Kushayikwa Alpha-1-antitrypsin: zviratidzo, hunokonzera, kuongorora chirwere uye kurapwa

Kushayikwa Alpha-1-antitrypsin - chirwere of tinozvarwa hunhu, chinopfuudzwa panguva nemagene pachiyero, pamwe pamuviri mwana. Zviratidzo dzinotanga kuonekwa mumazuva upenyu mwana wokutanga. With kurapwa zviratidzo vari kuva siyana, uye murwere nemamiriro zvishoma nezvishoma Zvinoipisisa pamusoro nguva. Varwere vakawanda, vanachiremba rokuongorora pulmonary chimiro zvirwere. 12 chete% wevarwere zviratidzo chiropa tishu. mushonga ano haana zvakananga zvinoreva kurapa chirwere. Naizvozvo, mukuru nechiyero zvinofanira zvainanga tava kuderera kunoita chirwere.

Chii Alpha-1 antitrypsin?

mapuroteni ichi, chiri kukonzera kugadzirwa chiropa masero. Kubva ipapo, inopinda muropa, uye waendeswa mumapapu. Nei tichifanira mapuroteni ichi? chikuru basa racho kudzivirira zvemapapu rinenge kubva kukuvara dzimwe mhando kwemapurotini - makemikari. Izvi zvinhu zvinokosha kuti kuipa nokufambira kukura yose muviri. Somuenzaniso, proteases vane basa achibvisa utachiona kubva mumapapu, kubatsira kurwisa utachiona. Pandakasunungurwa akawanda, naivowo kubatsira kudzivirirwa Formação de pakuona fodya utsi. Basa makemikari izvi zvinofanira kuva pakati nepakati. Mune chiitiko parameters kuwedzera pulmonary zvinhu dzinotanga itye. Alpha-1 antitrypsin unokubvumira kuchinja basa runovharidzira mamwe enzayemu.

Ko dhefisiti?

Mune imwe Sero neerekitironi rine okutodza 46 chete. Vose vari muzvikamu 23 nomwe. In peya imwe, mumwe chromosome nhaka kubva baba, mumwe - kubva mai. Chinhu filamentary marongerwo. Uyewo, mumwe Gene rine zvakananga kutevedzana kwezviitiko deoxyribonucleic asidhi uye anopindura imwe nzvimbo iri chromosome. Zvinoitawo anodzora rimwe basa kana kuita basa ravo mumuviri. DNA unoumba majini evanhu zvinhu.

Insufficiency Alpha-1-antitrypsin jini pamwe kuvadza chromosome №14. Kana kuti inoitwa mamwe makopi ose puroteni haina anogadzirwa waida akawanda, aumbwa dhefisiti. fuma iyi iiswe chiropa uye asingauyi kupinda zvemapapu tishu. kukanganisa kwakadaro kunosanganisira kuvadza Formação de uye emphysema kukura. Residual mapuroteni ari chiropa hwezvekukurukurirana paumbwe vanga tishu. Somugumisiro dzichiri chiropa kukundikana.

chirwere kufamba mashandiro

pathologies First dzinoratidzwa kushaya Arifa-1-antitrypsin, akarondedzerwa muna 1963 maererano Danish chiremba wacho Wall Erickson. Nhasi zvinonzi chirwere chaizvo kashoma. Zvisinei, nhamba varwere kunowedzera mune imwe geometrical kunoita. Sei? All nyaya iri majini hunhu zvirwere. The chirwere nhaka kana vabereki vose kutakura mwero Gene. anenge 1 munhu 25, Maererano nenhamba, zvakakanganisika akadaro chirwere. Kana vakaroorana vaviri vasikana chakaremara Gene vanosarudza pamuviri, rurefu ndingangokuvara chiedza chichava mwana hakuvaki. To musi, mumwe 5000 vanhu pane imwe chete kukura uzere kunotoda mufananidzo chete. Just Mumakumi emakore apfuura, nhamba idzi dzaiva nguva pazasi. zviuru 20 vanhu vakasangana chakaremara Gene imwe chete. nhamba idzi dzinoratidza pakagumira kuwanda pathologies.

Zviratidzo nezviratidzo kutanga chirwere

The kunotoda Mufananidzo pathological muitiro dzinongoita pakutsvaga chimiro chayo uye pakasvika lesion. Nokuti vanhu vakawanda, zviratidzo regai anozviratidza kwemakumi emakore. Vamwe, nekusiyana, iyo zvokurwara mberi nokukurumidza. Izvi imhaka mamiriro upenyu murwere uye nzira yake youpenyu.

Pulmonary kuratidzwa ndivo zvirwere zvakajairika. Vanosvuta vanoonekwa pashure pemakore 20. Varwere vanoomerera mararamiro utano, pachikumbaridzo iri kupfuura nenzira kusvika 2 nguva (40-45 years). Chokutanga, pane kupfupika mweya mushure zvikuru muviri. Zvishoma nezvishoma kunoita kuti kuparara kufema kwacho. Zvadaro anobatana chikosoro. chirwere ichi anowanzoonekwa dzinoratidzwa anogumbura maronda vaikudzwa kufema turakiti.

