Ka maʻi ʻo Wilson

Anter

• ʻO nā hōʻailona a me nā hōʻailona o ko Wilson maʻi
• Pili pili i ke ake
• Neurological
• Kāʻei ʻo Kayser-Fleischer a me ka cataract sunflower
• Nā ʻōuli ʻē aʻe
• He aha ke kumu a ʻo wai ka makaʻu no ka maʻi o Wilson?
• Pehea i ʻike ʻia ai ko Wilson maʻi?
• Hōʻike kino
• Nā hōʻike Lab
• Pehea e mālama ai i ka maʻi o Wilson?
• Anuu mua
• Pae ʻelua
• Pae ʻekolu
• He aha ka manaʻo no ka maʻi o Wilson?
• Hiki iā ʻoe ke pale aku i ka maʻi o Wilson?
• Nā ʻanuʻu aʻe

He aha ka maʻi o Wilson?

ʻO ka maʻi o Wilson, ʻo ia hoʻi ʻo ka hōʻino ʻia o ka hepatolenticular a me ka hoʻoneʻe ʻana o ka lenticular holomua, kahi maʻi maʻi ʻē aʻe i kumu ʻia ka lāʻau make keleawe i ke kino. Hoʻopili ia ma kahi o 1 i 30,000 poʻe ma ka honua holoʻokoʻa.

I ke kino olakino, kānana ke akepaʻa i ke keleawe he nui a hoʻokuʻu iā ia ma o ka mimi. Me ka maʻi o Wilson, ʻaʻole hiki i ke ake ke wehe pono i ke keleawe keu. Kūkulu ke keleawe keu i nā meaola e like me ka lolo, ke ake, a me nā maka.

He mea koʻikoʻi ka ʻike kakahiaka nui no ka hoʻōki ʻana i ka holomua o ka maʻi o Wilson. Hoʻopili paha ka mālama ʻana i ka lawe ʻana i ka lāʻau a i ʻole ke kiʻi ʻana i kahi transplant ake. ʻO ka hoʻolohi a loaʻa ʻole paha ka lāʻau e hiki ai ke hōʻeha i ka ate, hōʻino ka lolo, a i ʻole nā ​​ʻano hoʻoweliweli ʻē aʻe.

E kamaʻilio me kāu kauka inā loaʻa i kou ʻohana ka moʻolelo o ka maʻi o Wilson. Noho nā kānaka he nui me kēia ʻano i ke ola maʻamau.

ʻO nā hōʻailona a me nā hōʻailona o ko Wilson maʻi

Hoʻololi ka nui o nā ʻōuli a me nā ʻōuli o ko Wilson maʻi, kaukaʻi ʻia i ka ʻōpala hea ka hopena. Hiki ke kuhihewa lākou no nā maʻi ʻē aʻe a i ʻole nā ​​kūlana. Hiki ke ʻike wale ʻia ka maʻi o Wilson e ke kauka a ma o ka hoʻokolohua diagnostical.

Pili pili i ke ake

Hōʻike nā ʻōuli aʻe i ka hōʻiliʻili keleawe i ke ake:

• nāwaliwali
• luhi nei
• Kaumaha emi
• nausea
• ka luaʻi
• nalo ʻai ʻole
• ʻūlili
• jaundice, a melemele paha o ka ʻili
• edema, a i ʻole ka ʻōhū o nā wāwae a me ka ʻōpū
• ʻeha a ʻōpū paha i loko o ka ʻōpū
• punawelewele angiomas, a i ʻole nā ​​lālā koko e ʻike ʻia ma ka ʻili
• ʻūpē mākala

ʻO ka hapa nui o kēia mau ʻōuli, e like me ka jaundice a me ka edema, like ia no nā kūlana ʻē aʻe e like me ka hake a me ke kōpaʻa ʻole. E alakaʻi ana kāu kauka i nā hoʻokolohua he nui ma mua o ka hōʻoia ʻana i kahi kuhi o ka maʻi o Wilson.

