He aha ka Maʻi Granulomatous Mau Loa? Nā Hōʻailona, Nā Kumu, a me ka Lapaʻau

ʻO ka maʻi granulomatous mau loa (CGD) he maʻi hoʻoilina maʻamau loa ia kahi e hiki ʻole ai i kou ʻōnaehana pale ke pale aku i kekahi mau maʻi. E noʻonoʻo iā ia me he mau kiaʻi palekana lā e hiki ʻole ke hana pono i kā lākou hana - hiki iā lākou ke hopu i kekahi mau mea komo, akā naʻe e haʻalele i nā mea ʻē aʻe, e waiho ana iā ʻoe i ka pilikia nui i kekahi mau ʻano bacteria a me nā fungi.

Hoʻopili kēia maʻi i ke ʻano o ka hana ʻana o kou mau keʻena koko keʻokeʻo, ʻo ia hoʻi nā keʻena i kapa ʻia ʻo phagocytes. Pono kēia mau keʻena e pepehi i nā germs ma o ka hana ʻana i nā kemika ikaika, akā naʻe, i ka CGD, ʻaʻole hana pono kēia kaʻina hana. ʻOiai ke kani nui nei kēia, nui nā kānaka me ka CGD e ola ana i nā ola piha a hana nui me ka mālama lapaʻau kūpono a me ka lapaʻau.

He aha nā hōʻailona o ka Maʻi Granulomatous Mau Loa?

ʻIke pinepine ʻia nā hōʻailona CGD i ka wā kamaliʻi mua, ʻoiai ʻaʻole i ʻike ʻia kekahi poʻe a hiki i ko lākou mau makahiki ʻōpio a i ʻole ka wā makua. ʻO ka hōʻailona nui ka loaʻa ʻana o nā maʻi pinepine a nui hoʻi e paʻakikī ke hoʻōla ma mua o nā maʻi maʻamau o ka wā kamaliʻi.

Eia nā hōʻailona maʻamau loa āu e ʻike ai:

• Nā maʻi māmā hou a i ʻole pneumonia e hoʻi hou mai ana
• Nā maʻi ʻili, nā abscesses, a i ʻole nā ​​ʻeha e hoʻōla mālie ana
• Nā lymph nodes pehu e noho nui ana no nā hebedoma
• Nā maʻi ʻōpū a i ʻole nā ​​pilikia ʻōpū
• Nā abscesses ate a i ʻole nā ​​maʻi
• Nā maʻi iwi e hoʻokumu i ka ʻeha a me ka pehu
• Nā mahana pinepine me ka ʻole o ke kumu maopopo

Hoʻomohala kekahi poʻe i nā granulomas - nā hui liʻiliʻi o nā keʻena pale e hana ʻia ke ho'āʻo ka kino e hakakā i ka maʻi. Hiki i kēia mau mea ke pani i nā ʻōnaehana e like me ka ʻōpū, nā ʻōpū, a i ʻole ka ʻōnaehana mimi, e hoʻokumu ana i nā hōʻailona hou e like me ka pilikia o ka ʻai ʻana a i ʻole ka mimi ʻana.

I nā hihia maʻamau ʻole, hiki i ka CGD ke hoʻokumu i nā pilikia nui aʻe e like me nā abscesses lolo a i ʻole nā ​​maʻi naʻau. ʻOiai ke kani weliweli nei kēia mau mea, he mea maʻamau ʻole lākou a hiki ke mālama ʻia ke hopu ʻia i ka wā mua me ka mālama lapaʻau kūpono.

He aha ka mea e hoʻokumu ai i ka Maʻi Granulomatous Mau Loa?

Hana ʻia ka CGD ma muli o nā loli (mutations) i nā gene e kaohi ana i ke ʻano o ka hana ʻana o kou mau keʻena pale. Pale kēia mau loli genetic i kou mau keʻena koko keʻokeʻo mai ka hana ʻana i kahi enzyme complex i kapa ʻia ʻo NADPH oxidase, kahi mea nui no ka pepehi ʻana i kekahi mau germs.

