Kodi chikuchitika ndi chiani pa cholowa cha Angioedema?

Zamkati

• Zosintha zimachitika mu Kutumiza 1 jini
• C1 esterase inhibitor milingo imachepetsedwa kuchuluka kapena kugwira ntchito
• China chake chimayambitsa kufunikira kwa C1 esterase inhibitor
• Kallikrein yatsegulidwa
• Kuchuluka kwa bradykinin kumapangidwa
• Mitsempha yamagazi imatulutsa madzimadzi ochulukirapo
• Madzi amadzikundikira mthupi
• Kutupa kumachitika
• Zomwe zimachitika mu mtundu wa 3 HAE
• Kuthetsa chiwembucho

Anthu omwe ali ndi cholowa cha angioedema (HAE) amakumana ndi zotupa zotupa. Zoterezi zimachitika m'manja, m'mapazi, m'mimba, kumaliseche, kumaso, ndi kummero.

Panthawi yamavuto a HAE, kusintha kwamtundu wamunthu kumabweretsa kusokonekera kwa zinthu zomwe zimayambitsa kutupa. Kutupa ndikosiyana kwambiri ndi ziwengo.

Zosintha zimachitika mu Kutumiza 1 jini

Kutupa ndiko kuyankha kwabwino kwa thupi lanu ku matenda, kupsa mtima, kapena kuvulala.

Nthawi ina, thupi lanu limayenera kuthana ndi kutupa chifukwa zochuluka zimatha kubweretsa zovuta.

Pali mitundu itatu ya HAE. Mitundu iwiri yofala kwambiri ya HAE (mitundu 1 ndi 2) imayambitsidwa ndi kusintha kwa zolakwika mu jini yotchedwa Kutumiza 1. Jini ili lili pa chromosome 11.

Jini ili limapereka malangizo opangira C1 esterase inhibitor protein (C1-INH). C1-INH imathandiza pochepetsa kutupa poletsa zochitika za mapuloteni omwe amalimbikitsa kutupa.

C1 esterase inhibitor milingo imachepetsedwa kuchuluka kapena kugwira ntchito

Kusintha komwe kumayambitsa HAE kumatha kubweretsa kuchepa kwa milingo ya C1-INH m'magazi (mtundu 1). Zingathenso kuyambitsa C1-INH yomwe siyigwira bwino ntchito, ngakhale ili ndi mulingo wabwinobwino wa C1-INH (mtundu 2).

China chake chimayambitsa kufunikira kwa C1 esterase inhibitor

Nthawi ina, thupi lanu lidzafunika C1-INH kuti lithandizire kuchepetsa kutupa. Ziwopsezo zina za HAE zimachitika popanda chifukwa chomveka. Palinso zoyambitsa zina zomwe zimakulitsa kufunika kwa thupi lanu la C1-INH. Zomwe zimayambitsa zimasiyanasiyana malinga ndi munthu, koma zoyambitsa zomwe zimachitika ndi monga:

• zochitika zobwerezabwereza zolimbitsa thupi
• zinthu zomwe zimapangitsa kupanikizika m'dera limodzi la thupi
• nyengo yozizira kapena kusintha kwa nyengo
• kutentha kwambiri padzuwa
• kulumidwa ndi tizilombo
• kupsinjika mtima
• matenda opatsirana kapena matenda ena
• opaleshoni
• Njira zamano
• kusintha kwa mahomoni
• zakudya zina, monga mtedza kapena mkaka
• mankhwala ochepetsa kuthamanga kwa magazi, omwe amadziwika kuti ACE inhibitors

Ngati muli ndi HAE, mulibe C1-INH yokwanira m'magazi anu kuti muchepetse kutupa.

Kallikrein yatsegulidwa

Gawo lotsatira pazotsatira zomwe zimayambitsa kuukira kwa HAE zimakhudzana ndi enzyme m'magazi otchedwa kallikrein. C1-INH imapondereza kallikrein.

Popanda C1-INH yokwanira, zochitika za kallikrein sizimaletsedwa. Kallikrein kenako imang'amba (kulekanitsa) gawo lapansi lotchedwa kininogen yolemera kwambiri.

Kuchuluka kwa bradykinin kumapangidwa

Kallikrein ikagawanika ndi kininogen, zimatulutsa peptide yotchedwa bradykinin. Bradykinin ndi vasodilator, kampani yomwe imatsegula (yowunikira) kuwala kwa mitsempha yamagazi. Panthawi ya HAE, ma bradykinin ambiri amapangidwa.

Mitsempha yamagazi imatulutsa madzimadzi ochulukirapo

Bradykinin imalola kuti madzi ambiri adutse mumitsempha yamagazi mthupi. Kutayikira uku ndi kuchepa kwamitsempha yamagazi komwe kumayambitsa kumathandizanso kutsika kwa magazi.

Madzi amadzikundikira mthupi

Popanda C1-INH yokwanira kuyendetsa njirayi, madzimadzi amadzikundikira m'minyewa yathupi yathupi.

Kutupa kumachitika

Kuchuluka kwamadzimadzi kumabweretsa magawo a kutupa kwakukulu komwe kumawoneka mwa anthu omwe ali ndi HAE.

Zomwe zimachitika mu mtundu wa 3 HAE

Mtundu wachitatu, wosowa kwambiri wa HAE (mtundu wachitatu), umachitika mwanjira ina. Mtundu wachitatu ndi zotsatira za kusintha kwa jini lina, lomwe lili pa chromosome 5, lotchedwa F12.

Jini ili limapereka malangizo opangira puloteni yotchedwa coagulation factor XII. Puloteniyi imakhudzidwa ndikumanga magazi komanso imathandizira kupangitsa kutupa.

Kusintha kwa F12 jini imapanga puloteni XII yokhala ndi zochita zambiri. Izi zimapangitsa kuti bradykinin yambiri ipangidwe. Monga mitundu 1 ndi 2, kuchuluka kwa bradykinin kumapangitsa makoma amitsempha yamagazi kutayikira mosalamulirika. Izi zimabweretsa magawo a kutupa.

Kuthetsa chiwembucho

Kudziwa zomwe zimachitika pakuukira kwa HAE kwadzetsa kusintha kwamankhwala.

Pofuna kuti madzi asamange, anthu omwe ali ndi HAE ayenera kumwa mankhwala. Mankhwala a HAE amateteza kutupa kapena kuwonjezera kuchuluka kwa C1-INH m'magazi.

Izi zikuphatikiza:

• kulowetsedwa mwachindunji kwa plasma yopangidwa ndi mazira atsopano (yomwe ili ndi C1 esterase inhibitor)
• Mankhwala omwe amalowa m'malo mwa C1-INH m'magazi (awa ndi Berinert, Ruconest, Haegarda, ndi Cinryze)
• mankhwala a androgen, monga mankhwala otchedwa danazol, omwe amatha kuwonjezera kuchuluka kwa C1-INH esterase inhibitor yopangidwa ndi chiwindi
• ecallantide (Kalbitor), mankhwala omwe amaletsa kuphulika kwa kallikrein, zomwe zimalepheretsa kupanga bradykinin
• icatibant (Firazyr), yomwe imayimitsa bradykinin kuti isamangirire kulandirira (bradykinin B2 receptor antagonist)

Monga mukuwonera, kuukira kwa HAE kumachitika mosiyana ndi momwe zimachitikira. Mankhwala omwe amagwiritsidwa ntchito pochiza zovuta zina, monga antihistamines, corticosteroids, ndi epinephrine, sangagwire ntchito yolimbana ndi HAE.