He aha ka maʻi o Behçet?

He aha ka maʻi o Behçet?

ʻO ka maʻi o Behçet kahi maʻi e pili ana i ka mumū o nā kīʻaha koko. Hōʻike nui ʻia ia e nā maʻi ʻeha ma ka waha a i ʻole i nā ʻāpā, akā me ka hōʻino ʻia o nā maka, ka ʻili a me nā hono. ʻO nā hiʻohiʻona nui hou aʻe e pili ana i ka neurological a i ʻole digestive poino, venous thrombosis a me arterial aneurysms a me kekahi mau ophthalmological poino i hiki i ka makapō. ʻO ka hoʻomaʻamaʻa ka hōʻailona mua loa a hiki paha ke komo i ka colchicine a me nā corticosteroids me a i ʻole me ka ʻole o nā immunosuppressants no nā hōʻike ʻoi loa.

He aha ka maʻi o Behçet?

Ua wehewehe mua ʻia kēia maʻi e ka dermatologist Behçet i ka makahiki 1934. Kuhi ʻia kahi maʻi ʻōpala e komo pū paha me ka vasculitis, ʻo ia hoʻi ka huehue, o nā aʻa a me / a i ʻole nā ​​uaua o ka liʻiliʻi a nui paha. , a me nā thromboses, ʻo ia hoʻi nā clots e hana pū ana i nā aʻa a me / a i ʻole nā ​​uaua.

ʻO ka maʻi ʻo Behçet ka mea nui i ka Basin Mediterranean a ma Iapana. Hoʻopili ia i nā kāne a me nā wahine akā ʻoi aku ka paʻakikī i nā kāne. Hana ʻia ia ma waena o nā makahiki 18 a 40 a hiki ke ʻike ʻia i nā keiki. 

Hoʻololi ia i nā lūlū, interspersed me nā wā o ke kala. Hiki ke make i kekahi manawa, ma muli o nā pilikia neurological, vascular (aneurysm ruptured) a i ʻole nā ​​maʻi gastrointestinal. Hele ka heluna nui o nā mea maʻi i ke kala.

He aha nā kumu o ka maʻi o Behçet?

ʻAʻole ʻike ʻia ke kumu o ka maʻi o Behçet. 

Hoʻopili nā Immunological, e like me nā triggers autoimmune, a me viral (e like me ka herpes virus) a i ʻole ka bacteria (eg streptococci). ʻO ka alele HLA-B51 kahi mea nui weliweli. ʻOiaʻiʻo, hiki i nā mea lawe i kēia allele ke makaʻu i ka hoʻomohala ʻana i ka maʻi 1,5 a 16 mau manawa ke kiʻekiʻe i ka hoʻohālikelike ʻia me nā mea lawe ʻole.

He aha nā hōʻailona o ka maʻi o Behçet?

He ʻano like ʻole nā ​​hōʻike maʻi o ka maʻi o Behçet a hiki ke hoʻopau ʻia i nā hana o kēlā me kēia lā nohona. Pākuʻi ʻia kēia mau mea:

