Ke ʻulaʻula koke nei kou ʻili, pehu, a ʻili? E aʻo kākou e pili ana i kēia Stevens-Johnson Syndrome (SJS) weliweli?

Ua ʻike anei ʻoe, a i ʻole ua hiki mai paha iā ʻoe, kahi ʻulaʻula koke ma kou kino e ʻulaʻula ana, e like me ka ʻulaʻula, a laila ʻike ʻia nā pehu a hemo ka ʻili? Hiki i kēia mau ʻeha ke ʻike ʻia i loko o ka waha, i loko o nā maka, a ma ka ʻāpana genital. ʻAʻole kēia he mea maʻalahi, a hiki ke lilo i maʻi koʻikoʻi e pono ai ka mālama koke ʻia ʻana i ka haukapila . I kēia lā, e kamaʻilio mākou e pili ana i kēia maʻi i kapa ʻia ʻo Stevens-Johnson Syndrome (SJS).

He aha ka Stevens-Johnson Syndrome (SJS)?

I ka ʻōlelo maʻalahi, ʻo Stevens-Johnson Syndrome (SJS) a me Toxic Epidermal Necrolysis (TEN) he mau maʻi ʻili koʻikoʻi loa. ʻO ka mea e hana ʻia, hoʻomaka koke kou ʻili e ulu i nā ʻulaʻula, nā puʻupuʻu ʻeha, a laila pehu a hemo. Hiki iā ia ke hoʻopilikia i kou mau maka, nā ʻōpū, a me nā membrane mucous i loko o kou waha. Inā loaʻa iā ʻoe kēia mau maʻi, pono ʻoe e lawe ʻia i ka haukapila no ka mālama ʻana.

Manaʻo kekahi mau kauka he ʻelua maʻi ʻokoʻa ʻo SJS a me TEN, aʻo kekahi poʻe e ʻōlelo nei he mau pae like ʻole lāua o ka maʻi hoʻokahi. ʻO ka SJS kahi ʻano maʻi ʻoi aku ka liʻiliʻi ma mua o TEN. No ka laʻana, ma SJS, emi ma mua o 10% o ka ʻili ma ke kino holoʻokoʻa e nalowale paha. Ma TEN, ʻoi aku ma mua o 30% o ka ʻili e nalowale paha. Eia nō naʻe, he mea nui e hoʻomanaʻo he hiki i nā kūlana ʻelua ke hoʻoweliweli i ke ola .

He aha nā inoa ʻē aʻe o SJS?

ʻAe, hele ia ma kekahi mau inoa ʻē aʻe. Eia kekahi laʻana:

• ʻO ka maʻi Lyell
• ʻO ka maʻi Stevens-Johnson / ʻona epidermal necrolysis
• ʻO ka maʻi Stevens-Johnson syndrome toxic epidermal necrolysis spectrum

I kekahi manawa, loli ka inoa ma muli o ke kumu. Inā hoʻokumu ʻia e kahi lāʻau lapaʻau, hiki ke kapa ʻia ʻo "drug-induced Stevens-Johnson syndrome", a inā hoʻokumu ʻia e kahi maʻi i kapa ʻia ʻo "Mycoplasma", hiki ke kapa ʻia ʻo "mycoplasma-induced Stevens-Johnson syndrome".

ʻO wai ka mea i ʻoi aku ka paʻakikī o ka loaʻa ʻana o SJS?

ʻIke pinepine ʻia ka SJS i nā poʻe ʻōpio ma lalo o 30 mau makahiki a me nā keiki liʻiliʻi. Eia nō naʻe, hiki ke loaʻa i nā kānaka o kēlā me kēia makahiki, ʻoi aku hoʻi ka poʻe ʻelemakule. ʻOi aku ka nui o ka loaʻa ʻana o ka SJS i nā wahine ma mua o nā kāne.

