Hē o te Arepa-1-antitrypsin: tohumate, ai, tātaritanga, me te maimoatanga

Hē o te Arepa-1-antitrypsin - he mate o te natura tumu, tuku i te taumata iranga, me te haputanga o te tamaiti. timata tohu ki puta i roto i nga ra tuatahi o te ora o te tamaiti. Ki te tohu haumanu e hoko atu kanorau, me te huru o te manawanui āta worsens i runga i te wā. I roto i te nuinga o te tūroro, rata whakatau ahua pūkahukahu o te mate. 12 anake% o tūroro nga tohu o te ate kiko. e kore e rongoā Modern whai tikanga motuhake ki te hamani mate. Na reira, kia hāngai te mehua matua i tērā mate haereraa.

He aha te Arepa-1 antitrypsin?

Tenei pūmua, i te mea te kawenga mō te hanga o ngā pūtau ate. Mai i reira, tomo te reira i te bloodstream, a ka kawea ki te pūkahukahu. He aha e hiahia ana tatou i tenei pūmua? Ko tōna mahi matua, ko te ki te te tiaki i pūkahukahu kikokiko i kino ki te tahi atu momo o pūmua - whākōkī. He tika hoki te mahi noa me te whanaketanga o te kaiao katoa ēnei matū. Hei tauira, he kawenga mō te tango i huakita i te pūkahukahu, te āwhina ki te whawhai i te mate proteases. A, no te tukua i roto i ngā rahinga nui, whai wāhi hoki ratou ki te tiaki o te alveoli i te rongo ki te paowa tupeka. Me whärite te mahi o enei whākōkī. I roto i te tawhā kaupapa ki te whakanui ake i ngā āhuatanga pūkahukahu timata ki te ngohe. Alpha-1 antitrypsin taea ki a koe te whakatika i te mahi o te pūmua whākōkī protease.

Kia pehea te Ko te takarepa?

I roto i te kotahi pūtau kei karihi 46 anake pūira. Kua wehewehea katoa o ratou ki 23 takirua. I roto i ia takirua, riro tetahi pūira i te papa, te atu - i te whaea. Ko te hanganga filamentary. I roto i te tahuri, Kei ia ira o te raupapa motuhake o te waikawa deoxyribonucleic me noho he wāhi motuhake i roto i te pūira. Reira mana hoki he mahi motuhake ranei te mahi ratou ake mahi i roto i te tinana. DNA hanga he rauemi ira tangata.

tahi iti hoki Alpha-1-antitrypsin ira e kino ki te pūira №14. Ki te hoki tāruarua o te pūmua e kore e hua i roto i te rahinga e hiahiatia ana, i hanga te takarepa. whakatakotoria ana tēnei taonga i roto i te ate, me e kore e tomo ki te kiko tana pukapuka. Taua ofatiraa e titau kino ki te whanaketanga alveoli me emphysema. pūmua wäriu i roto i te ate whakahaere te hanganga o te nawe kiko. Ka rite ki te hua o te whakawhanake kore ate.

tikanga mate tuku

pathologies Tuatahi āhuatanga e te kore o te Arepa-1-antitrypsin, i whakaahuatia i roto i te tau 1963 e te rata Danemaka te Wall Erickson. I teie mahana te pāngia reira rawa uaua. Heoi, faarahi te maha o te tūroro i roto i te haereraa āhuahanga. Te aha? te mea katoa i roto i te āhua ira o mate. riro te mate te ka kawe matua e rua i te ira rerekē. E ai ki ngā tatauranga, e pā ana ki te 1 tangata 25, pāngia he koha taua te. Ki te whakatau i te hoa e rua o te takoto ritenga ki te ira ki te hapu i te tamaiti, ki te tūponotanga nui o te marama, ka waiho i te tamaiti hawata ana. Ki te rā, mo nga iwi 5000 i reira ko tetahi ki te whanaketanga o te pikitia haumanu tonu anake. Just he torutoru tekau tau ki muri, ko enei whika i nga wa i raro. tutaki 20 mano iwi ki tetahi anake ira. Enei tatauranga whakaatu te whānuitanga ki tonu o pathologies tini.

Tohu me nga tohu o te mate tuatahi

Kei te nuinga o whakaritea te pikitia haumanu o te tukanga pathological i tona āhua me te whānuitanga o te lesion. Hoki te nuinga o te iwi, e kore e nga tohu faaite mai ratou mo tekau tau. Etahi, i runga i te anga ke, kauneke tere te māuiuitanga. taia ana tēnei tukanga te tikanga ora o te manawanui, me tona ara o te ora.

faaiteraa pūkahukahu Ko te mate tino noa. Kaipaipa puta ratou i muri i te 20 tau. Tūroro e ū ki te āhua noho hauora, nui tenei paepae e tata 2 nga wa (40-45 tau). Tuatahi, i reira he hāhā o manawa i muri i te mahi kaha. Reira āta arata'i ki te ino o te tukanga romahā. Na ka honoa mare. Kei te pinepine tenei mate āhuatanga e pāpaka inflammatory o te wahi romahā o runga.