ʻO ka hapa nui o nā hihia o CGD ua hoʻoilina ʻia, ʻo ia hoʻi, ua hala ka maʻi mai nā mākua i nā keiki. Hoʻopili nui ka ʻano maʻamau i nā keikikāne ma mua o nā kaikamahine no ka mea aia ka gene hewa ma ka chromosome X. Loaʻa i nā keikikāne hoʻokahi chromosome X wale nō, no laila inā lawe ia i ka mutation, e loaʻa iā lākou ka CGD.

Loaʻa i nā kaikamahine ʻelua mau chromosome X, no laila ʻoiai inā lawe kekahi i ka mutation, hāʻawi pinepine ka mea olakino ʻē aʻe i ka palekana lawa. Eia naʻe, hiki i nā kaikamahine ke lilo i nā mea lawe a hiki ke loaʻa iā lākou nā hōʻailona māmā.

Aia nō hoʻi nā ʻano CGD ʻaʻole maʻamau e hiki ke hoʻopili like i nā keikikāne a me nā kaikamahine. Hana ʻia kēia mau mea ke hana nā mutations i nā gene i loaʻa ma nā chromosomes ʻē aʻe. I nā hihia maʻamau loa, hiki i ka CGD ke hoʻomohala me ka ʻole o ka mōʻaukala ʻohana ma muli o nā loli genetic hou.

He aha nā ʻano o ka Maʻi Granulomatous Mau Loa?

Hoʻokaʻawale nā ​​kauka i ka CGD ma muli o ka gene kikoʻī i hoʻopilikia ʻia a pehea ka hoʻoilina ʻana o ka maʻi. Ke hoʻomaopopo ʻoe i kou ʻano, e kōkua i kāu hui lapaʻau e koho i ke ala lapaʻau maikaʻi loa.

ʻO ka ʻano maʻamau loa ka X-linked CGD, kahi e loaʻa ai ma kahi o 65% o nā hihia a pau. Hoʻopili nui kēia ʻano i nā keikikāne a hoʻokumu pinepine i nā hōʻailona nui aʻe. Loaʻa pinepine i nā keikikāne me ka X-linked CGD kā lākou maʻi nui mua ma mua o 2 mau makahiki.

Hoʻokumu ka autosomal recessive CGD i nā hihia i koe a hoʻopili like i nā keikikāne a me nā kaikamahine. Loaʻa pinepine i kēia ʻano nā hōʻailona māmā a ʻaʻole paha e ʻike ʻia a hiki i ka wā ma hope o ka wā kamaliʻi a i ʻole ka wā makua. Hiki i nā kānaka me kēia ʻano ke loaʻa nā maʻi liʻiliʻi a i ʻole nā ​​pilikia liʻiliʻi.

I loko o kēia mau māhele nui, aia kekahi mau ʻano ma muli o ka ʻāpana kikoʻī o ka ʻōnaehana NADPH oxidase ʻaʻole e hana pono ana. Hiki i kāu kauka ke hoʻoholo i kou ʻano kikoʻī ma o ka hoʻokolohua genetic, kahi e kōkua ai e wānana i nā maʻi āu e loaʻa pinepine ai a alakaʻi i nā hoʻolālā pale.

I ka wā hea e ʻike ai i ke kauka no ka Maʻi Granulomatous Mau Loa?

Pono ʻoe e hoʻokaʻaʻike i kāu kauka inā ʻike ʻoe i kahi ʻano o nā maʻi pinepine, nui, a i ʻole maʻamau ʻole. ʻOiai ke maʻi nei kēlā me kēia keiki i kekahi manawa, ʻoi aku ka nui o nā maʻi CGD a ʻoi aku ka paʻakikī ke mālama ma mua o nā maʻi maʻamau o ka wā kamaliʻi.

E ʻimi i ka mālama lapaʻau koke inā loaʻa iā ʻoe ka pneumonia hou, nā abscesses ʻili ʻaʻole e hoʻōla me ka mālama maʻamau, a i ʻole nā ​​mahana mau me ka ʻole o ke kumu maopopo. Hiki i kēia mau mea ke hōʻailona e pono ana kāu ʻōnaehana pale i ke kākoʻo hou.

