Kwenzeka ntoni Ngexesha lokuhlaselwa kweLifa leAngioedema?

Umxholo

• Utshintsho olwenzekayo kwi UKUFAKWA KWENKONZO 1 Uhlobo
• Amanqanaba e-C1 esterase inhibitor ancitshisiwe kwimali okanye ekusebenzeni
• Into ethile ibangela ibango le-C1 esterase inhibitor
• IKallikrein iyasebenza
• Amanani agqithisileyo e-bradykinin aveliswa
• Imithambo yegazi ivuza ulwelo oluninzi
• Ulwelo luqokelela kwizicubu zomzimba
• Ukudumba kwenzeka
• Kwenzeka ntoni kuhlobo lwe-3 HAE
• Ukunyanga uhlaselo

Abantu abane-angioedema yelifa (HAE) bafumana iziqendu zokudumba kwezicubu ezithambileyo. Iimeko ezinjalo zenzeka ezandleni, ezinyaweni, kumathambo esini, kumalungu esini, ebusweni nasemqaleni.

Ngethuba lokuhlaselwa kwe-HAE, ukuguqulwa kwemfuza yomntu kubangela i-cascade yeziganeko ezikhokelela ekudumbeni. Ukudumba kwahluke kakhulu kuhlaselo lokungabikho komzimba.

Utshintsho olwenzekayo kwi UKUFAKWA KWENKONZO 1 Uhlobo

Ukuvuvukala yimpendulo eqhelekileyo yomzimba wakho kusulelo, ukucaphuka, okanye ukwenzakala.

Ngexesha elithile, umzimba wakho kufuneka ukwazi ukulawula ukuvuvukala kuba ubuninzi bunokukhokelela kwiingxaki.

Zintathu iintlobo ze-HAE. Ezi ntlobo zimbini zixhaphakileyo ze-HAE (iindidi 1 no-2) zibangelwa lutshintsho (iimpazamo) kwimfuza ebizwa ngokuba yi-HAE UKUFAKWA KWENKONZO 1. Olu luhlobo lubekwe kwi-chromosome 11.

Olu luhlobo lubonelela ngemiyalelo yokwenza iprotein ye-C1 esterase inhibitor (C1-INH). I-C1-INH inceda ekunciphiseni ukudumba ngokuthintela umsebenzi weeproteni ezikhuthaza ukudumba.

Amanqanaba e-C1 esterase inhibitor ancitshisiwe kwimali okanye ekusebenzeni

Ukutshintsha okubangela i-HAE kungakhokelela ekunciphiseni amanqanaba e-C1-INH egazini (uhlobo 1). Inokubangela ukuba i-C1-INH ingasebenzi kakuhle, ngaphandle kwenqanaba eliqhelekileyo le-C1-INH (uhlobo 2).

Into ethile ibangela ibango le-C1 esterase inhibitor

Ngexesha elithile, umzimba wakho uya kufuna i-C1-INH ukukunceda ukulawula ukuvuvukala. Olunye uhlaselo lwe-HAE lwenzeka ngaphandle kwesizathu. Kukwakho nezinye izinto ezibangela ukuba umzimba wakho ufune i-C1-INH. Izinto ezibangela ziyahluka ukusuka komnye umntu ukuya komnye, kodwa izinto eziqhelekileyo zibandakanya:

• imisebenzi yomzimba ephindaphindwayo
• imisebenzi eyenza uxinzelelo kwindawo enye yomzimba
• imozulu ebandayo okanye utshintsho kwimozulu
• ukubonakaliswa kakhulu lilanga
• Ukulunywa zizinambuzane
• uxinzelelo lweemvakalelo
• usulelo okanye ezinye izifo
• utyando
• iinkqubo zamazinyo
• utshintsho lwehomoni
• ukutya okuthile, njengamantongomane okanye ubisi
• uxinzelelo lwegazi lokunciphisa amayeza, awaziwa njenge-ACE inhibitors

Ukuba unayo i-HAE, awunayo i-C1-INH eyaneleyo egazini lakho ukulawula ukudumba.

