keʻokeʻo koko keʻokeʻo, ke hoʻohana pono i kā lākou mau mea kaua. ʻAʻole hiki iā lākou ke hana i ka enzyme kūikawā e pono ai lākou e pepehi i kēlā mau germs. Ua like ia me ka loaʻa ʻana o kahi pū akā ʻaʻohe pōkā. No laila he aha ka mea e hana ai? Ua piha ko mākou ʻōnaehana pale, ʻaʻole hiki ke hakakā i kekahi mau bacteria a me nā fungi e komo i loko o ke kino.

Hoʻopilikia pinepine kēia mau maʻi i ka ʻili, nā māmā, nā lymph node, a me ke ake . ʻOi aku ka maʻalahi o lākou e hana i nā abscesses , he mau puʻupuʻu piha i ka pus i loko o nā ʻōpū o ke kino.

ʻAʻole kēia he maʻi maʻamau loa. Ma kahi o hoʻokahi i loko o ʻelua a me ka hapa miliona mau kānaka ma ka honua holoʻokoʻa e loaʻa ai kēia maʻi. Eia kekahi, ua ʻike ʻia he ʻoi aku ka nui o ka loaʻa ʻana o ia maʻi i nā keikikāne .

He aha nā hōʻailona e hōʻike ai kekahi me CGD?

Hoʻomaka pinepine nā hōʻailona o ka CGD i ka wā kamaliʻi. Eia nō naʻe, hiki iā lākou ke ʻike ʻia i kekahi manawa i kēlā me kēia makahiki. ʻO ka hōʻailona nui a maʻamau ka hoʻi hou ʻana o nā maʻi bacteria a me nā maʻi fungal.

E nānā kākou i kēia mau hōʻailona i maʻalahi ke hoʻomaopopo ʻana i ke ʻano o lākou.

No ke aha e hana ʻia ai kēia CGD? He aha ke kumu?

I ka ʻōlelo maʻalahi, he maʻi genetic ka CGD.ʻO ia hoʻi, he mea kēia a kākou e hoʻoilina ai mai ko kākou mau mākua mai. Hoʻomalu ʻia kēlā me kēia hana i loko o ko kākou kino e nā mea i kapa ʻia he mau genes. Aia i loko o kēlā mau hunaola koko keʻokeʻo a kākou i kamaʻilio ai ma mua nā 'kuhikuhi' e hana ai i nā enzymes e pono ai e pepehi i nā germs.

I loko o CGD, aia kahi mutation i loko o kekahi o nā genes ʻelima i loaʻa kēia mau kuhikuhi. Ma muli o kēia mutation, ʻaʻole hiki i nā hunaola koko keʻokeʻo ke hana i ka enzyme, a inā hana lākou, ʻaʻole ia e hana pono. ʻO ka hopena, ʻaʻole hiki iā lākou ke hakakā i nā germs.

Ua māhele ʻia ʻo CGD i ʻelua ʻano ma muli o ke ʻano o ka hanana ʻana o kēia kīnā genetic.

• ʻO CGD i hoʻopili ʻia me X: ʻO kēia ke ʻano maʻamau. Hoʻokumu ʻia ia e kahi mutation i loko o kahi gene (ka gene `CYBB`) ma ka chromosome X. Hoʻokahi chromosome X ko nā kāne, a ʻelua mau chromosome X ko nā wahine. ʻO ia ke kumu i ʻoi aku ka maʻamau o ka maʻi i nā keikikāne .
• ʻO ka CGD recessive autosomal: Hoʻokumu ʻia kēia e nā mutations i nā genes ʻē aʻe (`CYBA`, `NCF1`, `NCF2`, `CYBC1` a i ʻole `NCF4`). Hiki i kēia ʻano ke hoʻopilikia like i nā kāne a me nā wahine .

He aha nā pilikia a me nā pilikia e hiki ke kū mai ma muli o kēia maʻi?

ʻO ke kanaka me CGD ka mea i pilikia nui inā loaʻa i kekahi o ko lākou ʻohana ka maʻi. Eia nō naʻe, he mea kākaʻikahi loa, ua hōʻike ʻia nā hihia o ka maʻi e kū koke ana, me ka ʻole o ka mōʻaukala ʻohana.

Aia kekahi mau pilikia e hiki ke hana ma muli o CGD.

• Nā pilikia hoʻoheheʻe: Hiki i nā ʻōpū a me ka mumū i loko o nā ʻōpū ke hoʻopilikia i ke kaʻina hana hoʻoheheʻe.
• Nā maʻi ʻōpū: No ka laʻana, aia ka nui o ka pilikia o ka hoʻomohala ʻana i nā kūlana e like me ka Inflammatory Bowel Disease (IBD), kahi mumū mau loa o nā ʻōpū.
• Nā pilikia ulu: Inā loaʻa kēia maʻi i nā ʻōpio a me nā pēpē, e lohi paha ko lākou ulu kino.
• Nā maʻi ʻē aʻe: Ma muli o ke ʻano o ke kīnā genetic e hoʻoulu ai i ka CGD, aia kekahi poʻe i ka pilikia o ka hoʻomohala ʻana i nā maʻi puʻuwai, nā maʻi puʻupaʻa, ka maʻi diabetes, a i ʻole nā ​​​​kūlana autoimmune ʻē aʻe.

Pehea ʻoe e ʻike ai inā loaʻa iā ʻoe ka CGD?

