ʻO angioedema hoʻoilina

ʻO ka hered angioedema he mea maʻamau akā pilikia koʻikoʻi me ka ʻōnaehana pale. Hoʻoholo ʻia ka pilikia e nā ʻohana. ʻO ia ke kumu o ka pehu ʻana, ʻo ia hoʻi o ka helehelena a me nā ala ea, a me ka ʻūhū o ka ʻōpū.

ʻO Angioedema ka ʻōhū e like me ka hives, akā aia ka pehu ma lalo o ka ʻili ma kahi o ka papa.

Hoʻokumu ʻia ka angioedema hereditary (HAE) e kahi pae haʻahaʻa a kūpono ʻole paha o kahi protein i kapa ʻia ʻo C1 mea kāohi. Hoʻopili ia i nā kīʻaha koko. Hiki i kahi hoʻouka HAE ke hopena i ka pehu wikiwiki o nā lima, nā wāwae, nā lālā, ke alo, ka ʻōpū o ka ʻōpū, larynx (voicebox), a i ʻole trachea (kahe makani).

Hiki ke lilo i ka hōʻeha o ka ʻōiwi i mea ʻoi loa i ka wā ʻōpio a me ka ʻōpio.

Aia kekahi mahele maʻamau o ke ʻano o ka ʻohana. Akā ʻike ʻole paha ka ʻohana i nā hihia ma mua, a i hōʻike ʻia paha me he mea lā i manaʻo ʻole ʻia, make koke, a make ʻole hoʻi o kahi makua, ʻanakē, ʻanakala, a makua makua paha.

Nā kaʻina hana niho, nā maʻi (e like me ke anu a me ka maʻi palū), a me ke kahakaha ʻana e hoʻomaka ai i ka hōʻeha ʻana o HAE.

ʻO nā hōʻailona me:

• Pā paʻa o ke kahawai
• E hana hou i nā māhele o ka hoʻopiʻi ʻana o ka ʻōpū me ke kumu ʻole
• ʻO ka pehu i nā lima, nā lima, nā wāwae, nā lehelehe, nā maka, ke alelo, ka ʻāʻī, a me nā genital
• ʻO ka pehu o nā ʻōpū - hiki ke koʻikoʻi a alakaʻi i ka ʻōpū o ka ʻōpū, ka luaʻi, ka make wai, ka palahī, ka ʻeha, a me nā haʻalulu i kekahi manawa.
• Kahi huehue ʻole, ʻulaʻula

Nā ho'āʻo koko (hana maikaʻi ʻia i ka wā o kahi hanana):

• C1 mea hana hoʻopōkā
• ʻO ka pae mea kāohi C1
• Pākuʻi 4

ʻAʻole holo pono nā Antihistamines a me nā lāʻau lapaʻau ʻē aʻe i hoʻohana ʻia no angioedema no HAE. Pono e hoʻohana i ka Epinephrine i loko o nā hopena hoʻoweliweli i ke ola. Aia kekahi mau lapaʻau hou aʻe i ʻāpono ʻia e ka FDA no HAE.

Hāʻawi ʻia kekahi ma o ka aa (IV) a hiki ke hoʻohana ʻia ma ka home. Hāʻawi ʻia nā poʻe ʻē aʻe ma ke ʻano he injection i lalo o ka ʻili e ka mea maʻi.

• ʻO ke koho o nā agena e hoʻokumu ʻia i ka makahiki o ke kanaka a kahi e loaʻa ai nā ʻōuli.
• ʻO nā inoa o nā lāʻau hou no ka mālama ʻana iā HAE ʻo Cinryze, Berinert, Ruconest, Kalbitor, a me Firazyr.

Ma mua o ka loaʻa ʻana o kēia mau lāʻau hou, hoʻohana ʻia nā lāʻau androgen, e like me danazol, e hōʻemi i ke alapine a me ke koʻikoʻi o nā hoʻouka. Kōkua kēia mau lāʻau i ke kino e hana i nā mea hōʻemi C1 hou aku. Eia nō naʻe, he nui nā wahine i loaʻa nā hopena maikaʻi ʻole mai kēia mau lāʻau. ʻAʻole hiki iā lākou ke hoʻohana i nā keiki.

Ke hiki mai kahi hoʻouka, hoʻopili ʻia ka mālama me ka ʻeha a me nā wai i hāʻawi ʻia ma o ka aa e kahi laina intravenous (IV).

Helicobacter pylori, kahi ʻano bacteria i loaʻa i loko o ka ʻōpū, hiki ke hoʻāla i ka hōʻeha ʻōpū. ʻO nā Antibiotics e mālama i nā bacteria e hōʻemi i ka hōʻeha ʻana o ka ʻōpū.

Hiki ke ʻike hou aku i ka ʻike a me ke kākoʻo no ka poʻe me ke ʻano o ka HAE a me ko lākou mau ʻohana ma:

• National Organization for Rare Disorder - rarediseases.org/rare-diseases/hereditary-angioedema
• ʻO ka Hui Angioedema Hereditary US - www.haea.org

Hiki i ka HAE ke hoʻoweliweli i ke ola a kau palena ʻia nā koho lapaʻau. Aia ke ʻano o ke kanaka ma muli o nā ʻōuli kikoʻī.

He mea make paha ke ʻōhū ʻana o nā ala ea.

Kāhea a kipa aku paha i kāu kahu mālama ola inā ʻoe e noʻonoʻo ana i ka loaʻa ʻana o nā keiki a loaʻa kahi moʻolelo ʻohana o kēia ʻano. Kāhea aku nō hoʻi inā loaʻa iā ʻoe nā hōʻailona o HAE.

ʻO ka huehue o ke ala ākea kahi pilikia weliweli loa. Inā paʻakikī ʻoe i ka hanu ʻana ma muli o ka pehu, e ʻimi koke i ka lāʻau.

He mea kōkua paha ke ʻōlelo aʻo kūlohelohe no nā mākua me ka moʻolelo ʻohana o ka HAE.

Maʻi Quincke; HAE - Hereditary angioedema; Mea kāohi iā Kallikrein - HAE; ʻO ka Bragonkinin receptor antagonist - HAE; C1-mea kūpale - HAE; Hives - HAE

• Nā Antibodies

ʻO Dreskin SC. Urticaria a me angioedema. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 237.

Longhurst H, Cicardi M, Craig T, et al; Nā kānaka noiʻi COMPACT. Kāohi i nā hoʻoilina angioedema hoʻoilina me kahi mea hōʻemi C1 subcutaneus. N Engl J Med. 2017; 376 (12): 1131-1140. PMID: 28328347 pubmed.ncbi.nlm.nih.gov/28328347/.

Zuraw BL, Christiansen SC. ʻO angioedema hereditary a me angioedema i waena waena o bradykinin. I: Burks AW, Holgate ST, O'Hehir RE et al., Eds. Middleton's Allergy: Nā Manaʻo a me ka Hoʻomaʻamaʻa. 9th ed. Piladelapia, PA: Elsevier; 2020: chap 36.