Kwenzekani Ngesikhathi Sokuhlaselwa Kwamagugu Kwe-Angioedema?

-Delile

• Izinguquko zofuzo zenzeka kufayela le- UKUSEBENZA 1 isakhi sofuzo
• Amazinga we-C1 esterase inhibitor ancishiswa ngenani noma ekusebenzeni
• Okuthile kubangela ukufunwa kwe-C1 esterase inhibitor
• IKallikrein iyasebenza
• Kukhiqizwa amanani amaningi we-bradykinin
• Imithambo yegazi ivuza uketshezi oluningi
• Uketshezi luhlangana ezicutshini zomzimba
• Ukuvuvukala kwenzeka
• Kwenzekani kuhlobo 3 HAE
• Ukwelapha ukuhlaselwa

Abantu abane-angioedema yefa (HAE) bahlangabezana neziqephu zokuvuvukala kwezicubu ezithambile. Izimo ezinjalo zenzeka ezandleni, ezinyaweni, emgodini wamathumbu, esithweni sangasese, ebusweni nasemphinjeni.

Ngesikhathi sokuhlaselwa kwe-HAE, ukuguqulwa kwezakhi zofuzo komuntu kubangela ukuqhuma kwemicimbi eholela ekuvuvukala. Ukuvuvukala kuhluke kakhulu ekuhlaselweni ukungezwani komzimba.

Izinguquko zofuzo zenzeka kufayela le- UKUSEBENZA 1 isakhi sofuzo

Ukuvuvukala kuyimpendulo ejwayelekile yomzimba wakho ekutheleleni, ukucasuka, noma ukulimala.

Ngesinye isikhathi, umzimba wakho udinga ukwazi ukulawula ukuvuvukala ngoba okuningi kungaholela ezinkingeni.

Kunezinhlobo ezintathu ezehlukene ze-HAE. Izinhlobo ezimbili ezivame kakhulu ze-HAE (izinhlobo 1 no-2) zidalwa ukuguqulwa (amaphutha) kufuzo olubizwa ngokuthi UKUSEBENZA 1. Lesi sakhi sitholakala ku-chromosome 11.

Lolu hlobo luhlinzeka ngemiyalo yokwenza iprotheni ye-C1 esterase inhibitor (C1-INH). I-C1-INH iyasiza ekwehliseni ukuvuvukala ngokuvimba ukusebenza kwamaprotheni akhuthaza ukuvuvukala.

Amazinga we-C1 esterase inhibitor ancishiswa ngenani noma ekusebenzeni

Ukuguqulwa okubangela i-HAE kungaholela ekwehliseni amazinga e-C1-INH egazini (uhlobo 1). Kungabanga futhi ne-C1-INH engasebenzi kahle, ngaphandle kwezinga elijwayelekile le-C1-INH (uhlobo 2).

Okuthile kubangela ukufunwa kwe-C1 esterase inhibitor

Ngesinye isikhathi, umzimba wakho uzodinga i-C1-INH ukusiza ukulawula ukuvuvukala. Okunye ukuhlaselwa kwe-HAE kwenzeka ngaphandle kwesizathu esicacile. Kukhona futhi izimbangela ezandisa isidingo somzimba wakho se-C1-INH. Izimbangela ziyahlukahluka kuye ngomuntu, kepha izimbangela ezivamile zifaka:

• imisebenzi yomzimba ephindaphindwayo
• imisebenzi edala ingcindezi endaweni eyodwa yomzimba
• isimo sezulu esibandayo noma izinguquko kusimo sezulu
• ukuchayeka kakhulu elangeni
• ukulunywa yizinambuzane
• ukucindezeleka ngokomzwelo
• ukutheleleka noma ezinye izifo
• ukuhlinzwa
• izinqubo zamazinyo
• izinguquko ze-hormonal
• ukudla okuthile, njengamantongomane noma ubisi
• imithi yokwehlisa umfutho wegazi, eyaziwa njenge-ACE inhibitors

Uma une-HAE, awunayo i-C1-INH eyanele egazini lakho ukulawula ukuvuvukala.

IKallikrein iyasebenza

Isinyathelo esilandelayo ochungechungeni lwemicimbi eholela ekuhlaselweni kwe-HAE sifaka i-enzyme egazini eyaziwa ngokuthi i-kallikrein. I-C1-INH icindezela i-kallikrein.