E nīnau mua kāu kauka iā ʻoe a me kāu keiki e pili ana i kāu mau hōʻailona a me ka mōʻaukala olakino ʻohana. A laila, e kauoha lākou i kekahi mau hoʻokolohua e hōʻoia i ka hōʻailona.

• Hoʻokolohua kino: Nānā pono ke kauka i ke kino no ka pehu ʻana, nā abscesses, a me nā granulomas.
• Nā hoʻāʻo koko: Aia kekahi hoʻāʻo koko kūikawā e hiki ke ʻike pololei i ka CGD. Ua kapa ʻia kēia ʻo ka hoʻāʻo DHR (hoʻāʻo dihydrorhodamine) . Ke ana kēia hoʻāʻo i ka hiki o nā hunaola koko keʻokeʻo ke pepehi i nā germs.
• Hoʻāʻo hoʻoilina:Lawe ʻia kahi hāpana koko a i ʻole kahi hāpana ʻiʻo e nānā i ke kīnā genetic kikoʻī e hoʻoulu ai i ka CGD. Hiki i kēia ke kōkua i ka hoʻoholo ʻana i ke ʻano kikoʻī o ka maʻi.

He aha nā lāʻau lapaʻau no CGD?

ʻOiai ʻaʻole hiki ke ho'ōla loa ʻia ka CGD, aia i kēia manawa he nui nā lāʻau lapaʻau maikaʻi e hiki ke kōkua i ka kaohi ʻana i nā hōʻailona, ​​​​pale i nā maʻi koʻikoʻi, a kōkua i nā kānaka e ola i ke ola maʻamau.

Lapaʻau me ka lāʻau lapaʻau

• Nā lāʻau antibiotic: Pono ka nui o nā poʻe maʻi CGD e lawe i nā lāʻau antibiotic no ke koena o ko lākou ola e pale aku i nā maʻi bacteria a mālama i nā maʻi e ulu ana.
• Nā lāʻau lapaʻau antifungal: Hoʻohana ʻia nā lāʻau lapaʻau e like me ka `(itraconazole)` e pale a mālama i nā maʻi fungal.
• Nā inikini Interferon-gamma: He ʻano synthetic kēia o kahi protein i hana ʻia e kā mākou ʻōnaehana pale ponoʻī. Kōkua kēia inikini i ka hōʻemi ʻana i ke alapine (frequency) a me ke koʻikoʻi o nā maʻi.

Hiki i kekahi mau maʻi, ʻoiai ka poʻe me nā maʻi koʻikoʻi, ke hana i kahi hoʻoili cell kumu . ʻO kēia ka hoʻokomo ʻana i nā cell kumu mai kahi kanaka olakino i loko o ke kino o ka mea maʻi, kahi e pani ai i nā cell koko keʻokeʻo hana ʻole me nā cell olakino. Eia nō naʻe, he ʻano paʻakikī a pilikia kēia lāʻau lapaʻau, no laila ke paipai nei nā kauka iā ia ma muli o nā kumu he nui, e like me ka makahiki a me ke olakino o ka mea maʻi.

He aha kā kākou e hana ai e pale aku iā kākou iho mai nā maʻi?

• E makaʻala me ka wai: E pale i ka ʻauʻau ʻana i nā muliwai, nā moana, a me nā loko. ʻAʻole kēia mau mea i hoʻohui ʻia i ka chlorine, no laila hiki i nā germs e hoʻoulu ai i nā maʻi ke komo maʻalahi i kou kino. No laila, inā ʻoe e ʻauʻau, e hoʻohana wale i kahi loko wai i hoʻohui ʻia i ka chlorine .
• E mālama i kāu māla: Mai hoʻopā a kau paha a puni nā ʻōpala māla, nā puʻu compost, nā puʻu mauʻu maloʻo, a me nā lau maloʻo. Hiki i kekahi o nā fungi i loaʻa i loko o kēia mau mea ke hana i ka maʻi pneumonia koʻikoʻi e hiki ke hoʻoweliweli i ke ola inā hanu ʻia e ka mea maʻi CGD. No laila, pono ʻoe e makaʻala loa i kēia.

Nā mea e pono ai ʻoe e ʻike e pili ana i ke ola ʻana me kēia maʻi

Hiki ke hopohopo ka lohe ʻana e pili ana i kēia maʻi. Akā ʻo ka ʻoiaʻiʻo, me ka mālama pono ʻana a me ka launa pū ʻana me kāu kauka, hiki i ka hapa nui o ka poʻe me CGD ke ola maʻamau, ʻeleu, a hauʻoli hoʻi. ʻO ka mea nui loa, ʻo ia ka ʻike ʻana i kahi maʻi a hoʻomaka i ka mālama ʻana i ka wā mua, ma mua o ka lilo ʻana i mea ʻino.

Inā ʻoe a i ʻole kāu keiki e ʻeha nei i nā maʻi pinepine i wehewehe ʻole ʻia, e ʻike koke i kāu kauka a kamaʻilio e pili ana i kēia.

Inā loaʻa i kāu keiki ka CGD, e hoʻomaopopo ʻaʻole na ʻoe ia hewa. He kūlana hoʻoilina ia. Me ke aʻo lapaʻau kūpono, loaʻa i kāu keiki kahi manawa kūpono e ola ai i kahi ola lōʻihi a hauʻoli. ʻO ka mea nui, ʻo ia ka ʻike pono e pili ana i ka maʻi a hahai pono i nā ʻōlelo a kāu kauka.