E noʻonoʻo, inā loaʻa i kahi keiki ʻōpiopio kahi maʻi e like me ka niumonia a laila loaʻa kahi pilikia ʻili e like me kēia ... hiki ke lilo i SJS. ʻO ke kumu nui o SJS i nā keiki he mau maʻi e like me ka niumonia. Akā i nā pākeke, ʻo ka hapa nui o SJS/TEN ke kumu o nā lāʻau lapaʻau a lākou e lawe ai.

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He aha nā kumu pilikia no ka hoʻomohala ʻana o SJS?

ʻAʻohe kumu hoʻokahi no kēia maʻi, akā he hui pū ʻana o nā kumu. ʻO ka mea nui loa o kēia, ʻo ia ka predisposition genetic. ʻO ia hoʻi, hiki i kāu mau genes ke loaʻa kekahi mana. Hiki i kekahi mau mea pili i ke kaiapuni ke hoʻoulu i kēlā mau genes. ʻO kekahi o kēia mau mea genetic nā ʻāpana kūikawā o ko mākou kino i kapa ʻia ʻo (Human Leukocyte Antigens - HLAs). ʻO ka poʻe me kekahi mau ʻano like ʻole o kēia mau (HLAs) he kiʻekiʻe aʻe ka pilikia o ka hoʻomohala ʻana iā SJS a i ʻole TEN ma mua o nā mea ʻē aʻe.

He aha nā hōʻailona o SJS?

Aia kekahi mau hōʻailona e hiki ke loaʻa i ka SJS. He mea nui e makaʻala i kēia mau mea:

• ʻEha nui ma ka ʻili: ʻO ka mea mua, hoʻomaka ka ʻili e ʻeha.
• Kuni: He kuni kou.
• ʻEha kino: Manaʻo ia e ʻeha ana kou kino holoʻokoʻa.
• Nā kiko ʻulaʻula a i ʻole nā ​​​​​​kikoʻī ma ka ʻili: ʻIke ʻia nā kiko ʻulaʻula a i ʻole nā ​​​​​​kikoʻī ma ka ʻili.
• Kuke.
• Nā pehu a me nā ʻeha: Hiki i nā pehu a me nā ʻeha ke ʻike ʻia ʻaʻole wale ma ka ʻili, akā ma nā membrane mucous hoʻi ma nā wahi e like me ka waha, ka ʻāʻī, nā maka, nā ʻōpū, a me ka rectum. E noʻonoʻo, ʻo nā ʻeha i loko o ka waha e paʻakikī ai ʻaʻole wale ka ʻai ʻana akā ke kamaʻilio pū kekahi.
• Ka ʻili ʻana o ka ʻili: ʻIli ka ʻili mai nā pehu.
• Kahe ʻana o ka waimaka: Ma muli o nā ʻeha waha, puka mai ka lawai ke hiki ʻole iā ʻoe ke pani i kou waha.
• Pehu ʻana o ka maka: ʻO nā pehu a me ka pehu ʻana i nā maka e pehu ai nā maka a hiki ʻole ke wehe ʻia.
• ʻEha i ka wā mimi: ʻO nā pehu i loko o nā membrane mucous e hoʻoulu ai i ka ʻeha nui i ka wā mimi.

E noʻonoʻo ua hoʻomaka ʻoe i kahi lāʻau lapaʻau hou, a i loko o kekahi mau lā ua maʻi ʻoe, loaʻa ke kuni, a me nā ʻeha, a laila ʻike koke ʻia nā kiko ʻulaʻula ma kou ʻili a ʻike ʻia nā pehu ... ʻO ia ka manawa e hoʻomaka ai ʻoe e kānalua he SJS kēia.

He aha ke kumu o SJS?

Aia kekahi mau kumu nui o SJS:

• ʻO ka hopena maʻi i kahi lāʻau: ʻO kēia ke kumu nui o ka hapa nui o SJS a me nā hihia TEN āpau. Hiki ke hana kēia inā ʻaʻole ʻoe e ʻike i kahi mahele o kahi lāʻau āu e lawe nei.
• Nā maʻi: No ka laʻana, nā maʻi e like me ka Mycoplasma pneumonia, Herpes virus, a me Hepatitis A.
• ʻO ka maʻi Graft-versus-host: He kūlana kēia e hiki ke hana, ʻoiai ma hope o ka hoʻoili ʻana o ka iwi iwi.
• ʻAʻohe kumu i loaʻa: I kekahi manawa hiki ke ulu aʻe ʻo SJS me ke kumu ʻole.