Inā loaʻa iā ʻoe ka mōʻaukala ʻohana o CGD a i ʻole nā ​​maʻi maʻamau ʻole, e haʻi i kēia i kāu mea mālama ola. ʻO ka ʻike mua a me ka mālama ʻana e hana i kahi ʻokoʻa nui i ka pale ʻana i nā pilikia nui a me ke kōkua ʻana iā ʻoe e noho olakino.

No nā kānaka i ʻike mua ʻia me ka CGD, e kāhea koke i kāu kauka inā loaʻa iā ʻoe nā hōʻailona hou e like me ka ʻeha ʻōpū nui, ka pilikia o ka hanu, ka luaʻi mau, a i ʻole kekahi hōʻailona o ka maʻi. Hiki i ka mālama wikiwiki ke pale i nā pilikia liʻiliʻi mai ka lilo ʻana i nā pilikia nui.

He aha nā mea pilikia no ka Maʻi Granulomatous Mau Loa?

ʻOiai ke kani nui nei nā pilikia CGD, e hoʻomanaʻo e hiki ke pale ʻia a mālama ʻia paha me ka mālama lapaʻau kūpono. ʻO ke kī ka noho ʻana ma luna o kāu hoʻolālā mālama a me ka hana pili me kāu hui mālama ola.

ʻO nā pilikia maʻamau loa e komo pū ana:

• Nā maʻi māmā nui e hiki ke hoʻokumu i ka ʻeha
• Nā abscesses ate e pono paha ke hoʻokahe
• Granulomas e pani ana i ka ʻōpū, nā ʻōpū, a i ʻole ka ʻōnaehana mimi
• Nā maʻi ʻōpū mau a me ka loaʻa ʻole o ke kaupaona
• Nā maʻi iwi a me nā hui
• Nā maʻi ʻili a me nā ʻeha hoʻōla mālie

ʻO nā pilikia liʻiliʻi akā nui aʻe e komo pū ana i nā abscesses lolo, nā maʻi naʻau, a i ʻole ka mumū ʻōpū nui. ʻOiai ke kani weliweli nei kēia mau mea, he mea maʻamau ʻole lākou ke mālama pono ʻia ka CGD me nā lāʻau pale a me ka nānā lapaʻau mau.

Hoʻomohala kekahi poʻe me ka CGD i nā pilikia autoimmune, kahi e hoʻouka ai ka ʻōnaehana pale i nā pūnaewele kino olakino. Hiki paha i kēia ke hoʻokumu i nā kūlana e like me ka inflammatory bowel disease a i ʻole ka maʻi ʻāʻī. E nānā kāu hui lapaʻau i kēia mau pilikia a mālama iā lākou inā hoʻomohala lākou.

Pehea ka ʻike ʻana i ka Maʻi Granulomatous Mau Loa?

Hoʻomaka ka ʻike ʻana i ka CGD me kāu kauka e ʻike ana i kahi ʻano o nā maʻi maʻamau ʻole a pinepine paha. E nīnau lākou i nā nīnau kikoʻī e pili ana i kāu mōʻaukala lapaʻau a me kekahi mōʻaukala ʻohana o nā pilikia pale a i ʻole nā ​​make mua mai nā maʻi.

ʻO ka hoʻokolohua nui no ka CGD ka hoʻokolohua dihydrorhodamine (DHR). Ana kēia hoʻokolohua koko i ke ʻano o ka pepehi ʻana o kou mau keʻena koko keʻokeʻo i nā germs. I nā kānaka me ka CGD, hōʻike kēia hoʻokolohua ʻaʻole hana pono nā keʻena.

Hiki i kāu kauka ke kauoha i ka hoʻokolohua genetic e hōʻoia i ka ʻike a hoʻoholo i ke ʻano kikoʻī o ka CGD āu e loaʻa nei. Hoʻopili kēia i ka nānā ʻana i kāu DNA e ʻike i nā mutations gene kikoʻī e hoʻokumu ana i ka maʻi.

Hiki i nā hoʻokolohua hou ke komo pū me nā moʻomeheu o nā wahi maʻi e ʻike i nā germs kikoʻī e hoʻokumu ana i nā pilikia. E kōkua kēia i kāu hui lapaʻau e koho i nā lāʻau antibiotic a me nā antifungal maikaʻi loa no kāu kūlana.

