He aha ka epidermolysis bullosa, nā ʻōuli a me nā lāʻau

Anter

• Nā hōʻailona nui
• Kumu o ka bullous epidermiolysis
• He aha nā ʻano
• Pehea e hana ai i ka lāʻau
• Ke pono ka ʻoki kino
• He aha e hana ai e pale ai i ke ʻano o nā huʻa
• Pehea e hana ai i ka lole
• He aha nā pilikia

ʻO Bullous epidermolysis kahi maʻi maʻi o ka ʻili e hoʻokumu i nā palapū ma ka ʻili a me nā membrane mucous, ma hope o nā hakakā a i ʻole trauma ʻeha e hiki ke kumu ʻia e ka ukiuki o ka lepili kapa ma ka ʻili a i ʻole, ma ka hemo ʻana i kōkua band, o kahi laʻana. Hana ʻia kēia ʻano ma muli o nā hoʻololi genetic i lawe ʻia mai nā mākua i kā lākou poʻe keiki, kahi e alakaʻi ai i nā loli i nā papa a me nā mea i loaʻa i ka ʻili, e like me keratin.

Pili nā ʻōuli a me nā ʻōuli o kēia maʻi i ke ʻano o ka ʻehaʻeha ma ka ʻili a ma nā ʻāpana o ke kino, a e ʻike ʻia paha ma ka waha, nā poho lima a me nā kapuwaʻi o nā wāwae. Kūlike kēia mau ʻōuli e like me ke ʻano a me ke koʻikoʻi o ka epidermolysis bullosa, akā ʻoi aku ka maikaʻi o ka hala ʻana o ka manawa.

ʻO ka mālama ʻana i ka epidermolysis bullous ka mea nui o ka mālama kōkua, e like me ka mālama ʻana i ka meaʻai kūpono a me ka ʻaʻahu ʻana i nā ʻili o ka ʻili. Hoʻohui ʻia, ke mālama ʻia nei nā noiʻi e hana i ka hoʻoneʻe iwi iwi no ka poʻe me kēia ʻano.

Nā hōʻailona nui

ʻO nā ʻōuli nui o ka bullous epidermolysis:

• Ka nahu ʻana o ka ʻili i ka wili liʻiliʻi;
• ʻIke ʻia nā pupuhi i loko o ka waha a i loko o nā maka;
• Ka hoʻōla ʻana o ka ʻili me ke ʻano ʻino a me nā kiko keʻokeʻo;
• Pākuʻi kui;
• Ka lahilahi lauoho;
• Ka hoʻoliʻiliʻi o ka hou a i ʻole ka nui o ka hou.

Kaukaʻi ʻia i ka paʻakikī o ka bullous epidermolysis, scarring o nā manamana a me nā manamana wāwae e hiki ke hana, hiki i nā deformities. ʻOiai ke ʻano o nā ʻōuli o ka epidermolysis, hiki i nā maʻi ʻē aʻe ke alakaʻi i ka helehelena o nā palapū ma ka ʻili, e like me herpes simplex, epidermolytic ichthyosis, bullous impetigo a me pigmentary incontinence. E ʻike i ka imploigo bullous a he aha ka lāʻau.

Kumu o ka bullous epidermiolysis

Hoʻokumu ʻia ka epidermolysis Bullous e nā mutation genetical i hoʻoili ʻia mai ka makua a ke keiki, a he mana nui paha, kahi o nā mākua i loaʻa ka maʻi maʻi, a i ʻole recessive, kahi e hāpai ai ka makuakāne a me ka makuahine i ka ʻāpana maʻi akā ʻaʻohe hōʻike o nā ʻōuli a me nā ʻōuli o ka maʻi.

ʻO nā keiki i loaʻa nā ʻohana pili loa me ka maʻi a i ʻole me ka gen bullous epidermolysis e hānau ʻia me kēia ʻano o ke ʻano, no laila inā ʻike ka mau mākua ua loaʻa lākou i ka ʻāpana maʻi ma o ka hoʻāʻo ʻana i ka genetic. E ʻike he aha nā ʻōlelo aʻoaʻo kūpuna a pehea e hana ai.