Neurological

Hiki ke hōʻiliʻili keleawe i ka lolo i nā ʻōuli e like me:

• hoʻomanaʻo, haʻiʻōlelo, a i ʻole hoʻopilikia ʻike
• hele wāwae maʻamau
• neʻe hele
• paheʻe
• ʻōlohelohe
• clumsiness me nā lima
• loli ke ʻano
• loli i ke ʻano
• kaumaha
• nā pilikia i ke kula

I nā pae holomua, hiki i kēia mau hōʻailona ke hoʻopili i nā spasms muscle, ka hopu ʻana, a me ka ʻehaʻeha i ka neʻe ʻana.

Kāʻei ʻo Kayser-Fleischer a me ka cataract sunflower

E nānā pū kāu kauka no nā apo ʻo Kayser-Fleischer (K-F) a me ka cataract sunflower i loko o nā maka. ʻO nā apo K-F he discolorations gula-ʻeleʻele maʻamau i nā maka i hopena ʻia e nā waihona o ke keleawe keu. Hōʻike nā apo K-F ma kahi o 97 pakeneka o ka poʻe me ka maʻi ʻo Wilson.

Hōʻike nā cataract Sunflower ma 1 o 5 mau kānaka me ka maʻi o Wilson. He kikowaena multicolored like ʻole kēia me nā haʻi ʻōlelo e hoʻomālamalama i waho.

Nā ʻōuli ʻē aʻe

ʻO ke kūkulu ʻana o ke keleawe i nā meaola ʻē aʻe ke kumu:

• discoloration bluish i nā kui
• pōhaku kīhāpai
• ʻosteoporosis ma mua, a i ʻole ka nele o ka iwi i ka nui
• ʻōiwi
• nā hana kūpono o ka menstrual
• haʻahaʻa haʻahaʻa haʻahaʻa

He aha ke kumu a ʻo wai ka makaʻu no ka maʻi o Wilson?

He hoʻololi i loko o ka ATP7B gen, nā code no ka lawe keleawe, ke kumu o ka maʻi o Wilson. Pono ʻoe e hoʻoilina i ka ʻāpana mai nā mākua ʻelua i mea e loaʻa ai ka maʻi ʻo Wilson. Hiki i kēia ke ʻano o kekahi o kou mau mākua ke ʻano a lawe paha i ka ʻāpana.

Hiki i ka moʻokūʻauhau ke lele i kahi hanauna, no laila makemake ʻoe e nānā ma mua o kou mau mākua a i ʻole lawe i kahi hoʻokolohua kūlohelohe.

Pehea i ʻike ʻia ai ko Wilson maʻi?

He paʻakikī paha ka maʻi o Wilson no nā kauka e hōʻike mua. Ua like nā ʻōuli me nā pilikia olakino ʻē aʻe e like me ka make ʻana o nā metala kaumaha, ka maʻi hepa C, a me ka palsy cerebral.

I kekahi manawa e hiki i kāu kauka ke kāpae i ka maʻi o Wilson ke loaʻa nā hōʻailona neurological a ʻaʻohe ke kani K-F e ʻike ʻia.Akā ʻaʻole kēia ka hihia mau no ka poʻe me nā ʻōuli kikoʻī a ʻaʻohe hōʻailona ʻē aʻe.

E nīnau kahi kauka e pili ana i kāu mau hōʻailona a noi i ka mōʻaukala o kou ʻohana. E hoʻohana pū lākou i nā ʻano hoʻokolohua like ʻole e nānā no ka hōʻino ʻia e nā hōʻiliʻili keleawe.