IKallikrein iyasebenza

Inyathelo elilandelayo kuthotho lweziganeko ezikhokelela kuhlaselo lwe-HAE lubandakanya i-enzyme egazini elibizwa ngokuba yi-kallikrein. I-C1-INH icinezela i-kallikrein.

Ngaphandle kwe-C1-INH eyaneleyo, umsebenzi we-kallikrein awuthintelwanga. I-kallikrein emva koko icanda (yahlulahlula) i-substrate eyaziwa ngokuba yi-high-molecular-weight kininogen.

Amanani agqithisileyo e-bradykinin aveliswa

Xa i-kallikrein yahlulahlula i-kininogen, ibangela ipeptide eyaziwa ngokuba yi-bradykinin. I-Bradykinin yi-vasodilator, ikhompawundi evula (ivule) ukukhanya kwemithambo yegazi. Ngexesha lokuhlaselwa kwe-HAE, izixa ezigqithileyo ze-bradykinin ziveliswa.

Imithambo yegazi ivuza ulwelo oluninzi

I-Bradykinin ivumela ulwelo oluninzi ukuba ludlule kwimithambo yegazi kwizicubu zomzimba. Oku kuvuza kunye nokonyuka kwenqanawa yegazi kubangela iziphumo zoxinzelelo lwegazi olusezantsi.

Ulwelo luqokelela kwizicubu zomzimba

Ngaphandle kwe-C1-INH eyaneleyo yokulawula le nkqubo, ulwelo luyakheka kwizicwili zomzimba ezingaphantsi.

Ukudumba kwenzeka

Iziphumo zolwelo ezingaphezulu kwesiqendu sokudumba okunzima kubonwe kubantu abane-HAE.

Kwenzeka ntoni kuhlobo lwe-3 HAE

Uhlobo lwesithathu, olunqabileyo kakhulu lwe-HAE (uhlobo 3), lwenzeka kumcimbi owahlukileyo. Uhlobo lwe-3 sisiphumo sokutshintsha kwemfuza eyahlukileyo, ebekwe kwi-chromosome 5, ebizwa F12.

Le gene ibonelela ngemiyalelo yokwenza iprotein ebizwa ngokuba yi-coagulation factor XII. Le protein iyabandakanyeka ekunqandeni igazi kwaye ikwanoxanduva lokukhuthaza ukudumba.

Utshintsho kwi F12 Uhlobo lwento ludala iprotein ye-XII ngomsebenzi owandileyo. Oku kubangela ukuba i-bradykinin engaphezulu iveliswe. Njengeentlobo 1 no-2, ukwanda kwe-bradykinin kwenza ukuba udonga lwempahla yegazi luvuze ngokungalawulekiyo. Oku kukhokelela kwiziqendu zokudumba.

Ukunyanga uhlaselo

Ukwazi okwenzekayo ngexesha lokuhlaselwa kwe-HAE kukhokelele kuphuculo kunyango.

Ukuyekisa ulwelo ekwakheni, abantu abane-HAE kufuneka bathathe amayeza. Iziyobisi ze-HAE zinokuthintela ukudumba okanye zonyuse inani le-C1-INH egazini.

Oku kubandakanya:

• ukumiliselwa ngokuthe ngqo kwiplasma efriziwe efriziweyo (equlathe i-C1 esterase inhibitor)
• amayeza athatha indawo ye-C1-INH egazini (ezi ziquka iBerlinert, iRuconest, i-Haegarda, neCinryze)
• Unyango lwe-androgen, njengechiza elibizwa ngokuba yi-danazol, elinokunyusa inani le-C1-INH esterase inhibitor eveliswe sisibindi sakho
• I-ecallantide (Kalbitor), iyeza elithintela ukucocwa kwe-kallikrein, oko kuthintela ukuveliswa kwe-bradykinin
• icatibant (Firazyr), eyeka i-bradykinin ekubambeni kwi-receptor yayo (bradykinin B2 receptor antagonist)

Njengoko ubona, ukuhlaselwa kwe-HAE kwenzeka ngokungafaniyo nokuphendula ngokuthe ngqo. Iziyobisi ezisetyenziselwa ukunyanga i-allergen, njenge-antihistamines, i-corticosteroids, kunye ne-epinephrine, ngekhe isebenze kuhlaselo lwe-HAE.