He aha nā ʻano

Hiki ke hoʻokaʻawale ʻia ka epidermolysis Bullous i ʻekolu mau ʻano e pili ana i ka papa o ka ʻili e hana ai i nā palapū, e like me:

• Epidermolisis bullous maʻalahi: hū ka maʻi i ka papa o luna o ka ʻili, i kapa ʻia ʻo epidermis, a he mea maʻamau ia no lākou e hōʻike ai ma nā lima a me nā wāwae. I kēia ʻano hiki ke ʻike i nā kui kuihi a mānoanoa a ʻaʻole hoʻōla koke nā ʻōpala;

• Dystrophic epidermolysis bullosa: kū nā pehu i kēia ʻano ma muli o nā hemahema i ka hana ʻana o ke ʻano V | I collagen a kū i ka papa superficial o ka ʻili, i ʻike ʻia ʻo dermis;
• Jointal epidermolysis bullosa: hōʻike ʻia e ke hoʻokumu ʻia ʻana o nā palapū ma muli o ke kaʻawale ʻana o ka ʻāina ma waena o ka papa superficial a waena o ka ʻili a ma kēia hihia, kū ka maʻi i nā hoʻololi ʻana i nā genes e pili ana i nā dermis a me nā epidermis, e like me Laminin 332.

ʻO Kindler's Syndrome kekahi ʻano o ka bullous epidermolysis, akā he kakaikahi loa ia a pili i nā papa āpau o ka ʻili, a hiki i ka nāwaliwali loa. Nānā ʻole i ke ʻano o kēia maʻi, he mea nui e hoʻomaopopo he maʻi ʻole ka bullous epidermolysis, ʻo ia hoʻi, ʻaʻole ia e hele aʻe mai kekahi kanaka a i kekahi ʻē aʻe ma o ka pili ʻana i nā ʻili o ka ʻili.

Pehea e hana ai i ka lāʻau

ʻAʻohe mālama kūikawā no ka epidermolysis bullosa, a he mea nui loa ke kūkākūkā mau ʻana me ka dermatologist e nānā i ke ʻano o ka ʻili a pale i nā pilikia, e like me nā maʻi, no ka laʻana.

ʻO ka mālama ʻana no kēia maʻi me nā hana kākoʻo, e like me ka ʻaukā ʻana i nā ʻeha a me ka kaohi ʻana i ka ʻeha a, i kekahi mau manawa, pono ka haukapila e hana i nā lole sterile, me ka ʻole o nā microorganism, no laila lawelawe pololei ʻia nā lāʻau i loko o ke aa, e like me nā lāʻau ʻino i hihia o ka maʻi, a e hoʻokahe i nā palapū ma ka ʻili. Eia nō naʻe, ke hoʻomohala ʻia nei kekahi mau noiʻi e hoʻokō i nā transplants cell stem i ka mālama ʻana i ka epidtrolysis dystrophic bullous.

ʻAʻole like me nā palapū i hana ʻia e nā wela, pono e hoʻopili ʻia nā pūhū e ka epidermolysis bullosa me kahi nila kuʻikuʻi, e hoʻohana ana i nā compresses sterile, e pale aku ai i ka hoʻolaha ʻana a hōʻeha hou i ka ʻili. Ma hope o ka hoʻokahe ʻana, he mea nui e noi i kahi huahana, e like me ʻehu antibacterial, e pale aku i nā maʻi.

Ke pono ka ʻoki kino

Hōʻike pinepine ʻia ke kahakaha dermatitis bullous no ka hihia kahi i waiho ʻia nā scars e nā huʻupuʻu e ālai ai i ka neʻe ʻana o ke kino a i ʻole e hana i nā deformities e hōʻemi i ka maikaʻi o ke ola. I kekahi mau hihia, hiki ke hoʻohana ʻia ke ʻoki e hana i nā ʻili ʻili, keu hoʻi i nā ʻeha e hoʻolōʻihi ana i ka manawa e hoʻōla ai.

He aha e hana ai e pale ai i ke ʻano o nā huʻa

ʻOiai ʻaʻohe mea ola, mālama ʻia ka lāʻau lapaʻau e hōʻoluʻolu wale i nā ʻōuli a hoʻēmi i ka manawa kūpono o nā palapū hou. ʻO ka hana mua e mālama i kahi mālama ma ka home, e like me:

• E komo i ka lole pulupulu, e pale ana i nā lole synthetic;
• Wehe i nā hōʻailona mai nā lole āpau;
• Ua hoʻohuli i lalo i nā lole komo i lalo e pale i ka hoʻopili ʻana o ka lahilahi me ka ʻili;
• E komo i nā kāmaʻa māmā a ākea ākea e hōʻoluʻolu ai e komo i nā kākini humuhumu ʻole;
• E akahele loa i ka hoʻohana ʻana i nā kāwele ma hope o ka ʻauʻau ʻana, kaomi mālie ʻana i ka ʻili me kahi kāwele palupalu;
• E noi iā Vaseline i ka nui ma mua o ka hemo ʻana i nā lole a mai hoʻoikaika i ka hemo ʻana.
• Inā pili ka lole i ka ʻili, waiho i ka ʻāina i pulu i ka wai a hiki i ka wā e hemo ai nā lole mai ka ʻili wale nō;
• E uhi i nā ʻeha me kahi lole lina ʻole a me ka lole wili wili i hoʻokuʻu ʻia;
• E hiamoe me nā kākini a me nā mīkina lima e pale i nā hōʻeha e hiki ke hana ʻia i ka wā hiamoe.

Hoʻohui ʻia, inā he ʻili ʻili, hiki i ke kauka ke kuhikuhi i ka hoʻohana ʻana o corticosteroids, e like me prednisone a i ʻole hydrocortisone, e hōʻoluʻolu i ka hoʻā o ka ʻili a hoʻēmi i nā hōʻailona, ​​e hōʻalo ana i ka ʻili ʻana o ka ʻili, e hana ana i nā leona hou. He mea pono nō hoʻi e akahele i ka ʻauʻau ʻana, e pale ana i ka wela o ka wai.

Ke noi a botox ma nā wāwae e like me ka maikaʻi o ka pale ʻana i nā palapū i kēia wahi, a hōʻike ʻia ka gastrostomy ke hiki ʻole ke ʻai pono me ka ʻole o nā pūlehu i ka waha a i ʻole ka esophagus.

Pehea e hana ai i ka lole

ʻO ka ʻaʻahu kahi ʻāpana o ka maʻamau o nā mea i loaʻa i ka epidermolysis bullous a pono e hana ʻia kēia mau lole me ke akahele e paipai ia i ka hoʻōla, hoʻoliʻiliʻi i ka hakakā a pale i ke kahe ʻana mai ka ʻili, no ka mea nui kēia e hoʻohana i nā huahana pili ʻole ma ka ʻili. , ʻo ia hoʻi, ʻaʻohe kāpili e hoʻopili ikaika loa.

No ka ʻaukā ʻana i nā ʻeha i loaʻa kahi huna nui, pono ia e hoʻohana i nā kapa komo i hana ʻia me ka hua polyurethane, ʻoiai lākou e omo ai i kēia mau wai a hāʻawi i ka pale no nā microorganism.

I nā hihia kahi e maloʻo ai nā ʻeha, makemake ʻia e hoʻohana i nā lole hydrogel, no ka mea kōkua lākou i ka hoʻopau ʻana i ka ʻili o ka ʻili make a hōʻoluʻolu i ka ʻeha, ka ʻili a me ka hōʻoluʻolu ʻole ma ia wahi. Pono e hoʻopaʻa pono i nā kapa komo me nā meshes tubular a i ʻole elima, ʻaʻole ia e koi ʻia e hoʻohana i nā mea hoʻopili ma ka ʻili.

He aha nā pilikia

Hiki i ka bullous epidermolysis ke hana i kekahi mau pilikia e like me nā maʻi, ʻoiai ke kūkulu ʻia ʻana o nā palapū e waiho i ka ʻili i maʻalahi i ka hoʻohaumia ʻia e nā bacteria a me nā fungi, no ka laʻana. I kekahi mau hanana koʻikoʻi hou aʻe, hiki i kēia mau koʻohune ke komo i ka ʻili o ke kanaka me ka bullous epidermolysis ke hiki i ke kahe o ke koko a pāhola i ke koena o ke kino, e hana ana i nā sepsis.

ʻO ka poʻe me ka epidermolysis bullosa e ʻeha pū kekahi i nā hemahema o ke olakino, e ala aʻe mai nā palapū i ka waha a i ʻole mai ka anemia, i hoʻokumu ʻia e nā kahe mai nā leona. Hiki ke ʻike ʻia kekahi mau pilikia niho, e like me nā caries, no ka mea, palupalu ka uhi ʻana o ka waha i ka poʻe me kēia maʻi. Eia kekahi, ʻo kekahi ʻano o ka epidermolysis bullosa e hoʻonui i ka makaʻu o ke kanaka e ulu ana i ka maʻi ʻaʻai ʻili.