I-angioedema yefa

I-hereditary angioedema yinkinga engajwayelekile kepha ebucayi ngesimiso somzimba sokuzivikela ezifweni. Inkinga idluliselwa ngemindeni. Kubanga ukuvuvukala, ikakhulukazi kobuso nemigwaqo yomoya, nokucinana kwesisu.

I-Angioedema ukuvuvukala okufana nesifuba, kepha ukuvuvukala kungaphansi kwesikhumba esikhundleni sobuso.

I-heereditary angioedema (HAE) ibangelwa izinga eliphansi noma umsebenzi ongafanele weprotheni ebizwa nge-C1 inhibitor. Kuthinta imithambo yegazi. Ukuhlaselwa kwe-HAE kungadala ukuvuvukala okusheshayo kwezandla, izinyawo, izitho, ubuso, umgudu wamathumbu, i-larynx (i-voicebox), noma i-trachea (i-windpipe).

Ukuhlaselwa kokuvuvukala kungaba nzima kakhulu ebusheni nasebusheni.

Imvamisa kunomlando womndeni waleso simo. Kepha izihlobo zingahle zingazi ngamacala adlule, okungenzeka ukuthi abikwa njengokufa okungalindelekile, okungazelelwe, nokufika ngaphambi kwesikhathi komzali, umalumekazi, umalume noma ugogo noma umkhulu.

Izinqubo zamazinyo, ukugula (kubandakanya imikhuhlane nomkhuhlane), nokuhlinzwa kungadala ukuhlaselwa kwe-HAE.

Izimpawu zifaka:

• Ukuvinjelwa kwe-Airway - kufaka ukuvuvukala komphimbo nokushuba okungazelelwe
• Phinda iziqephu zokuminyana kwesisu ngaphandle kwesizathu esisobala
• Ukuvuvukala ezandleni, izingalo, imilenze, izindebe, amehlo, ulimi, umphimbo, noma izitho zangasese
• Ukuvuvukala kwamathumbu - kungaba nzima futhi kuholele ekucingeni kwesisu, ekuhlanzeni, ekuphelelweni ngamanzi emzimbeni, kuhudo, ebuhlungwini nasekushaqekeni ngezikhathi ezithile
• Ukuqhuma okungabonakali, okubomvu

Ukuhlolwa kwegazi (kwenziwe kahle ngesikhathi sesiqephu):

• Umsebenzi we-C1 inhibitor
• Izinga le-C1 inhibitor
• Qedela ingxenye 4

Ama-antihistamine nezinye izindlela zokwelapha ezisetshenziselwa i-angioedema azisebenzi kahle ku-HAE. I-Epinephrine kufanele isetshenziswe ekuphenduleni okusongela impilo. Kunezindlela eziningi zokwelashwa ezivunyelwe yi-FDA ze-HAE.

Ezinye zinikezwa ngomthambo (IV) futhi zingasetshenziswa ekhaya. Abanye banikezwa njengomjovo ngaphansi kwesikhumba isiguli.

• Ukukhetha ukuthi imuphi umenzeli ongasuselwa eminyakeni yomuntu nalapho izimpawu zivela khona.
• Amagama emithi emisha yokwelashwa kwe-HAE afaka phakathi iCinryze, Berinert, Ruconest, Kalbitor, neFirazyr.

Ngaphambi kokuba le mithi emisha itholakale, imithi ye-androgen, efana nedanazol, yayisetshenziswa ukunciphisa imvamisa nobukhulu bokuhlaselwa. Le mithi isiza umzimba ukuthi wenze i-C1 inhibitor engaphezulu. Kodwa-ke, abesifazane abaningi banemiphumela emibi kakhulu evela kule mithi. Zingasetshenziswa nasezinganeni.

Uma ukuhlasela kwenzeka, ukwelashwa kufaka ukukhululeka kobuhlungu noketshezi olunikezwa ngomthambo ngomugqa we-intravenous (IV).

I-Helicobacter pylori, uhlobo lwama-bacterium atholakala esiswini, lungadala ukuhlaselwa yisisu. Imithi elwa namagciwane yokwelapha amabhaktheriya isiza ukunciphisa ukuhlaselwa kwesisu.

Imininingwane engaphezulu nokusekelwa kwabantu abanesimo se-HAE nemindeni yabo kungatholakala ku:

• Inhlangano Kazwelonke Yezinkinga Ezingajwayelekile - rarediseases.org/rare-diseases/hereditary-angioedema
• Inhlangano ye-US Hereditary Angioedema Association - www.haea.org

I-HAE ingasongela impilo futhi izinketho zokwelashwa zikhawulelwe. Ukuthi umuntu wenza kahle kangakanani kuya ngezimpawu ezithile.

Ukuvuvukala kwemigwaqo yomoya kungaba yingozi.

Shayela noma vakashela umhlinzeki wakho wezokunakekelwa kwezempilo uma ucabanga ukuba nezingane futhi unomlando womndeni walesi simo. Fonela futhi uma unezimpawu ze-HAE.

Ukuvuvukala komoya kuyisimo esiphuthumayo esisongela impilo. Uma unenkinga yokuphefumula ngenxa yokuvuvukala, funa usizo lwezokwelapha ngokushesha.

Ukwelulekwa ngofuzo kungasiza kulabo abazoba abazali abanomlando womndeni we-HAE.

Isifo seQuincke; I-HAE - i-angioedema yefa; I-Kallikrein inhibitor - HAE; Umphikisi we-Bradykinin receptor - HAE; Ama-C1-inhibitors - HAE; Isifuba - HAE

• Amasosha omzimba

IDreskin SC. I-Urticaria ne-angioedema. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 237.

ILonghurst H, uCicardi M, uCraig T, et al; Abaphenyi be-COMPACT. Ukuvimbela ukuhlaselwa kwe-angioedema kwefa nge-C1 inhibitor engaphansi. N Engl J Med. 2017; 376 (12): 1131-1140. I-PMID: 28328347 pubmed.ncbi.nlm.nih.gov/28328347/.

IZuraw BL, iChristianen SC. I-angioedema yefa kanye ne-angioedema ye-bradykinin-Mediated. Ku: Burks AW, Holgate ST, O'Hehir RE et al., Ama-eds. Ukungezwani komzimba noMiddleton: Izimiso kanye Nokuzijwayeza. Umhlaka 9. IPhiladelphia, PA: Elsevier; 2020: isahluko 36.