He aha ka lapaʻau no ka Maʻi Granulomatous Mau Loa?

Hoʻokumu ka lapaʻau CGD i ka pale ʻana i nā maʻi a me ka mālama wikiwiki ʻana iā lākou ke loaʻa. ʻOiai ʻaʻohe lāʻau lapaʻau no ke kūlana genetic kumu, kōkua nā lapaʻau maikaʻi i ka hapa nui o nā kānaka me ka CGD e ola i nā ola maʻamau a olakino.

ʻO ke kumu o ka lapaʻau CGD ka lawe ʻana i nā lāʻau antibiotic pale i kēlā me kēia lā. Lawe ka hapa nui o nā kānaka i ka trimethoprim-sulfamethoxazole (i kapa ʻia hoʻi ʻo Bactrim a i ʻole Septra) e pale aku i nā maʻi bacterial. E lawe paha ʻoe i kahi lāʻau antifungal e like me ka itraconazole e pale aku i nā maʻi fungal.

Loaʻa i nā kānaka he nui nā inikini interferon gamma, i hāʻawi ʻia ma kahi o ʻekolu manawa i ka hebedoma. Kōkua kēia lāʻau e hoʻoikaika i ka hiki o kāu ʻōnaehana pale ke hakakā i nā maʻi. ʻOiai ke kani weliweli nei ka hāʻawi ʻana iā ʻoe iho i nā pana, nui nā kānaka e maʻa koke i ka hana maʻamau.

Ke loaʻa nā maʻi, mālama nui ʻia lākou me nā lāʻau antibiotic ikaika a i ʻole nā ​​lāʻau antifungal. Pono paha ʻoe e noho ma ka haukapila no nā lāʻau intravenous, ʻoiai no nā maʻi nui e like me ka pneumonia a i ʻole nā ​​abscesses ate.

No nā hihia nui, hiki i nā kauka ke ʻōlelo i kahi hoʻololi iwi iwi (i kapa ʻia hoʻi ʻo ka hoʻololi ʻana i nā keʻena stem). Hiki i kēia kaʻina hana ke ho'ōla i ka CGD ma o ka hoʻololi ʻana i kou mau keʻena pale hewa me nā mea olakino mai ka mea hāʻawi. Eia naʻe, lawe nā hoʻololi i nā pilikia nui a ʻaʻole kūpono no kēlā me kēia.

Pehea e mālama ai i ka Maʻi Granulomatous Mau Loa ma ka home?

ʻO ka ola maikaʻi me ka CGD ʻo ia ka lawe ʻana i kahi kuleana hana i ka pale ʻana i nā maʻi ʻoiai e mālama ana i kahi ola maʻamau e like me ka hiki. Hiki i ka hapa nui o nā kānaka me ka CGD ke hele i ke kula, hana, hoʻoikaika kino, a hauʻoli i nā hana maʻamau me kekahi mau palekana maʻalahi.

E lawe i kāu mau lāʻau e like me ka mea i kuhikuhi ʻia, ʻoiai ke olakino maikaʻi nei ʻoe. ʻO ka haʻalele ʻana i nā lāʻau antibiotic a i ʻole antifungal pale e hoʻonui nui i kou pilikia maʻi. E hoʻonohonoho i nā ʻōnaehana e like me nā mea hoʻonohonoho papa a i ʻole nā ​​hoʻomanaʻo kelepona e kōkua iā ʻoe e noho kūpaʻa.

E hoʻomaʻamaʻa i ka hoʻomaʻemaʻe maikaʻi ma o ka holoi pinepine ʻana i kou mau lima a me ka hoʻomaʻemaʻe pono. E pale i nā hana e hiki ke hōʻike iā ʻoe i ka nui o nā bacteria a i ʻole nā ​​fungi, e like me ka mālama māla me ka ʻole o nā mīkina lima, ka hoʻomaʻemaʻe ʻana i nā ʻōpala holoholona, ​​a i ʻole ka ʻauʻau ʻana i nā loko a i ʻole nā ​​pā wai wela.