Kushayikwa Alpha-1-antitrypsin nevana, kana kwete chiropa chake zviratidzo kuonekwa mumazuva okutanga muupenyu. Vanoratidza pachavo muchimiro nehepatitis ose vasingazivikanwi etiology, jaundice ganda. By makore 14-16 ruzhinji kurapwa zviitiko kugadziriswa nenzira kurerutsa hepatic zviratidzo. Mune dzimwe kuyaruka akaponesa neapo anokakavara kurwadziwa zvakarurama hypochondrium. The pakuonekwa hepatic zviratidzo mune vakuru pangozi yokuita yematambudziko. Ndizvo kunoratidzwa scarring ari nhengo rinenge vakazochinja inoenda zvikuru cirrhosis uye rufu womunhu.

differential kuongorora chirwere

Protein Alpha-1-antitrypsin inoita basa rinokosha kushanda yose muviri. kukundikana kwake pamwe zvakakomba matambudziko, uye dzimwe nguva inotungamirira kurufu munhu. The mikana zvirwere zvinosanganisira:

• kuputa vanhu vane emphysema, akaonekwa makore 45;
• varwere panniculitis;
• vasiri vanosvuta vane emphysema chirwere makore 45;
• vana vachangoberekwa zvakaoma. jaundice uye yakakwirira chiropa makemikari;
• Varwere bronchiectasis pamusoro zvisingazivikanwi etiology.

Nokuti differential chirwere murwere unofanira kupa ropa kuti Alpha-1 antitrypsin. Kana kuongorora kunoratidza puroteni kushaikwa, nhengo imwe neimwe yemhuri kuti murwere vanofanira narwo zvizere zvokurapa kuzviongorora kuona chakaremara Gene. All mamwe mabasa Diagnostics zvainanga Pakusarudza mwero kukuvara tishu. Nguva nenguva unofanira firimu kuti:

• dzinoongorora upenyu zvemapapu vhoriyamu uye respiratoria;
• tsananguro spirometry;
• ropa bvunzo chiropa shanda nemiedzo.

kurapwa Guidelines

Emphysema chinokurwadzai kusingarapiki kuchinja. Saka, chero Zvakarurama kurapa hakurevi dzosera zvemapapu mamiriro kana kuvandudza mashandiro avo, asi kunogona kumisa kufambira mberi chirwere. Marapirwo zvinodhura chaizvo uye chete pachii varwere vasiri kuputa vane mwero kwazvo nhego dysfunction. Zvinonzi kuratidzwa kuti kana tikaongorora Arifa-1 antitrypsin uri mukati rezasi muganhu.

Kurega kusvuta, kushandisa bronchodilators - zvinokosha zvinoumba mushonga. Experimental kurapwa kushandisa phenylbutyric acid, izvo anobvumira kuti vaswedere kugurwa nokuumbwa mapuroteni ari hepatocytes achiri pasi kukura. Varwere vakawanda vaduku nemakore 60, vanachiremba vanoti zvemapapu transplantation. Information pamusoro zvakanakira kuvhiyiwa kuderedza muviri inzwi emphysema dzinopesana chaizvo.

Mabatirwo chiropa chirwere symptomatic. Kuzvitsinhanisa kuti makemikari ari ruzhinji dzacho basa, sezvo chirwere anokonzerwa pathological muitiro. Varwere chiropa kukundikana dzakaratidza nhego transplantation. Nokuti kurapa panniculitis vakashandisa corticosteroids uye tetracyclines.

mataridzikiro

Chii kutarisira mune varwere chirwere kuti "kushayiwa yeAlpha-1-antitrypsin"? Pakurapa chirwere hakungorevi zvakazorodza murwere ezvinhu. Chose kukurira zvokurwara ichi hazvibviri. The fungidziro kunobva mwero kukuvara nenyere. In kunyanya zvakakomba zviitiko, zviri zvisingafadzi.. Vanhu vanofa emphysema kana cirrhosis.

Masayendisiti pasi pose kuramba vachishingairira kuongorora chirwere, tsvaka kuti mushonga zvakasikwa. Somuenzaniso, muna US uye dzimwe nyika dzokuEurope, murwere asati atanga kuti experimental kurapwa aida mumaoko pamusoro kuongorora achisimbisa chirwere. Alpha-1-antitrypsin, zvinobva muplasma vanhu zvachose utano, izvozvo chinotarisirwa nomutsinga. Treatment rinoitwa pasi yakarongerwa zvirongwa. nzira kwakadaro inodzokororwa kakawanda pamwedzi. nzira iyi yokurapa inodhura uye proactive. Uyezve, hazvina akakodzera varwere hepatic zvinoratidza chirwere, kana ari pathological muitiro zvinobatanidzwa hakusi nyore. Muzviitiko zvakadaro, kurapwa ndiko kubvisa zviratidzo chirwere.

matanho

Dzakananga Kudzivirira chirwere nzira, mushonga ano unogona kupa. Vanachiremba vanokurudzira kuti vakaroorana kuronga pamuviri, kupinda kubvunza genetics. Izvi ndezvechokwadi kunyanya vabereki, mumwe uyo vanotova chakaremara Gene.

To muchidimbu

The chirwere kunoratidzwa kushaya pulmonary runovharidzira mamwe uye kuunganidza ari chiropa pathological antitrypsin, ndiko dererei. Zvisinei, rimwe gore nhamba varwere nezviratidzo inowedzera exponentially. Zvinosuruvarisa kuti kudzivirira kukura kwayo hazvibviri.