Hē o te Arepa-1-antitrypsin i roto i ngā tamariki, ranei, kaua puta ana tohu ate i roto i te ra tuatahi o te ora. faaite mai ratou ia ratou i roto i te puka o te kakā o te etiology unknown, jaundice o te kiri. Na roto i te 14-16 tau te nuinga o ngā take haumanu whakatau i roto i te ahunga o te whakaiti tohu ate. I roto i etahi o nga taiohi i whakaorangia mamae pukukino ruarua i roto i te hypochondrium tika. Ko te ahua o te tohu ate i roto i te mōrea pakeke o te whakawhanake pōauautanga. āhuatanga te tauira rite e scarring o te kiko okana, i muri arata'i ki cirrhosis nui, me te mate o te tangata.

tātaritanga pārōnaki

Pūmua alpha-1-antitrypsin e whai wāhi nui i roto i te mahi o te rauropi katoa. tahi tona kore e pōauautanga nui, a ētahi wā arata'i ki te mate o te tangata. Ko te kaha ake te tūpono o te mate ngā:

• e paowa iwi ki emphysema, puta ki te 45 tau;
• tūroro ki panniculitis;
• -Kaipaipa kore e whai emphysema pāngia i muri i 45 tau;
• tamariki whanau hou ki jaundice nui me whākōkī ate teitei;
• Tūroro ki bronchiectasis o etiology unknown.

Hoki tātaritanga pārōnaki me hoatu te manawanui toto mo te Arepa-1 antitrypsin. Ki te haapapu te tātari te hē pūmua, Me te melo tata'itahi o te hapu o te manawanui ki te mamae i te whakamātautau hauora tonu ki te kitea te ira. Katoa atu ngā mahi tātaritanga hāngai ana te whakatau i te tohu o te kino kiko. Wā, e hiahia ana koe ki te kia whakaaturanga hoki:

• aromatawai rōrahi ora pūkahukahu me te auau romahā;
• whakamāramatanga o te spirometry;
• whakamātautau toto i runga i ngā whakamātautau mahi ate.

Aratohu maimoatanga

Emphysema whakaoho huringa tina heke. Na reira, e kore e tetahi therapy tīpakongia whakahokia te hanganga pūkahukahu ranei te whakapai ake to ratou mahi, engari ka taea e mutu te reira i te ahunga whakamua o te mate. Ko te tino utu me te anake tūtohutia hoki tūroro kore-e paowa nei i tika nui pōrearea okana te maimoatanga. Kei te whakaaturia te reira e te mea ko te tātari o te Arepa-1 antitrypsin i roto i te rohe o raro.

Kai paipa, te whakamahi o bronchodilators - wāhanga nui o te therapy. maimoatanga whakamātau mā te whakamahi i te waikawa phenylbutyric, e taea ana ki te utu ko te hanganga pakaru o pūmua i roto i nga hepatocytes tonu i raro i te whanaketanga. He maha tūroro muri atu i te 60 tau, tākuta whakaaro tana pukapuka transplantation. He tino fepakipaki mōhiohio e pā ana te painga o te pokanga ki te whakaiti rōrahi tinana ki emphysema.

Ko symptomatic Treatment o mate ate. He whai hua whakauru o whākōkī i te nuinga o te wā, mai i te mate meinga e te tukanga pathological. Tūroro ki te kore ate whakaaturia transplantation okana. Hoki te maimoatanga o te panniculitis whakamahia corticosteroids me tetracyclines.

outlook

He aha ki te titau i roto i te tūroro ki te tātaritanga o "hē o Arepa-1-antitrypsin"? Treatment o te mate tā te tanga anake o huru o te manawanui. Mutu hinga kore e taea tenei māuiuitanga. Tei te waitohunga i runga i te tohu o te kino okana. I roto i te wā tino nui, he mea kino. mate te iwi i te emphysema cirrhosis ranei.

Scientists i huri noa i te ao tonu ki te tūhura mātātoa te mate, titiro hoki he rongoā ao. Hei tauira, i roto i te US me etahi whenua Pākehā, te manawanui i mua i te tīmata i te maimoatanga whakamātau tapaea ki te ringa i runga i te tātari whakau te mate. Alpha-1-antitrypsin, i takea mai i te wē o iwi tino hauora, kua whakahaerea reira e pâmuhia. kawea maimoatanga i roto i runga i te kaupapa i āta hangaia. Taua tikanga toutou te rave rahi taime ia marama. He utu a kōkiri i tēnei tikanga o te maimoatanga. I roto i te tua, e kore te mea te reira e tika ana mō ngā tūroro ki faaiteraa ate o te mate, ka i roto i te tukanga pathological whai wāhi kore he ngāwari. I roto i ngā take pēnei i, maimoatanga ko ki te tango i te tohu o te mate.

āraihia

Motuhake tikanga ārai mate, e taea rongoā hou whakahere. tūtohu Doctors e faaipoipo whakamahere ki te hapu i te tamaiti, te haereraa i te iranga kōrero. Ko rawa pono o ngā mātua, tetahi o nei kē te ira tenei.

Hei whakarāpopoto

huru no te mate e kore o protease pūkahukahu me te haaputuputuraa i roto i te antitrypsin ate pathological, he mea he onge āhua. Heoi, nga tau whakanuia ana te maha o te tūroro ki tohumate ē. Kia aroha mai, ki te ārai i tona whanaketanga e kore e taea.