E mālama i kou wahi maʻemaʻe akā mai lilo i mea obsessive e pili ana iā ia. Laha ka hoʻomaʻemaʻe hale maʻamau - ʻaʻole pono ʻoe e noho i loko o kahi pōpō sterile. E nānā i nā wahi kahi e hōʻiliʻili pinepine ai nā germs, e like me nā lumi ʻauʻau a me nā kīhini.

E noho hou me nā immunizations āpau i ʻōlelo ʻia, akā e pale i nā immunizations ola ke ʻae ʻole kāu kauka iā lākou. Hiki paha i kāu ʻōnaehana pale ke mālama ʻole i nā immunizations ola me ka palekana, no laila e alakaʻi kāu hui lapaʻau iā ʻoe i nā immunizations kūpono.

Pehea ʻoe e hoʻomākaukau ai no kāu hālāwai kauka?

ʻO ka hoʻomākaukau ʻana no kāu mau hālāwai CGD e kōkua i ka hōʻoia ʻana e loaʻa iā ʻoe ka mea maikaʻi loa mai kāu manawa me kāu hui mālama ola. E hele mai e kūkākūkā i nā hōʻailona hou, nā pilikia lāʻau, a i ʻole nā ​​nīnau e pili ana i ka mālama ʻana i kou kūlana.

E mālama i kahi diary hōʻailona e hoʻomaopopo ana i nā mahana, nā maʻi, a i ʻole nā ​​hōʻailona maʻamau ʻole mai kāu kipa hope loa. E komo pū i nā kikoʻī e like me ka manawa i hoʻomaka ai nā hōʻailona, ​​pehea ka lōʻihi o ko lākou mau, a me nā mālama i kōkua. E kōkua kēia ʻike i kāu kauka e ʻike i nā ʻano a hoʻoponopono i kāu mālama inā pono.

E lawe mai i kahi papa inoa piha o nā lāʻau āpau āu e lawe nei, me nā nui kikoʻī a me ka pinepine o kāu lawe ʻana. E haʻi pū i nā mea hoʻohui, nā lāʻau over-the-counter, a i ʻole nā ​​mea kanu āu e hoʻohana nei, ʻoiai hiki i kēia mau mea ke launa pū me kāu mau lāʻau CGD.

E kākau i nā nīnau ma mua o kāu hālāwai i ʻole ʻoe e poina i kekahi mea nui. Hiki i nā nīnau maʻamau ke komo pū i nā hopohopo e pili ana i nā hōʻailona hou, nā hopena mai nā lāʻau, nā palena hana, a i ʻole ka mea e hana ai inā maʻi ʻoe.

Inā ʻike ʻoe i ke kauka hou a i ʻole ka mea kūikawā, e lawe mai i nā kope o nā hopena hoʻokolohua hou, nā moʻolelo haukapila, a me kahi hōʻuluʻulu o kāu mōʻaukala CGD. E kōkua kēia ʻike hope i nā mea hoʻolako hou e hoʻomaopopo i kou kūlana kikoʻī a hana i nā hoʻoholo mālama maikaʻi aʻe.

He aha ka lawe nui e pili ana i ka Maʻi Granulomatous Mau Loa?

ʻO ka CGD he kūlana koʻikoʻi akā hiki ke mālama ʻia e pono ai ka mālama lapaʻau mau a me ka nānā ʻana i ka pale ʻana. ʻOiai hiki i ka ʻike ke kani nui, e hoʻomanaʻo ua holomua nui nā lapaʻau i nā makahiki i hala, a hiki i ka hapa nui o nā kānaka me ka CGD ke ola i nā ola piha a hana nui.

ʻO ka mea nui loa āu e hiki ke hana ai, ʻo ia ka lawe mau ʻana i kāu mau lāʻau pale a me ka hana pili me kāu hui mālama ola. Kōkua kēia ala pili i ka hopu ʻana i nā pilikia i ka wā mua a mālama iā ʻoe i ka olakino e like me ka hiki.

Mai ʻae i ka CGD e hoʻomaopopo a i ʻole e kaupalena iā ʻoe me ka pono ʻole. Me nā palekana kūpono a me ka mālama lapaʻau, hiki iā ʻoe ke alualu i ka hoʻonaʻauao, nā pahuhopu ʻoihana, nā pilina, a me nā leʻaleʻa e like me nā poʻe ʻē aʻe. ʻO ke kī ka loaʻa ʻana o ke kaulike kūpono ma waena o ka makaʻu kūpono a me ka ola piha.

