Cholowa cholowa angioedema

Cholowa cholowa angioedema ndi vuto lachilendo koma lowopsa ndi chitetezo cha mthupi. Vutoli limaperekedwa kudzera m'mabanja. Zimayambitsa kutupa, makamaka kumaso ndi m'mlengalenga, komanso kupindika m'mimba.

Angioedema ndikutupa komwe kumafanana ndi ming'oma, koma kutupa kumakhala pansi pakhungu m'malo mokhala pamwamba.

Hereditary angioedema (HAE) imayambitsidwa chifukwa chotsika kapena chosayenera cha puloteni yotchedwa C1 inhibitor. Zimakhudza mitsempha ya magazi. Kuukira kwa HAE kumatha kubweretsa kufulumira kwa manja, mapazi, miyendo, nkhope, matumbo, larynx (voicebox), kapena trachea (windpipe).

Kuukira kwa kutupa kumatha kukhala koopsa kwambiri kumapeto kwaubwana ndi unyamata.

Nthawi zambiri pamakhala mbiri yabanja yokhudza vutoli. Koma achibale sangadziwe za milandu yam'mbuyomu, yomwe mwina akuti imamwalira mosayembekezereka, mwadzidzidzi, komanso msanga kwa kholo, azakhali, amalume, kapena agogo.

Njira zamano, matenda (kuphatikiza chimfine ndi chimfine), ndikuchitidwa opaleshoni zimatha kuyambitsa HAE.

Zizindikiro zake ndi izi:

• Kutsekeka kwa njira yampweya - kumaphatikizapo kutupa pakhosi komanso kuwonongeka kwadzidzidzi
• Bwerezani zigawo zakumapweteka m'mimba popanda chifukwa chomveka
• Kutupa m'manja, mikono, miyendo, milomo, maso, lilime, mmero, kapena kumaliseche
• Kutupa kwa matumbo - kumatha kukhala koopsa ndipo kumayambitsa kupunduka m'mimba, kusanza, kuchepa kwa madzi m'thupi, kutsegula m'mimba, kupweteka, komanso kugwedezeka nthawi zina
• Kutupa kosafinya, kofiira

Kuyezetsa magazi (komwe kumachitika nthawi yayitali):

• C1 inhibitor ntchito
• Mulingo wa C1 inhibitor
• Phatikizani gawo 4

Antihistamines ndi mankhwala ena omwe amagwiritsidwa ntchito pa angioedema sagwira ntchito bwino kwa HAE. Epinephrine iyenera kugwiritsidwa ntchito pakuwopseza moyo. Pali mitundu ingapo yamankhwala yatsopano yovomerezedwa ndi FDA ku HAE.

Zina zimaperekedwa kudzera mumtsempha (IV) ndipo zitha kugwiritsidwa ntchito kunyumba. Ena amaperekedwa ngati jakisoni pansi pa khungu ndi wodwalayo.

• Kusankha wothandizila woyenera kutengera msinkhu wa munthu komanso komwe zizindikirazo zimachitika.
• Mayina a mankhwala atsopano ochizira HAE ndi Cinryze, Berinert, Ruconest, Kalbitor, ndi Firazyr.

Mankhwala atsopanowa asanayambe kupezeka, mankhwala a androgen, monga danazol, anali kugwiritsidwa ntchito kuchepetsa pafupipafupi komanso kuopsa kwa ziwopsezo. Mankhwalawa amathandiza thupi kupanga C1 inhibitor yambiri. Komabe, azimayi ambiri amakhala ndi zovuta zina chifukwa cha mankhwalawa. Sangagwiritsidwenso ntchito kwa ana.

Pomwe chiwonongeko chachitika, chithandizo chimaphatikizapo kupumula kwa ululu ndi madzi amadzimadzi omwe amaperekedwa kudzera mumitsempha yolowera (IV).

Helicobacter pylori, mtundu wa mabakiteriya omwe amapezeka m'mimba, umatha kuyambitsa matenda am'mimba. Maantibayotiki ochiza mabakiteriya amathandizira kuchepa kwa m'mimba.

Zambiri ndi chithandizo kwa anthu omwe ali ndi vuto la HAE ndi mabanja awo amapezeka ku:

• National Organisation for Rare Disways - rarediseases.org/rare-diseases/hereditary-angioedema
• US cholowa Angioedema Association - www.haea.org

HAE ikhoza kukhala yowopseza moyo ndipo zosankha zamankhwala ndizochepa. Momwe munthu amachitira bwino zimadalira zizindikilo zenizeni.

Kutupa kwa maulendowa kungakhale koopsa.

Itanani kapena pitani kwa omwe amakuthandizani ngati mukuganiza zokhala ndi ana ndikukhala ndi mbiri yabanjayi. Komanso itanani ngati muli ndi zizindikiro za HAE.

Kutupa kwa njira yapaulendo ndiwopseza moyo. Ngati mukuvutika kupuma chifukwa cha kutupa, pitani kuchipatala mwachangu.

Upangiri wa chibadwa ungakhale wothandiza kwa oyembekezera kukhala makolo omwe ali ndi mbiri yabanja ya HAE.

Quincke matenda; HAE - cholowa angioedema; Kallikrein choletsa - HAE; Wotsutsa wa Bradykinin receptor - HAE; C1-zoletsa - HAE; Ming'oma - HAE

• Ma antibodies

Dreskin SC. Urticaria ndi angioedema. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 237.

Pezani nkhaniyi pa intaneti Longhurst H, Cicardi M, Craig T, et al; Ofufuza a COMPACT. Kupewa matenda a angioedema obadwa nawo omwe ali ndi kachilombo ka C1 inhibitor. N Engl J Med. 2017; 376 (12): 1131-1140. PMID: 28328347 adasankhidwa.ncbi.nlm.nih.gov/28328347/.

Zuraw BL, Christianen SC. Cholowa cholowa angioedema ndi bradykinin-mediated angioedema. Mu: Burks AW, Holgate ST, O'Hehir RE et al., Olemba. Ziwombankhanga za Middleton: Mfundo ndi Zochita. 9th ed. Philadelphia, PA: Elsevier; 2020: chap 36.