Chii Chiri Kuitika Panguva Yenhaka YeAngioedema Attack?

Zvemukati

• Shanduko dzinoitika mu SERPING1 geni
• C1 esterase inhibitor mazinga anoderedzwa muhuwandu kana basa
• Chinhu chinokonzeresa kudiwa kweC1 esterase inhibitor
• Kallikrein yakaitwa
• Yakawanda mari yebradykinin inogadzirwa
• Tsinga dzeropa dzinodonha mvura yakawandisa
• Fluid inounganidzwa mumuviri munyama
• Kuzvimba kunoitika
• Chii chinoitika murudzi rwechitatu HAE
• Kurapa kurwisa

Vanhu vane nhaka angioedema (HAE) vanoona zvikamu zvekupfava kweturuvara kuzvimba. Zviitiko zvakadaro zvinoitika mumaoko, tsoka, dumbu, mudumbu, kumeso uye pahuro.

Munguva yekurwiswa kweHAE, iyo nhaka yakagarwa nhaka inokonzera kukwira kwezviitiko zvinotungamira mukuzvimba. Iko kuzvimba kwakasiyana kwazvo nekurwisa kweanorwara.

Shanduko dzinoitika mu SERPING1 geni

Kuzvimba kuita kwemuviri wako zvakajairika kuhutachiona, kutsamwa, kana kukuvara.

Pane imwe nguva, muviri wako unofanirwa kukwanisa kudzora kuzvimba nekuti kuwanda kunogona kutungamira kumatambudziko.

Kune matatu matatu akasiyana HAE. Mhando mbiri dzinojairika dze HAE (mhando 1 ne2) dzinokonzerwa nekuchinja (zvikanganiso) mugene inonzi SERPING1. Iri jeni rinowanikwa pane chromosome 11.

Iri geni rinopa rairo yekugadzira iyo C1 esterase inhibitor protein (C1-INH). C1-INH inobatsira pamwe nekuderedza kuzvimba nekuvharira chiitiko chemapuroteni anokurudzira kuzvimba.

C1 esterase inhibitor mazinga anoderedzwa muhuwandu kana basa

Shanduko inokonzeresa HAE inogona kukonzeresa kudzikiswa kwemazinga eC1-INH muropa (mhando 1). Inogona zvakare kukonzera C1-INH isingashande nemazvo, kunyangwe paine mwero weC1-INH (mhando 2).

Chinhu chinokonzeresa kudiwa kweC1 esterase inhibitor

Pane imwe nguva, muviri wako unoda C1-INH kubatsira kudzora kuzvimba. Kumwe kurwiswa kweHAE kunoitika pasina chikonzero chakajeka. Kune zvimwe zvakare zvinokonzeresa izvo zvinowedzera muviri wako kudiwa kweC1-INH. Zvinokonzeresa zvinosiyana kubva kumunhu kumunhu, asi zvinowanzoitika zvinosanganisira:

• kudzokorora zviitiko zvemuviri
• zviitiko zvinogadzira kumanikidza mune imwe nzvimbo yemuviri
• kutonhora kwemamiriro ekunze kana shanduko mumamiriro ekunze
• kupenya kwakanyanya kuzuva
• kurumwa nezvipembenene
• kushungurudzika mupfungwa
• zvirwere kana zvimwe zvirwere
• kuvhiya
• maitiro emazino
• Hormone shanduko
• zvimwe zvekudya, senzungu kana mukaka
• BP - inoderedza mishonga, inozivikanwa seACE inhibitors

Kana uine HAE, hauna C1-INH yakakwana muropa rako kudzora kuzvimba.

Kallikrein yakaitwa

Nhanho inotevera muketani yezviitiko zvinotungamira ku HAE kurwisa inosanganisira enzyme muropa inozivikanwa se kallikrein. C1-INH inodzvinyirira kallikrein.

Pasina kukwana C1-INH, kallikrein chiitiko hachina kubviswa. Iyo kallikrein inozobvarura (ichipatsanura) substrate inozivikanwa seyakakwira-mamorekuru-huremu kininogen.

Yakawanda mari yebradykinin inogadzirwa

Kana kallikrein ikatsemuka kininogen, inoguma ne peptide inozivikanwa se bradykinin. Bradykinin is vasodilator, mukomboni unovhura (unowedzeredza) lumen yemitsipa yeropa. Munguva yekurwiswa kwe HAE, zvakawandisa bradykinin zvinogadzirwa.

Tsinga dzeropa dzinodonha mvura yakawandisa

Bradykinin inobvumidza yakawanda mvura kuti ipfuure nemidzi yeropa mumiviri yemuviri. Izvi zvinoyerera uye kuwedzerwa kwemidziyo yeropa zvinokonzeresa zvakare mukudzikira kweropa.

Fluid inounganidzwa mumuviri munyama

Pasina C1-INH yakakwana yekudzora maitiro aya, fluid inowanda mumatumbu emuviri anotsvedza.

Kuzvimba kunoitika

Kuwedzera kwemvura kunoguma muzvikamu zvekuzvimba kwakanyanya kunoonekwa kune vanhu vane HAE.

Chii chinoitika murudzi rwechitatu HAE

Chechitatu, chisingawanzoitika mhando ye HAE (mhando 3), inoitika mune imwe nyaya. Type 3 ndiyo mhedzisiro yekuchinja mune rimwe jeni, iri pane chromosome 5, inonzi F12.

Iri geni rinopa mirairo yekugadzira protein inonzi coagulation factor XII. Iyi protein inobatanidzwa mukugwamba kweropa uye zvakare inokonzera kukurudzira kuzvimba.

Shanduko mu F12 geni inogadzira chinhu XII protein ine yakawedzera chiitiko. Izvi zvinokonzeresa kuti yakawanda bradykinin ibudiswe. Senge mhando 1 ne2, kuwedzera kwe bradykinin kunoita kuti ropa remidziyo reropa ribude zvisingadzoreke. Izvi zvinotungamira kuzvikamu zvekuzvimba.

Kurapa kurwisa

Kuziva zvinoitika panguva yekurwiswa kweHAE kwakatungamira mukuvandudzwa kwemishonga.

Kumisa fluid kubva pakuvaka, vanhu vane HAE vanofanirwa kutora mishonga. HAE zvinodhaka zvinogona kudzivirira kuzvimba kana kuwedzera huwandu hweC1-INH muropa.

Izvi zvinosanganisira:

• kuiswa kwakananga kweiyo yakapihwa nyowani yakaomeswa echando plasma (iyo ine C1 esterase inhibitor)
• mishonga inotsiva C1-INH muropa (izvi zvinosanganisira Berinert, Ruconest, Haegarda, uye Cinryze)
• androgen therapy, yakadai semushonga unonzi danazol, iyo inogona kuwedzera huwandu hweC1-INH esterase inhibitor inogadzirwa nechiropa chako
• ecallantide (Kalbitor), mushonga unodzivisa kuchenesa kwekallikrein, nekudaro ichitadzisa kugadzirwa kwe bradykinin
• icatibant (Firazyr), iyo inomisa bradykinin kubva pakusunga kune yayo inogamuchira (bradykinin B2 receptor anopikisa)

Sezvauri kuona, kurwisa kweHAE kunoitika zvakasiyana nechero allergic reaction. Zvinodhaka zvinoshandiswa kurapa kusagadzikana, sema antihistamine, corticosteroids, uye epinephrine, hazvishande mukurwisa kwe HAE.