I-angioedema yelifa

I-angioedema yelifa lelona ngxaki inqabileyo kodwa enzima kumajoni omzimba. Ingxaki idluliselwa kwiintsapho. Kubangela ukudumba, ngakumbi ubuso kunye neendlela zomoya, kunye nokubamba isisu.

I-Angioedema kukudumba okufana nemingxuma, kodwa ukudumba kuphantsi kwesikhumba endaweni yomphezulu.

I-Hereditary angioedema (HAE) ibangelwa kwinqanaba eliphantsi okanye umsebenzi ongafanelekanga weprotheni ebizwa ngokuba yi-C1 inhibitor. Ichaphazela imithambo yegazi. Uhlaselo lwe-HAE lunokubangela ukudumba ngokukhawuleza kwezandla, iinyawo, imilenze, ubuso, ithumbu, ityhefu (i-voicebox), okanye i-trachea (umbhobho womoya).

Ukuhlaselwa kokudumba kunokuba nzima kakhulu ebuntwaneni nasekufikiseni.

Kuhlala kunembali yosapho ngale meko. Kodwa izalamane zisenokungazi ngamatyala angaphambili, anokuthi axelwe njengokufa okungalindelekanga, ngequbuliso, kunye nokusweleka ngaphambi kwexesha komzali, umakazi, umalume okanye utatomkhulu.

Iinkqubo zamazinyo, ukugula (kubandakanya ukubanda kunye nomkhuhlane), kunye notyando kunokubangela uhlaselo lwe-HAE.

Iimpawu zibandakanya:

• Ukuvalwa kwomoya-kubandakanya ukudumba komqala kunye nokudakumba ngesiquphe
• Phinda iziqendu zesisu esiswini ngaphandle kwesizathu
• Ukudumba ezandleni, iingalo, imilenze, imilebe, amehlo, ulwimi, umqala, okanye amalungu esini
• Ukudumba kwamathumbu- kunokuba nzima kwaye kukhokelele ekubambeni esiswini, ukugabha, ukuphelelwa ngamanzi emzimbeni, urhudo, iintlungu, kwaye ngamanye amaxesha ukothuka
• Irhashalala engabomvu, ebomvu

Uvavanyo lwegazi (olwenziwe ngokufanelekileyo ngexesha lesiqendu):

• Umsebenzi we-C1 inhibitor
• Inqanaba le-C1 inhibitor
• Cwangcisa icandelo 4

I-Antihistamines kunye nolunye unyango olusetyenziselwa i-angioedema alusebenzi kakuhle kwi-HAE. I-Epinephrine kufuneka isetyenziswe kwiimpendulo ezisongela ubomi. Kukho inani elitsha lonyango olwamkelweyo lwe-FDA lwe-HAE.

Ezinye zinikezelwa ngomthambo (IV) kwaye zinokusetyenziswa ekhaya. Ezinye zinikwa ngenaliti phantsi kolusu ngumguli.

• Ukukhetha ukuba yeyiphi iarhente enokusekwa kubudala bomntu kwaye apho zivela khona iimpawu.
• Amagama amachiza amatsha onyango lwe-HAE aquka iCinryze, iBerinert, iRuconest, iKalbitor, neFirazyr.

Ngaphambi kokuba la mayeza matsha afumaneke, amayeza e-androgen, afana nedanazol, ayesetyenziselwa ukunciphisa ukubanda nokuqina kohlaselo. La mayeza anceda umzimba ukuba wenze i-C1 inhibitor engaphezulu. Nangona kunjalo, uninzi lwabasetyhini luneziphumo ebezingalindelekanga ezimandundu ezivela kula mayeza. Kananjalo azinakusetyenziswa ebantwaneni.

Nje ukuba uhlaselo lwenzeke, unyango lubandakanya ukukhululeka kwentlungu kunye nolwelo olunikezwa ngethambo ngomgca we-intravenous (IV).

Helicobacter pylori, Uhlobo lweebhaktiriya olufumaneka esiswini, lunokubangela ukuhlaselwa sisisu. Amayeza okubulala iintsholongwane anyanga ukuhla kwesisu.

Olunye ulwazi kunye nenkxaso kubantu abane-HAE kunye neentsapho zabo zifumaneka kwi:

• Umbutho weSizwe woNxunguphalo oluNqabileyo- rarediseases.org/rare-diseases/redited-angioedema
• Umbutho wase-US Hereditary Angioedema Association- www.haea.org

I-HAE inokuba sengozini yobomi kwaye unyango luthintelwe. Ukusebenza kakuhle komntu kuxhomekeke kwiimpawu ezithile.

Ukudumba komoya kungabulala.

Tsalela umnxeba okanye undwendwele umboneleli wakho wezempilo ukuba ucinga ukuba nabantwana kwaye unembali yosapho yale meko. Biza kwakhona ukuba unempawu ze-HAE.

Ukudumba kwendlela yomoya sisisongelo esisongela ubomi. Ukuba unengxaki yokuphefumla ngenxa yokudumba, funa unyango kwangoko.

Ingcebiso ngemfuzo ingaluncedo kubazali abanokuba nembali yosapho ye-HAE.

Isifo Quincke; I-HAE - i-angioedema yelifa; I-Kallikrein inhibitor-HAE; Umchasi we-Bradykinin receptor-HAE; I-C1-inhibitors-HAE; Imihlathi - HAE

• Iintsholongwane

IDreskin SC. Urticaria kunye angioedema. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl 237.

Ixesha elide H, uCicardi M, uCraig T, et al; INDLELA yophando. Uthintelo lohlaselo lwe-angioedema yelifa kunye ne-C1 inhibitor engaphantsi. N Engl J Med. Ngo-2017; 376 (12): 1131-1140. IINKCUKACHA: 28328347 pubmed.ncbi.nlm.nih.gov/28328347/.

I-Zuraw BL, iChristianen SC. I-angioedema yelifa kunye ne-bradykinin-Mediated angioedema. Ku: IBurks AW, iHolgate ST, u-O'Hehir RE et al., Ii-eds. I-Middleton's Allergy: Imigaqo kunye nokuziqhelanisa. Umhla we-9. IPhiladelphia, PA: Elsevier; 2020: isahluko 36.