Hōʻike kino

I kou kino, kāu kauka e:

• e nānā i kou kino
• hoʻolohe i nā kani i loko o ka ʻōpū
• e nānā i kou mau maka ma lalo o ke kukui ʻōlinolino no nā apo K-F a i ʻole nā ​​cataract sunflower
• hōʻoia i kāu kaʻa a me kou mākau hoʻomanaʻo

Nā hōʻike Lab

No nā hoʻokolohua koko, e kahakiʻi kāu kauka i nā laʻana a kālailai ʻia lākou ma kahi keʻena e nānā ai:

• nā mea kūpono ʻole i loko o kou enzymes ate
• pae pae keleawe i ke koko
• nā pae haʻahaʻa o ceruloplasmin, kahi protein e lawe i ke keleawe ma o ke koko
• he gen mutated, i kapa ʻia hoʻi ka hoʻāʻo genetic
• kō kō kō haʻahaʻa

E noi paha kāu kauka iā ʻoe e hōʻiliʻili i kou mimi no 24 mau hola e nānā ai i ka hōʻiliʻili keleawe.

Pehea e mālama ai i ka maʻi o Wilson?

ʻO ka mālama kūleʻa ʻana i ka maʻi o Wilson kaukaʻi ʻia i ka manawa ma mua o ka lāʻau. Hana pinepine ka hoʻomaʻamaʻa i ʻekolu mau hanana a pono e mau i ke ola holoʻokoʻa. Inā kū kekahi kanaka i ka lawe ʻana i nā lāʻau, hiki i ke keleawe ke kūkulu hou i luna.

Anuu mua

ʻO ka hana mua e wehe i ka nui o ke keleawe mai kou kino ma o chelating therapy. ʻO nā mea lawelawe Chelating me nā lāʻau e like me d-penicillamine a me trientine, a i ʻole Syprine. E hoʻopau kēia mau lāʻau i ke keleawe keu mai kāu mau lālā a hoʻokuʻu i loko o ke kahe o ke koko. A laila e kānana kāu mau puʻupaʻa i ke keleawe i ka mimi.

Ua hōʻike ʻo Trientine i nā hopena i hōʻike ʻia ma mua o d-penicillamine. Loaʻa nā hopena ʻaoʻao d-penicillamine:

• fiva
• hūwī
• nā pilikia o ka hakuʻala
• pilikia pili iwi

E hāʻawi kāu kauka i nā lāʻau haʻahaʻa o nā lāʻau chelating inā hāpai ʻoe, no ka mea hiki iā lākou ke hōʻeha i nā hānau hānau.

Pae ʻelua

ʻO ka pahuhopu o ka pae ʻelua ka mālama ʻana i nā pae maʻamau o ke keleawe ma hope o ka hemo ʻana. E kau kauka i kāu zinc a i ʻole tetrathiomolybdate inā ʻoe i hoʻopau i ka lapaʻau mua a hōʻike ʻole i nā hōʻailona akā loaʻa ka maʻi ʻo Wilson.

Lawe waha ʻia ʻo Zinc ma ke ʻano he paʻakai a acetate paha (Galzin) mālama i ke kino mai ka lawe ʻana i ke keleawe mai nā meaʻai. Hiki paha iā ʻoe ke huhū i kou ʻōpū mai ka lawe ʻana i ka zinc. ʻO nā keiki me ka maʻi o Wilson akā ʻaʻohe mau ʻōuli e makemake e lawe i ka zinc e pale ai i ke kūlana mai ka hōʻemi a hoʻolōʻihi i kāna holomua.

Pae ʻekolu

Ma hope o ka hoʻomaikaʻi ʻana o nā ʻōuli a he maʻamau nā pae keleawe, makemake ʻoe e kau i ka mālama mālama wā lōʻihi. Hoʻopili kēia i ka hoʻomau zinc a i ʻole chelating therapy a me ka nānā mau ʻana i kāu pae keleawe.

Hiki iā ʻoe ke mālama i kāu pae keleawe ma ka hōʻalo ʻana i nā meaʻai me nā pae kiʻekiʻe, e like me:

• hua maloʻo
• ake
• ʻulā
• hua kukui
• pūpū
• kokoleka
• multivitamins

Makemake paha ʻoe e nānā i kāu pae wai ma ka home. Aia paha he keleawe keu i loko o kāu wai inā he paipu keleawe kou home.