Ngaphandle kwe-C1-INH eyanele, umsebenzi we-kallikrein awuvinjelwe. I-kallikrein bese isika (ihlukanisa) i-substrate eyaziwa njenge-high-molecular-weight kininogen.

Kukhiqizwa amanani amaningi we-bradykinin

Lapho i-kallikrein ihlukanisa i-kininogen, iphumela ku-peptide eyaziwa ngokuthi i-bradykinin. I-Bradykinin iyi-vasodilator, inhlanganisela evula (ivule) ukukhanya kwemithambo yegazi. Ngesikhathi sokuhlaselwa kwe-HAE, kukhiqizwa amanani amaningi ngokweqile ama-bradykinin.

Imithambo yegazi ivuza uketshezi oluningi

I-Bradykinin ivumela uketshezi oluningi ukuthi lungene emithanjeni yegazi luye ezicutshini zomzimba. Lokhu kuvuza nokuvuleka komthambo wegazi okukubangelayo nakho kuphumela ekunciphiseni kwegazi.

Uketshezi luhlangana ezicutshini zomzimba

Ngaphandle kwe-C1-INH eyanele ukulawula le nqubo, uketshezi luyakheka ezicutshini ezingaphansi komzimba.

Ukuvuvukala kwenzeka

Uketshezi olweqile lubangela iziqephu zokuvuvukala okukhulu okubonwa kubantu abane-HAE.

Kwenzekani kuhlobo 3 HAE

Uhlobo lwesithathu, olungavamile kakhulu lwe-HAE (uhlobo 3), lwenzeka ngodaba oluhlukile. Uhlobo lwe-3 kungumphumela wokuguquka kwesakhi sofuzo esihlukile, esisendaweni ye-chromosome 5, ebizwa F12.

Lolu hlobo luhlinzeka ngemiyalo yokwenza iprotheni ebizwa ngokuthi yi-coagulation factor XII. Le phrotheni ibandakanyekile ekunqandeni igazi futhi ibuye ibhekane nokukhuthaza ukuvuvukala.

Ukuguquka kwesimo ku- F12 ufuzo lwakha iprotheni ye-XII enomsebenzi owandayo. Lokhu-ke kudala ukuthi kukhiqizwe i-bradykinin eningi. Njengezinhlobo 1 no-2, ukwanda kwe-bradykinin kwenza izindonga zemithambo yegazi zivuze ngokungalawuleki. Lokhu kuholela eziwombeni zokuvuvukala.

Ukwelapha ukuhlaselwa

Ukwazi okwenzekayo ngesikhathi sokuhlaselwa kwe-HAE kuholele ekuthuthukisweni kwezindlela zokwelashwa.

Ukuvimba uketshezi ekwakheni, abantu abane-HAE badinga ukuthatha imishanguzo. Izidakamizwa ze-HAE zivimbela ukuvuvukala noma zenyuse inani le-C1-INH egazini.

Lokhu kufaka phakathi:

• ukumnika okuqondile kweplasma efriziwe efriziwe (equkethe i-C1 esterase inhibitor)
• imishanguzo ebuyisela i-C1-INH egazini (lokhu kubandakanya i-Berinert, i-Ruconest, i-Haegarda, neCinryze)
• ukwelashwa kwe-androgen, njengesidakamizwa esibizwa nge-danazol, esingakhuphula inani le-C1-INH esterase inhibitor ekhiqizwa isibindi sakho
• i-ecallantide (Kalbitor), umuthi ovimbela ukuhlukaniswa kwe-kallikrein, ngaleyo ndlela uvimbele ukukhiqizwa kwe-bradykinin
• icatibant (Firazyr), eyeka i-bradykinin ekuzibophezeleni kwi-receptor yayo (i-bradykinin B2 receptor antagonist)

Njengoba ukwazi ukubona, ukuhlaselwa kwe-HAE kwenzeka ngokungafani nokusabela okweqile. Izidakamizwa ezisetshenziselwa ukwelapha ukusabela okweqile, njengama-antihistamine, ama-corticosteroids, ne-epinephrine, ngeke zisebenze ekuhlaselweni kwe-HAE.