  • ʻinoʻino o ka ʻili e like me nā puʻupuʻu waha i loaʻa i 98% o nā hihia, loaʻa i nā maʻi ʻōpū genital ma 60% o nā hihia a me kahi kūpono i nā kāne ma ka scrotum, pseudo-follikulitis, dermo-hypodermic nodules i 30 a 40% o nā hihia hihia.
  • nā hōʻino pū, e like me ka arthralgia a me ka oligoarthritis inflammatory o nā hono nui (nā kuli, nā kuʻekuʻe wāwae), i loaʻa i 50% o nā hihia;
  • pohō nā mākala, kakaikahi loa;
  • poino ka maka, e like me uveitis, hypopyon a choroiditis paha, i loaʻa i 60% o nā hihia, a me ke kumu o nā pilikia koʻikoʻi e like me cataract, glaucoma, blindness;
  • ʻino neurological i kēia manawa ma 20% o nā hihia. Hoʻomaka ka hoʻomaka ʻana o ka lapalapa ahi me ke kuni a me ka maʻi poʻo. Hoʻopili pū lākou me ka meningoencephalitis, ka hōʻino ʻana i nā ʻalolo cranial, thrombophlebitis o nā maʻi cerebral;
  • hōʻino kino: venous thrombosis, superficial pinepine, ma 30 a 40% o nā hihia; poino arterial, kakaikahi, e like me ka arteritis inflammatory a i ʻole aneurysms;
  • maʻi kīnā ʻole, laha ʻole, e like me myocarditis, endocarditis a i ʻole pericarditis; 
  • gastrointestinal kāna hana, kakaʻikahi i loko o ʻEulopa, ʻike ʻia lākou e ka ʻeha o ka ʻōpū, ka ʻeha o ka ʻōpū a me ka diarrhea me nā maʻi maʻi ʻōpū, like me ka laha ʻana o ka maʻi o Crohn a i ʻole colitis ulcerative;
  • hiki i nā maʻi ʻē aʻe ke ʻike ʻia, i loko o ke kiko a me ka testicular.

Pehea e mālama ai i ka maʻi o Behçet?

ʻAʻohe lāʻau lapaʻau no ka maʻi o Behçet. Kūpono nā mālama ʻana e kaohi i ka maʻi ma o ka hōʻemi ʻana i ka mumū.

ʻO ka mālama ʻana i ka maʻi o Behçet he multidisciplinary (kauka laulima, ophthalmologist, internist, etc.). Pili ka mālama ʻana i nā hōʻike maʻi.

  • colchisin (1 a 2 milligrams i kēlā me kēia lā) ke kumu o ka mālama ʻana, keu hoʻi i ka ʻili a me nā hui pū. Ua lawa paha ma nā ʻano akahai;
  • pono i ka neurological, ocular and vascularʻino e mālama me nā corticosteroids a i ʻole nā ​​immunosuppressants (cyclophosphamide, azathioprine, mycophenolate mofetil, methotrexate) e ka ʻōnaehana;
  • i kekahi mau ʻano ocular koʻikoʻi, hiki ke hoʻohana ʻia ka alpha interferon e nā subcutaneous injection;
  • anti-TNF alpha antibodies e hoʻomāhuahua mau ʻia i nā ʻano koʻikoʻi o ka maʻi a i ʻole nā ​​ʻano kūpaʻa i nā hana ma mua;
  • nā pono kūloko, ma nā ʻano ocular, pono paha (nā kulu maka e pili ana i nā corticosteroids i hui pū ʻia me nā kulu maka e hoʻonui ai i ka haumāna i mea e pale ai i nā pilikia o ka uveitis);
  • hoʻohana ʻia nā anticoagulants waha e lahilahi i ke koko e mālama ai i ka thrombosis.

I ka manawa like, koi ʻia e hōʻoki i ka puhipaka, ʻo ka paka kahi kumu kūpono no ka hōʻemi ʻana i nā maʻi vaskular. Lawe i nā corticosteroids, keu hoʻi i nā koina kiʻekiʻe, pono e hele pū me ka papaʻai i haʻahaʻa i nā kō a me nā paʻakai. I ka hanana o ka ʻeha pū, ka hoʻomaʻamaʻa ʻana i nā hoʻoikaika kino ikaika, kaawale me nā pahu, hiki ke kōkua i ka mālama ʻana i ka maʻalahi o nā hono a me ka ikaika o ka muscular.

ʻO ka mea hope loa, ʻoiai ʻo ka maʻi ʻo Behçet e hiki ai ke hoʻolilo i ka hopohopo a me ka loli o ke kiʻi ponoʻī o kekahi, hiki i ke kākoʻo psychological ke kōkua e ʻae maikaʻi i kahi maʻi a e kūpale pū me ia i kēlā me kēia lā.

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