Nhaka angioedema

Hereditary angioedema idambudziko risingawanzo asi rakakomba nema immune system. Dambudziko rinopfuudzwa kuburikidza nemhuri. Inokonzeresa kuzvimba, kunyanya kumeso nemumhepo, uye kupomba mudumbu.

Angioedema kuzvimba kwakafanana nemukoko, asi iko kuzvimba kuri pasi peganda panzvimbo pepamusoro.

Hereditary angioedema (HAE) inokonzerwa nehuremu hwakaderera kana basa risiri iro repuroteni inonzi C1 inhibitor. Inokanganisa tsinga dzeropa. Kurwiswa kweHAE kunogona kukonzera kukurumidza kuzvimba kwemaoko, tsoka, mitezo, kumeso, matumbo, larynx (voicebox), kana trachea (windpipe).

Kurwiswa kwekuzvimba kunogona kuve kwakanyanya mukupedzisira kwehudiki uye kuyaruka.

Iko kunowanzo kuve nhoroondo yemhuri yemamiriro acho. Asi hama dzinogona kunge dzisingazive nezvenyaya dzakapfuura, dzinogona kunge dzakataurwa sekufa kusingatarisirwi, nekukasira, uye kusati kwasvika kwemubereki, tete, babamukuru, kana sekuru.

Maitiro emazino, kurwara (kusanganisira dzihwa uye dzihwa), uye kuvhiyiwa kunogona kukonzera kurwisa kweHAE.

Zviratidzo zvinosanganisira:

• Kuvharika kwemumhepo - kunosanganisira kuzvimba kwehuro uye hoarseness nekukurumidza
• Dzokorora zvikamu zvekudzvanywa kwemimba pasina chikonzero chiri pachena
• Kuzvimba mumaoko, maoko, makumbo, miromo, maziso, rurimi, huro, kana sikarudzi
• Kuzvimba kwematumbo - kunogona kuve kwakakomba uye kunotungamira mukurumwa kwemimba, kurutsa, kupera mvura mumuviri, manyoka, kurwadziwa, uye dzimwe nguva kushamisika
• Iko kusiri-kujeka, kutsvuka kutsvuka

Ropa bvunzo (zvakaitwa panguva yechikamu):

• C1 inhibitor basa
• C1 inhibitor chikamu
• Zadzisa chikamu 4

Antihistamines uye mamwe marapirwo anoshandiswa angioedema haashande zvakanaka kune HAE. Epinephrine inofanirwa kushandiswa mukutyisidzira hupenyu. Kune akati wandei matsva ekurapa-anotenderwa FDA marapirwo e HAE.

Zvimwe zvinopihwa kuburikidza netsinga (IV) uye zvinogona kushandiswa pamba. Zvimwe zvinopihwa sejekiseni pasi peganda nemurwere.

• Sarudzo yekuti mumiririri angave akaenderana nezera remunhu uye panowanikwa zviratidzo.
• Mazita emishonga mitsva yekurapa HAE anosanganisira Cinryze, Berinert, Ruconest, Kalbitor, uye Firazyr.

Mishonga mitsva iyi isati yavapo, mishonga ye androgen, senge danazol, yaishandiswa kudzikamisa kuwanda uye kuomarara kwekurwiswa. Iyi mishonga inobatsira muviri kuita yakawanda C1 inhibitor. Nekudaro, vakadzi vazhinji vane zvakakomba mhedzisiro kubva kumishonga iyi. Izvo zvakare hazvigone kushandiswa muvana.

Kamwe kurwisa paunongoitika, kurapwa kunosanganisira kuzorodzwa uye zvinwiwa zvinopihwa kuburikidza netsinga neiyo intravenous (IV) mutsetse.

Helicobacter pylori, mhando yebhakitiriya inowanikwa mudumbu, inogona kukonzeresa kurwiswa kwemudumbu. Mishonga inorwisa mabhakitiriya yekurapa mabhakitiriya anobatsira kudzikisira kurwisa kwemimba.

Rumwe ruzivo uye rutsigiro rwevanhu vane HAE mamiriro uye mhuri dzavo zvinogona kuwanikwa pa:

• National Sangano reRare Dambudziko - rarediseases.org/rare-diseases/hereditary-angioedema
• US Hereditary Angioedema Association - www.haea.org

HAE inogona kutyisidzira hupenyu uye sarudzo dzekurapa dzakashomeka. Kuita zvakanaka kunoita munhu kunoenderana nezvakanangana nezviratidzo.

Kuzvimba kwemafambiro emhepo kunogona kuuraya.

Fonera kana kushanyira wako mupi wezvehutano kana iwe uri kufunga kuve nevana uye uine nhoroondo yemhuri yeichi chiitiko. Zvakare fona kana iwe uine zviratidzo zve HAE.

Kuzvimba kwenzira yemhepo ndeyekutyisidzira hupenyu. Kana iwe uchinetseka kufema nekuda kwekuzvimba, tsvaga kurapwa nekukurumidza.

Genetic counselling inogona kubatsira kune vangangove vabereki vane nhoroondo yemhuri yeHAE.

Quincke chirwere; HAE - Nhaka angioedema; Kallikrein inhibitor - HAE; Bradykinin receptor anopikisa - HAE; C1-inhibitors - HAE; Mikoko - HAE

• Antibodies

Dreskin SC. Urticaria uye angioedema. Mune: Goldman L, Schafer AI, eds. Goldman-Cecil Mushonga. 26th ed. Philadelphia, PA: Elsevier; 2020: chitsiko 237.

Longhurst H, Cicardi M, Craig T, et al; VANYORI vaongorori. Kudzivirira kwekugara nhaka angioedema kurwisa ine subcutaneous C1 inhibitor. N Engl J Med. 2017; 376 (12): 1131-1140. PMID: 28328347 pubmed.ncbi.nlm.nih.gov/28328347/.

Zuraw BL, maChristianen SC. Hereditary angioedema uye bradykinin-mediated angioedema. Mune: Burks AW, Holgate ST, O'Hehir RE et al., Eds. Middleton's Allergy: Maitiro uye Maitiro. 9th ed. Philadelphia, PA: Elsevier; 2020: chitsiko 36.