Inā loaʻa iā ʻoe ka SJS ma muli o kahi lāʻau lapaʻau, hoʻomaka pinepine nā hōʻailona i loko o hoʻokahi, ʻelua, a ʻekolu paha pule o ka hoʻomaka ʻana i ka lāʻau. Hiki iā ʻoe ke hoʻomaka me ke kuni, ka ʻū, a me ke poʻo ʻeha, a ukali ʻia e nā ʻulaʻula ʻili a me ka ʻili ʻana. I kekahi poʻe me TEN, hiki i ka lauoho a me nā kui ke hāʻule. He mea kākaʻikahi loa, ua hōʻike ʻia ʻo SJS i hana ʻia e kahi lāʻau pale hou.

He aha nā lāʻau lapaʻau e hiki ke hoʻoulu iā SJS?

Aia kekahi mau lāʻau lapaʻau i ʻoi aku ka nui o ka pilikia o ke kumu ʻana o SJS. ʻO kēia mau mea:

• Nā lāʻau sulfa antibacterial.
• Nā lāʻau lapaʻau anti-epileptic: ʻO nā laʻana e komo pū me Phenytoin (Dilantin®), Carbamazepine (Tegretol®), Lamotrigine (Lamictal®), a me Phenobarbital (Luminal®).
• (Allopurinol (Aloprim®, Zyloprim®): He lāʻau lapaʻau kēia i hoʻohana ʻia no ka gout a me nā pōhaku puʻupaʻa.
• Nā lāʻau anti-inflammatory ʻaʻole steroidal (NSAID): ʻO nā laʻana e komo pū me Piroxicam (Feldene®), Nevirapine (Viramune®), a me Diclofenac (Cambia®, Flector®).
• Nā lāʻau antibiotic ʻē aʻe.

He mea nui: ʻAʻole kēia manaʻo e loaʻa i nā mea a pau e lawe ana i kēia mau lāʻau lapaʻau ka SJS. Eia nō naʻe, ua ʻike ʻia ua pili kēia mau lāʻau lapaʻau me ka piʻi ʻana o ka pilikia o ka loaʻa ʻana o ka SJS.

Aia kekahi mau kumu ʻē aʻe e hoʻonui ai i ka pilikia o ka hoʻomohala ʻana i ka SJS?

ʻAe, aia kekahi mau kūlana ʻē aʻe e hoʻonui ai i ka pilikia o ka hoʻomohala ʻana i ka SJS. ʻO lākou kēia:

• Ma hope o ka hoʻoili ʻana o ka iwi iwi.
• Loaʻa i nā maʻi autoimmune e like me (Systemic lupus erythematosus - SLE).
• ʻO ka maʻi pale ʻole kanaka (HIV).
• Nā maʻi maʻi ʻē aʻe o nā hono a me nā ʻiʻo pili.
• Ka maʻi ʻaʻai.
• Loaʻa i kahi ʻōnaehana pale maʻi nāwaliwali.
• Inā ua loaʻa mua i kekahi o kou ʻohana ka SJS (mōʻaukala ʻohana).
• Loaʻa iā ia kahi ʻano like ʻole o kahi gene kikoʻī i kapa ʻia ʻo "Human Leukocyte Antigen-B - HLA-B".

Pehea e ʻike ai nā kauka i kēia maʻi SJS?

Hoʻoponopono nā kauka iā SJS a me TEN ma ka noʻonoʻo ʻana i kēia mau mea:

• Ka nānā ʻana i ka ʻili a me nā membrane mucous i hoʻopilikia ʻia: ʻO ka maʻamau ma ka liʻiliʻi he ʻelua mau membrane mucous i hoʻopilikia ʻia.
• Ma muli o ke kiʻekiʻe o ka ʻeha āu e manaʻo nei.
• Ma muli o ka wikiwiki o ka hoʻopilikia ʻia ʻana o kou ʻili.
• Ma muli o ka nui o ka hopena o kou ʻili.
• Biopsy ʻili: I kekahi manawa, lawe ʻia kahi ʻāpana liʻiliʻi o ka ʻili a nānā ʻia ma lalo o kahi microscope e hōʻoia i ka maʻi.