Hiki i nā lāʻau ke lawe i kēlā me kēia wahi mai ʻehā a i ʻeono mau mahina e hana i kahi kanaka e loaʻa nei nā ʻōuli. Inā pane ʻole kahi kanaka i kēia mau ʻano hana, koi paha lākou i kahi transplant ake. Hiki i kahi transplant ake kūleʻa ke hoʻōla i ka maʻi o Wilson. ʻO ka helu kūleʻa o nā hoʻololi o ke ake he 85 pākēneka ma hope o hoʻokahi makahiki.

He aha ka manaʻo no ka maʻi o Wilson?

ʻO ka mea ma mua e ʻike inā loaʻa iā ʻoe ka ʻohana no ka maʻi o Wilson, ʻo ka ʻoi aku ka maikaʻi o kāu wānana. Hiki i ka maʻi o Wilson ke ulu a lilo i ka hōʻeha o ke ake a me ka hōʻino o ka lolo inā haʻalele ʻole ʻia.

Hiki i ka mālama mua ʻana ke kōkua i ka hoʻohuli i nā pilikia neurological a me ka hōʻino o ke ake. ʻO ka mālama ʻana i kahi ma hope e pale aku paha i ka holomua o ka maʻi, akā ʻaʻole ia e hoʻihoʻi mau i ka pohō. Pono paha ka poʻe i nā pae holomua e aʻo pehea e hoʻokele ai i kā lākou mau hōʻailona ma ke ʻano o ko lākou ola.

Hiki iā ʻoe ke pale aku i ka maʻi o Wilson?

ʻO ka maʻi o Wilson kahi ʻāpana hoʻoilina i hoʻoili ʻia mai nā mākua i kā lākou poʻe keiki. Inā he keiki kā nā mākua me ka maʻi o Wilson, hiki iā lākou ke loaʻa nā keiki ʻē aʻe me ka maʻi pū kekahi.

ʻOiai ʻaʻole hiki iā ʻoe ke pale aku i ka maʻi o Wilson, hiki iā ʻoe ke hoʻolohi a hoʻolohi paha i ka hoʻomaka ʻana o ke ʻano. Inā ʻoe e ʻike he maʻi ʻo Wilson i ka wā ma mua, hiki paha iā ʻoe ke pale i nā hōʻailona mai ka hōʻike ʻana i ka lawe ʻana i nā lāʻau e like me ka zinc. Hiki i kahi loea genetical ke kōkua i nā mākua e hoʻoholo i kā lākou pilikia no ka hele ʻana i ka maʻi ʻo Wilson i kā lākou keiki.

Nā ʻanuʻu aʻe

E hana i kahi manawa me kāu kauka inā ʻo ʻoe a ʻo kekahi mea paha āu e ʻike nei he maʻi ʻo Wilson a ke hōʻike nei paha i nā ʻōuli o ke kūpaʻa ʻole o ke ake. ʻO ka mea hōʻike nui loa no kēia ʻano he moʻolelo ʻohana, akā hiki i ka moʻokūʻauhau ke kāpae i kahi hanauna. E makemake paha ʻoe e noi i kahi hoʻokolohua kūlohelohe me nā hoʻokolohua ʻē aʻe a kāu kauka e hoʻonohonoho ai.

Makemake ʻoe e hoʻomaka koke i kāu lapaʻau inā loaʻa ʻoe i kahi hōʻailona no ka maʻi o Wilson. Hiki ke kōkua i ka mālama mua ʻana i ka pale ʻana a hoʻolohi paha i ke kūlana, keu hoʻi inā ʻaʻole ʻoe e hōʻike nei i nā ʻōuli. Hoʻopili nā lāʻau i nā chelating agents a me ka zinc a hiki i ʻeono mau mahina e hana ai. ʻOiai ma hope o ka hoʻi ʻana o kāu pae keleawe i ka maʻamau, pono ʻoe e hoʻomau i ka lawe ʻana i ka lāʻau, ʻoiai ʻo ka maʻi o Wilson kahi ola holoʻokoʻa.