E noho pili me nā hui kākoʻo a i ʻole nā ​​kaiaulu pūnaewele no nā kānaka me ka CGD a me ko lākou mau ʻohana. ʻO ka kaʻana like ʻana i nā ʻike me nā poʻe ʻē aʻe e hoʻomaopopo i kou mau pilikia hiki ke hāʻawi i nā ʻōlelo aʻo maikaʻi a me ke kākoʻo manaʻo e hana i kahi ʻokoʻa maoli i kou ola i kēlā me kēia lā.

Nā nīnau pinepine e pili ana i ka Maʻi Granulomatous Mau Loa

Hiki i nā kānaka me ka CGD ke ola i nā ola lōʻihi maʻamau?

ʻAe, hiki i nā kānaka he nui me ka CGD ke ola i nā ola lōʻihi maʻamau a kokoke paha i ka mālama lapaʻau kūpono. Ua hoʻomaikaʻi nui ka ʻike mua a me ka mālama mau ʻana me nā lāʻau pale i nā hopena. ʻOiai pono ka CGD i ka mālama lapaʻau mau, hiki i ka hapa nui o nā kānaka ke alualu i ka hoʻonaʻauao, nā ʻoihana, nā pilina, a me ka ola ʻohana me ka holomua.

Ua hoʻopilikia ʻia anei ka CGD?

ʻAʻole, ʻaʻole hoʻopilikia ʻia ka CGD iho. He maʻi genetic ia āu i hānau ʻia ai, ʻaʻole ia he mea āu e hiki ke hopu ai mai nā poʻe ʻē aʻe a hāʻawi aku paha i nā poʻe ʻē aʻe. Eia naʻe, ʻoi aku ka maʻalahi o nā kānaka me ka CGD i kekahi mau maʻi, no laila pono lākou e makaʻala i nā poʻe ʻē aʻe e maʻi ana e pale aku iā lākou iho.

Hiki i nā wāhine me ka CGD ke loaʻa nā keiki?

ʻAe, hiki i nā wāhine me ka CGD ke loaʻa pinepine nā keiki, ʻoiai pono ka hāpai keiki i ka nānā lapaʻau akahele. ʻO nā mea nui e noʻonoʻo ʻia, ʻo ia ka mālama ʻana i nā lāʻau i ka wā hāpai a me ka hoʻomaopopo ʻana i nā pilikia genetic no ke keiki. Ke kōkua ka ʻōlelo aʻo genetic ma mua o ka hāpai ʻana i nā ʻohana e hoʻomaopopo i nā ʻano hoʻoilina a hana i nā hoʻoholo ʻike.

He aha nā maʻi āu e loaʻa pinepine ai nā kānaka me ka CGD?

ʻOi aku ka nāwaliwali o nā kānaka me ka CGD i nā maʻi mai nā bacteria e like me Staphylococcus, Serratia, a me Burkholderia, a me nā fungi e like me Aspergillus a me Candida. Loaʻa i kēia mau mea ola nā ʻano kūikawā e hana ai iā lākou i ka paʻakikī no nā ʻōnaehana pale CGD e hakakā. Eia naʻe, hoʻemi nui nā lāʻau pale i ka pilikia o kēia mau maʻi.

Hiki ke ho'ōla ʻia ka CGD?

I kēia manawa, ʻo ka hoʻololi ʻana i ka iwi iwi wale nō ka ho'ōla hiki ke loaʻa no ka CGD, akā ʻaʻole kūpono ia no kēlā me kēia ma muli o nā pilikia e komo pū ana. Mālama ka hapa nui o nā kānaka i ka CGD me ka holomua me nā lāʻau pale a me ka nānā akahele. Hōʻike ka noiʻi gene therapy i ka manaʻolana no ka wā e hiki mai ana, akā naʻe, he mea hoʻokolohua kēia mau lapaʻau a ʻaʻole i loaʻa nui i kēia manawa.