Mea kāohi esterase C1

ʻO C1 esterase mea kāohi (C1-INH) kahi protein i loaʻa i ka ʻāpana wai o kou koko. Hoʻopili ia i kahi protein i kapa ʻia ʻo C1, kahi ʻāpana o ka ʻōnaehana hoʻokō.

ʻO ka ʻōnaehana hoʻopihapiha kahi hui o kahi kokoke i 60 mau protein i ka plasma koko a i ʻole ma luna o kekahi mau hunaola. Hana nā proteins kōkua me kāu ʻōnaehana pale e pale ai i ke kino mai nā maʻi. Kōkua pū lākou i ka hemo ʻana i nā hunaola make a me nā mea haole. ʻEiwa mau protein hoʻopiha nui. Kapa ʻia lākou ʻo C1 a hiki i C9. Kākaʻikahi, hiki i nā kānaka ke hoʻoili i ka hemahema o kekahi mau protein hoʻopiha. Kūpono kēia poʻe i kekahi mau maʻi a i ʻole nā ​​maʻi autoimmune.

Kūkākūkā kēia ʻatikala i ka hoʻāʻo e hana ʻia e ana i ka nui o C1-INH i kou koko.

Pono kahi laʻana koko. Lawe pinepine ʻia kēia ma o ke aa. Kapa ʻia ke kaʻina hana he venipuncture.

ʻAʻole pono kahi hoʻomākaukau kūikawā.

Ke hoʻokomo ʻia ke kui kele e huki i ke koko, manaʻo kekahi poʻe i ka ʻeha kaulike. ʻO ka manaʻo ʻē aʻe wale nō he prick a i ʻole sensation stinging paha. Ma hope, aia paha kekahi ʻōkā.

Pono ʻoe i kēia hōʻoia inā loaʻa iā ʻoe nā hōʻailona o ka hoʻoilina a i ʻole loaʻa angioedema. Hoʻokumu ʻia nā ʻano ʻelua o angioedema e nā pae haʻahaʻa o C1-INH.

He mea nui paha nā mea hoʻopiha i ka hoʻāʻo ʻana no nā maʻi autoimmune, e like me ka lupus erythematosus systemic.

Hiki ke loli iki nā pae waiwai maʻamau i waena o nā hale hana ʻokoʻa. E ana ana kāu mea mālama ola i ka pae hana o kāu mea hoʻopaʻa C1 esterase. E kamaʻilio i kāu mea hāʻawi e pili ana i ke ʻano o kāu mau hopena hōʻike.

ʻO nā pae haʻahaʻa o C1-INH ke kumu i kekahi ʻano angioedema. Nā hualoaʻa Angioedema i ka huʻi koke o nā mea o ka maka, luna o ka ʻāʻī a me ke alelo. He kumu paʻakikī paha ia i ka hanu ʻana. ʻO ka pehu ʻana i loko o ka ʻōpū a me ka ʻeha pūpū pū kekahi. Aia ʻelua ʻano angioedema i hopena ʻia mai nā pae hōʻemi o C1-INH. Hoʻopili ka angioedema hereditary i nā keiki a me nā ʻōpio ʻōpio ma lalo o ka makahiki 20. ʻIke ʻia ka angioedema i loaʻa i ka poʻe mākua ma mua o 40 mau makahiki.

ʻO nā pōpilikia e pili ana i ke kahe ʻana o ke koko he liʻiliʻi, akā hiki ke hoʻopili ʻia:

• Kahe nui ke koko
• Pāleʻale a ʻoluʻolu paha
• Hematoma (hōʻiliʻili koko ma lalo o ka ʻili)
• Maʻiʻaʻai (i iki kamepiula i kēlā me kēia manawa ka ʻili i haki)

C1 mea hōʻemi; C1-INH

• Hōʻaʻo koko

Cicardi M, Aberer W, Banerji A, et al. Hoʻokaʻawale, ʻike, a me nā ala e mālama ai no angioedema: hōʻike ʻae mai ka Hereditary Angioedema International Working Group. ʻAhaʻi ʻaʻano. 2014; 69 (5): 602-616. PMID: 24673465 www.ncbi.nlm.nih.gov/pubmed/24673465.

Leslie TA, Greaves MW. ʻO angioedema hoʻoilina. I: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, eds. Ka mālama ʻana i ka maʻi ʻili: nā ʻōnaehana Therapyutic Comprehensive. 5th lā. Piladelapia, PA: Elsevier; 2018: chap 101.

Zanichelli A, Azin GM, Wu MA, et al. ʻIke, papa, a me ka mālama ʻana i angioedema i nā mea maʻi me ka loaʻa ʻole o ka C1-inhibitor deficit. Hoʻomaʻamaʻa ʻo J Allergy Clin Immunol. 2017; 5 (5): 1307-1313. PMID: 28284781 www.ncbi.nlm.nih.gov/pubmed/28284781.