Pehea e mālama ʻia ai ʻo SJS?

He maʻi ʻo SJS e pono ai ka mālama koke ʻana i ka haukapila a me ka mālama ʻana. ʻO ka hoʻāʻo ʻana e mālama iā ia ma ka home hiki ke hoʻonui i ka maʻi. Eia kekahi o nā lāʻau lapaʻau:

• E hoʻōki koke i ka lāʻau lapaʻau i manaʻo ʻia ke kumu o ka pilikia: ʻO kēia ka mea mua e hana ai.
• Nā wai intravenous (IV) e hoʻihoʻi i nā electrolytes: Hana ʻia kēia e kāohi i ka nalowale o ka wai mai ke kino i ka wā e ʻili ai ka ʻili.
• Ke kau nei i nā pāpale pipili ʻole i ka ʻili i hoʻopilikia ʻia.
• ʻO ka hāʻawi ʻana i nā meaʻai kiʻekiʻe i ka calorie e wikiwiki ai ka ho'ōla ʻana: I kekahi manawa, pono paha ka hānai ʻana me ka paipu.
• E hoʻolako i nā antibiotics inā pono e pale aku i ka maʻi.
• Ke hāʻawi nei i nā lāʻau hōʻeha.
• Lāʻau lapaʻau ma ka haukapila, malia paha ma ka Intensive Care Unit (ICU) a i ʻole Burn Unit.
• Ke ʻimi nei i ke kōkua mai nā hui loea e like me Dermatology a me Ophthalmology (inā pilikia nā maka).
• I kekahi mau hihia, hiki ke hāʻawi ʻia nā lāʻau lapaʻau kikoʻī e like me ka IV immunoglobulin, cyclosporine, IV steroids, a i ʻole nā ​​​​​​grafts membrane amniotic (no nā maka).

He aha nā pilikia e hiki ke loaʻa mai ka SJS?

Ma muli o nā maʻi koʻikoʻi ʻo SJS lāua ʻo TEN, hiki ke koʻikoʻi nā pilikia.

• Make: ʻO kēia ka pilikia koʻikoʻi loa. Ma kahi o 10% o nā maʻi me SJS a ma kahi o 50% o nā maʻi me TEN hiki ke make.
• Pneumonia.
• Sepsis: He maʻi bacteria koʻikoʻi kēia, e like me ka lāʻau make koko.
• Kūlana haʻalulu (Haʻalulu).
• Ka hāʻule ʻana o nā ʻōkana he nui.

Ma muli o kēia mau pilikia koʻikoʻi e pono ai nā maʻi SJS/TEN e hoʻokipa koke ʻia i ka haukapila.

Hiki ke pale ʻia ka SJS?

ʻO kaʻoiaʻiʻo, i ka hapanui o nā hihia, ʻaʻole hiki ke pale ʻia ʻo SJS. No ka mea, pinepine ia i hana ʻia e kahi hopena i manaʻo ʻole ʻia i kahi lāʻau lapaʻau, ʻaʻohe ala e ʻike ai inā e hana ʻia kēia ma mua o ka lawe ʻana i kahi lāʻau lapaʻau.

Eia nō naʻe, inā ua ʻike ʻia ʻoe me kahi maʻi i hoʻoulu ʻia e ka lāʻau lapaʻau, ʻo ka mea nui loa, ʻaʻole loa e hoʻohana hou i kēlā lāʻau lapaʻau a i ʻole kahi papa like o nā lāʻau lapaʻau. He mea nui loa ia e hoʻomaopopo i kāu kauka e pili ana i kēia a e hoʻopaʻa iā ia i kāu mau moʻolelo lapaʻau.

He aha ka manaʻo no kekahi me SJS? (Outlook)

ʻAʻole ʻike nā kānaka a pau me SJS i nā hōʻailona like. Hoʻōla kekahi poʻe i loko o kekahi mau pule. Eia nō naʻe, inā koʻikoʻi nā hōʻailona, ​​hiki ke hala i nā mahina e hoʻōla piha ai. Hiki i kekahi poʻe ke ʻike i nā hopena lōʻihi. Eia kekahi laʻana:

• ʻIli: ʻIli maloʻo, maneʻo, hoʻololi ke ʻano o ka ʻili.
• Nā maka: Pehu pinepine a/a maloʻo paha, mumū pinepine o nā maka, ʻike pōwehiwehi, ʻike i ka mālamalama (photophobia).
• Ka hou nui ʻana.
• Ka pōʻino o ka māmā: Nā kūlana e like me ka maʻi pulmonary obstructive mau loa (COPD), ka hānō.
• Nā kui e hāʻule ana a i ʻole e lilo ana i mea ʻino.
• Ka nalowale o ka lauoho (Alopecia).
• Maloʻo o nā membrane mucous: Hiki i kēia ke hana i nā mea e like me ka paʻakikī o ka mimi ʻana.
• Ka maʻi luhi mau loa.
• Nā loli i ka ʻono.

ʻO ka mea nui, hiki i ka maʻi ke hoʻi hou mai inā ua hoʻopili hou ʻia ke kanaka i ka lāʻau i hoʻokumu i ka SJS ma kahi mua. Inā hana ʻia kēlā, ʻoi aku ka koʻikoʻi o nā hōʻailona i ka lua o ka manawa.

Ma kahi o 10% o nā maʻi SJS a me 30% o nā maʻi TEN e make ana, ʻo ke kumu nui ma muli o ka sepsis, ka acute respiratory distress syndrome (ARDS), a me ka hāʻule ʻana o nā ʻōkana he nui.

Eia kekahi mau mea āu e hoʻomanaʻo ai mai kā mākou i kamaʻilio ai (Leka Lawe-Home):

ʻAe, ua kamaʻilio nui mākou e pili ana i kēia kūlana weliweli i kapa ʻia ʻo SJS. Eia kekahi mau mea e pono ai ʻoe e hoʻomanaʻo:

• He mau maʻi ʻili koʻikoʻi loa ka Stevens-Johnson Syndrome (SJS) a me ka Toxic Epidermal Necrolysis (TEN), a he mau maʻi ʻili hoʻi e hoʻoweliweli ana i ke ola.
• Inā loaʻa iā ʻoe nā hōʻailona e like me ka ʻeha o ka ʻili, nā kiko ʻulaʻula, nā pehu, ka ʻili ʻana o ka ʻili, a i ʻole nā ​​ʻeha waha a maka paha ma hope o ka hoʻomaka ʻana i kahi lāʻau hou, ʻoiai i loko o hoʻokahi a ʻelua paha pule, e ʻimi koke i ke kōkua lapaʻau. Hiki paha iā ia ke lilo i SJS.
• E haʻi mau i kāu kauka inā ua loaʻa iā ʻoe kahi maʻi allergy a i ʻole kahi hopena maikaʻi ʻole i kahi lāʻau lapaʻau ma mua.
• Mai hoʻohana hou i kahi lāʻau lapaʻau a i ʻole nā ​​​​lāʻau like i ʻike ʻia he kumu no SJS .**
• Inā kānalua ʻoe i kēia maʻi, mai noho i ka home, e hele koke i ka haukapila. Hiki i ka lāʻau lapaʻau wikiwiki ke hoʻopakele i kou ola.

Manaʻolana wau he mea pono kēia ʻike iā ʻoe. E ola kino!

ʻO Stevens-Johnson Syndrome, SJS, Toxic Epidermal Necrolysis, TEN, Nā maʻi ʻili, Nā maʻi ʻaleka, Nā hopena ʻaoʻao lāʻau, Nā pehu, Nā ʻūlū ʻili, Nā maʻi ʻili